Chris' Journey to Health - Amyloidosis Blog

Chris at almost three years

2011-04-02T08:54:00.002-04:00

Another long overdue update, but as this blog serves as both a communication vehicle as well as an historic log of her journey, even a late update is required. This week, the last week of March, 2011, Chris had her formal "three day annual check up" at the Boston University Amyloidosis Center. Things are going very well. The check up was done in two rather then three days, with the normal discussions with the Hematologist, Nephrologist, Cardioligist, etc. Each of the doctors had a similar observation in the clinic - Chris is doing very well. She seems very healthy, she is very energetic and has a great disposition, and she outwardly appears even strongetr then she was a year prior to her diagnosis. Just as importantly, her bio markers were ALL in the right direction. Specifically, the famous "free light chains (FLC)" metric was down to 36. This number has been bouncing around, so getting a trend has been difficult. Her prior FLC was 49 and prior to that 39 (so 39=>49=>36) but 36 has been the best number she really has had. Normal is 6-26. Her BNP ( which is a measure of congestive heart failure) was under 100 for the first time ever. A number over 100 is an indication of congestive heart failure and hers has steadily decreased. At the same time her ejection fraction has improved and her left ventricle wall thickness has decreased, these are all great indicators of improvement. In fact her kappa/lambda ration is within normal range, and her bone marrow plasma cell was less then 5%. Bottom line: all very good. Chris will go back in 3 more months for another blood test to see if we can get a trend in the right direction, not just these up and down spikes. The best two numbers were the BNP and the FLC, so while we remain very cautious, we had no bad news this week, only positive news. We did meet with both Dr. Skinner and Dr Sanchorawala and we have learned to read their expressions and voice and it is clear they are pleased. The disappointing part of the conversation was one around definition. Effectively you get labelled as having a hematologic complete response (HCR) only at the one year interval post Stem Cell Transplant. Thus Chris will never formally be a complete response, only a "very good partial response". She is eligible for another high dose chemo/stem cell transplant if required in the future and of course I continue to watch every single trial for Amyloidosis as well as all of the published research. Chris has committed to make conditioning her primary job. She continues to go to her personal trainer three times per week and to yoga two times per week with almost daily 2-3 mile walks with the dog. She admits to being out of breath at the top of hills, but never avoids the stairs. This past September we went to Sedona where she kept up with all of the others as we climbed some of the most difficult trails in the area. It was truly amazing how well she did at such altitude. She has recently taken two different ski trips to Utah and a visit to Florida. Overall, other then some shortness of breath after hills, Chris is simply doing great. Recently, I wrote an article for the Amyloidosis Foundation which was published in their newsletter. I titled it " It Takes a Family and a Village" with the central theme being that nobody can battle amyloidosis alone. It has taken a very special family and the amazing support and caring of the village around her to get Chris to where she is today. Believe me, we take nothing for granted and remain steeled against this disease coming back at any time. Yet as of April 2011, three years after Chris' diagnosis of Systemic AL Amyloidosis, Chris is living a very good life, and enjoying every day with appreciation for all the support she has been given by so many. More updates to come in the future and as always thanks for your continued interest and support.

Day Two Update of Two Year Evaluation

2010-06-29T21:06:00.004-04:00

Chris and I participated in the weekly Tuesday Support Group and then met with the cardiologist at the BU Clinic. We also are getting some of the key numbers. Overall, her markers have held steady or in some cases improved. Chris' Lambda free light chains were at 39.7. (Normal range is 5.7 to 26.3) This has effectively held steady for the past year where it has fluctuated between 39 and 41 - within the margin of error for the test. She had a high of over 660 prior to treatment and getting down to 40 or so represents about a 95% improvement which is consistently called a "very good partial response". Her Kappa/Lambda ratio was .22 which is outside of the normal range of .26 to 1.65. Her 24 hour urine protein was 1430/Mg which is down from a high of over 4000. Normal is 0-150. So the net result at the end of day two is that her numbers have held steady or improved. Yet, without a hematologic complete response, it is very likely that she will see a gradual re occurrence in the future. As I have stated before, this could be another stem cell transplant (she has half of her stem cell collection from 2008 still frozen and able to be used for replant) or a cocktail mix given orally or via a drip. For now, I believe the course of action effectively remains the same: watch and wait. Watch with blood and urine tests every 3 months and wait for any significant movement in the key bio-markers: lambda light chains, BNP. 24 hour protein, and the echocardiagram.

The cardiologist told us today that her ejection fraction was 40% of normal (not good) but her left ventricle wall thickness had actually decreased. That was actually good news as it shows the potential that the heart muscle is starting to repair itself. In other words, the continued deposition from the high free light chains is not keeping up with the pace of the repair that the heart is making to itself. This whole area of cardiac repair is not well understood and there is little research done to date, but there has been clear evidence of significant improvement to those with a complete response. Again, Chris did not get a complete response and she still has congestive heart failure, but her heart seems to be improving. We'll know more tomorrow when we meet with Dr. Skinner who is the Director of the BU Amyloid program.

So overall no bad news, some good news, and more to find out tomorrow. Thanks to those who continue to call Chris, follow her progress, and keep her active.

Day 1 Update of Two Year Evaluation

2010-06-28T20:17:00.002-04:00

Chris spent the first of three days at BU today and all went well. She had a number of tests, for which we do not yet have results, but the discussions with the doctors were all positive. Chris had a chest x-ray, blood tests, the 24 hour urine test, and the painful bone marrow biopsy. We did meet with two doctors, including Dr. Sanchorawala for clinical observations and they were pleased with how she looked, her current course of medication, and her general level of activity. Of course I asked many questions about the Amyloidosis Rome Symposium that we missed and Dr. Sanchorawala was very willing to share with me the major findings around new diagnosis, type determination (e.g. AL, TTR, etc), treatments, and cardiac markers. I had read much of the findings already so knew most of the major news. It was clear to me that the progress in drug therapies continues to be positive. The various combinations seem to provide the most effective impact. Combinations of Velcade, Melphalan and Dex (V-MD) is one example with promise.
So tomorrow we will know her free light chains, her proteinurea, her ejection fraction, and her heart wall thickness. All key indicators of her health.
Thanks to all for the ongoing interest and support.

Two Year Mark for Chris' battle with Amyloidosis

2010-06-21T06:53:00.003-04:00

Chris and the family celebrated her two year "birthday" yesterday as she received her stem cell infusion in 2008, and thus her newly updated hematological blood system. While I have been long delayed in updating this blog, we continue to focus on Amyloidosis every day. Overall, she is doing very, very well. She remains extremely active with daily vigorous walks and visits to the gym or yoga 2-3 times per week. Her pill regiment continues with a cocktail of different things, primarily focused on reducing stress on the heart. Although one would never know it through observation, she still has congestive heart failure - harsh as that sounds. She is on medication to lower blood pressure (lasik) and prevent clots (Coumadin) and then a variety of vitamins, fish oils, etc. She generally feels very good with some occasional issues around sleeping well and bouts of nausea every few months. Her weight is close to where they want it and her hair is as thick as ever.

We will go back to BU Medical and the Amyloidois clinic next week for the formal three day evaluation. The timing is good as it will mean a full two years and lets us start the summer with up to date knowledge of her status. BU will run the full battery of tests but the ones we will be most interested in will be her free light chains, her 24 hour urine protein, the left ventricle ejection fraction, and the the heart wall thickness. Chris' free light chains continue to be above normal and are very slowly inching up, but the slowness and the the degree that they are above normal have caused the doctors to wait and see how things evolve over time. I am always more aggressive and want to take a more proactive approach, but they look at her and are comfortable that waiting before any further treatment makes sense. Next week will be an important week.

Chris has hit some other milestones in the last six months. In March she finally hit the ski slopes, after much painful prodding from me. She picked a great day, went with her friend Pam and myself, and skied at her own pace. It was great to see her out there enjoying herself, despite her cardiologist warning against it. I think she will be more comfortable next year now that she had this first day under her belt. Chris and I also celebrated our twenty-fifth anniversary this past week. Imagine her tolerating me for twenty five years. Of course I have big plans for the next twenty five so thus my vigilance on watching her progress.

We had planned a celebration of out twenty-fifth by going to Paris, Rome, and Tuscany for two weeks in the spring. Our itinerary was built around the Amyloidois Symposium in Rome which is the big conference held every ten years attracting the world leaders in amyloidosis research and clinical trials. I was honored to be asked by Mary O'Donnell to represent the Amyloidosis Foundation at the event and sit through the three days of presentations which really would have made me as current as possible on the state of research and clinical trials. Unfortunately, the volcano ash proved to be our undoing. Despite a number of attempts and many hours on the phone with the airlines, we just could not get there in time for the conference. As we had planned to go to Paris to see our friends and then get to Rome a few days later, we thought we could be flexible but our flights kept being cancelled and we were rescheduled for a week later, thus missing the entire conference. We are are now planning our trip for late September/early October and can spend more time in Tuscany which Chris wanted to do more then Rome as we had been there a number of times. Thus we will still celebrate our 25th, just a few months later. I am anxious to spend some time with the doctors at BU to validate what I did read from the conference. The trial data seems to be encouraging. I won't go into detail here, but my reaction to the trial outcomes is that the cocktail mixtures seem to be more successful. The combination of Velcade, Melphalen, and Dexamethasone (VMD) seems to have encouraging results.

A few other brief updates: Melissa graduated with her Registered Nursing degree from the University of Vermont last month and we are very proud of her. She is currently studying for her board exam, then heading to Argentina for much of August to attend a Spanish immersion program for health care professionals. This is on top her trip to Bangladesh in March to work with a health care center there. Melissa has been offered an RN job in Salt Lake City at the Huntsman Cancer and Research Center which is what she was looking for (by the way, they are well known for their work on Multiple Myeloma). She has found that oncology is her passion and she pursued this opportunity that she thought was a great fit, flew to SLC to interview for it and they offered her a position the next day. Over 100 applicants for 6 openings and Melissa came through with flying colors.
Abby had a great year at Hartwick and continues her studies on special education and sociology. She really enjoys working with young children in need of special support and has been doing internships in the Oneonta schools and YMCA. Her passion is inspiring to see. This summer she is coaching a swim team and lifeguarding and the kids all love her. She has such an open heart and children gravitate to her so quickly it is amazing. She is working plans for a term abroad and is active in her new sorority.
Stephen continues at Hamilton and is focused this summer on his triathlon training. As this is his last year as a junior, he hopes to do very well at the US National Championship and earn All-American honors again. Since my last update, he earned his EMT license, completed his Triathlon and Cycling coaching certification, completed the winter season as a member of the National Ski Patrol at Sunapee, and is lifeguarding and coaching triathletes this summer. He also shaved his head yesterday in recognition of the Chris' journey through the chemo and stem cell transplant. I think he has now done this every year since Chris' chemo.

Finally, we had dinner with Jenifer and Steve Gershman Saturday night. Jen was the woman who chronicled her battle with Amyloidosis in the press and Discovery Channel and whose commitment to raising awareness resulted in Chris' diagnosis. Jen had two stem cell transplants and replaced two hips and his doing fantastic. They are living life with their son and enjoying every minute of it. We will be forever indebted to Jen and Steve for their support.

I will update the blog with Chris' results next week. While I know I have been very poor at the outbound communication, I also know that we have hundreds of friends who ask about Chris and pray for her all the time. I know that continues to make a huge difference.

Update on Chris - October 6

2009-10-06T07:57:00.003-04:00

It has been far too long and as always my apologies for the long delayed updates. There seems to be little news to update the blog on until something more interesting comes along. Today will be very interesting however. First however is Chris' status. Overall she is doing and feeling very well. Other than some complaints about sore muscles in her legs, she is really feeling very energetic and is very active. As an example, this past Friday she drove up to Maine and back with my sister who is in the midst of some of her own medical issues. Chris went with her and participated in the discussions with the doctor, trying to understand the issues, the medical jargon, etc. For me it was great to hear her talk about it. Chris is now entering a new role, albeit just a small first time event, where she is the patient advocate. My sister has been very smart about being aggressive, but it is always a plus, as I have written about many times, to have some one else listening, posing questions, taking notes, etc, Chris, the patient advocate - this is very rewarding for me to see how far she is doing. She got home Friday, hosted some great family friends from Pittsburgh, went over to a small party, and then drove back up to Maine Saturday night to watch Melissa compete in here first half-marathon in Portland (Melissa did great). Then back home Sunday and immediately started painting some new windows we had installed. To me, this is evidence of her doing very well. She is active, generally with a great disposition, and living "in the moment". One way we will live in the moment is to take a family cruise this winter with all three kids home from college, along with my sister and her family. This disease has significantly shortened Chris perspective on time and she feels the need to do things in the "now", not in the future. I understand this completely. but I also know we still have a long future ahead of us and we need to plan for it. So overall, Chris is really doing very well. She continues to read every amyloidosis blog she can find, and devotes a great deal of time tracking her friends, even though she has never met them. In Ohio, NJ, Florida, California, she hurts for them when they hurt and she is thrilled for them when they are dong well. It is a strange social network, as Chris feels so much emotion based on there individual stories, yet has never met them, except Tim and Connie in Ohio. Chris wants to reach out and offer any help she can, but she also does not want to invade any privacy. For now, she just prays for all who have been impacted by this disease.

Tonight is a special night. Chris and I, and much of our family members will be in NYC at the annual Amyloidosis Research Foundation dinner. Aislinn and Stephen will be honored for their fund raising efforts based on the 1500 mile bike ride completed last March. This will be a night of acknowledgement for their hard work and appreciation for the contribution of so many many people. People from all over Holliston, friends from Worcester Academy, those who have lost loved ones, and those who have been watching Stephen and Aislinn blog all share in this celebration tonight. I believe Dr Skinner and Comenzo will be there along with the host, Mary O'Donnell, President of the Foundation. Also there will be some names of people who I cannot wait to meet. I will not put their names on the blog here, but I have been looking forward to this night for along time. Not only to see Stephen and Aislinn being recognized, but to meet some people I have been very anxious to meet.
Finally, I'll let you know that ABC News was at the house for a few hours last night interviewing Chris and Stephen. I expect this segment to be on the news in Boston - Channel 5 today or tomorrow. When it does run, I'll post a clip on the blog. This will be Stephen's second news story by a television crew. Articles have run in newspapers across the spectrum of print media, from local papers in Jacksonville, North Carolina, and Holliston to national publications such as AARP and Triathlon magazine. Stephen set out on a mission to improve awareness about amyloidosis, and for a 17, now 18 year old kid, he's done pretty damn well. Last night it was the 6:00 and 11:00 anchor in Boston who came to the house to do the story.

So possibly, while Stephen and Aislinn are being recognized in NYC tonight, there will be a story running in Boston - all focused on the need to improve awareness. I'll try to post some pictures of the event if I can.

Chris' next medical event is the blood and urine workup in Boston the end of October. This will be an important data point as it will tell us any change in direction or confirm Chris' overall improvement.

Thanks for being patient with me as I slowly update the blog. The news is usually not as exciting as it is today, but as always, all of our thanks to those who have been supporting, watching, calling, and helping Chris.

Report from BU Medical

2009-08-02T16:31:00.003-04:00

So just as expected, the team at BU reported back with their recommendation to come back for another round of testing in October. This will not be the 3 days of full evaluation by each of the specialists, but instead the full blood and 24 hour urine analysis. We are really in a strange situation here. Chris is feeling good, getting stronger, and yet her numbers have clearly stabilized outside of the normal limits. Thus the harsh reality is that we do not have a hematologic complete response. We have a "very good" response, but not a complete one. So the advocate in me says "let's take action", but I also know that action means another round of chemo, either another stem cell or an oral cocktail. Chris watches her fellow patients go through the oral chemo and the stories are not easy, but they know they need to do it. Chris is going to need to do something, so the question is when and what? The various treatment options are being tested and improved all the time, but we can't wait while more amyloid proteins continue to be deposited, albeit at a slow rate.

So we will wait for October, and until then we will do some vacations, get kids off to college, do some small trips, and most importantly focus on one thing - getting as strong as possible. As strong as she is now, any treatment will by extremely difficult. The stronger she is when she does it, the easier and safer it will be. She has to have a confidence, both physically and mentally as she enters another round. I am sure she will work hard at this. As far as I am concerned, she can join every gym, get any personal trainer, and spend as many hours as she wants on this single focus.

So, again, a very difficult conflict. Chris is doing and looking great. She has done incredibly well since last summer and continues to state she feels better than she did months before her diagnosis. Yet, without a complete response, this fight continues.

We see Dr Falk tomorrow, Monday, and I'll provide an update after that. It will be very interesting to see what he says.

Thanks for your interest and ongoing support.

Day 3 Update

2009-07-31T07:30:00.002-04:00

The third day of evaluation at BU was very uneventful. I'm not sure if that is good or bad. Chris met with the Nephrologist and they went through some of her numbers which are all generally good. Chris has had some minor kidney involvement, but nothing that is significant to have a long term impact. Overall the discussion went well and then turned to Stephen's bike ride and awareness campaign. Many doctors at BU are still buzzing a little bit about and bring it up when we go and meet with them.

So hopefully today we will here the results of the team meeting. I am almost sure that the direction we will take will be wait and see. The numbers are effectively flat and she is feeling very good, so they will want to see if any thing trends in any other direction. Yet in the long term this is un unsustainable strategy from my perspective. At some point, with the knowledge that Chris did not get a complete response, action must be taken. Without a complete respoonse, further amyloid deposition occurs. It might be a slow drip, but nobody leaves a dripping faucet as is for years. I would rather err on the side of action and want to get the leak plugged. Sitting idle for another 3-4 months really does not make too much sense to me, but we'll wait to see what we hear today.

In all the meetings with the doctors, Chris asked "what should I be doing" that she may not be doing currently. This was great to hear as she is really advocating now for herself. Last year she effectively sat in a minor stupor as things passed her by. She didn't really understand what the doctors were saying and just followed the directions given. Now she is asking the questions, seeking advice, getting a better understanding. It is great to see. The answer to the question about what she should be doing is always the same: Get strong, keep exercising regularly, eat well, and try to avoid getting sick. Efffectively her primary focus is the same it has been for the last 12 months - conditioning. Chris has been very good about that, but sometimes her now busy schedule gets in the way, like all of us. She has to make it a priority.

In the past, the famous "Friday Calls" have been major milestones. Does she have amyloidosis, yes or no? Will we do a Stem Cell/High Dose Chemo regime? Have we seen any progress since the transplant? Todays call will be uneventful I believe. Wait and watch.

On Monday we see Dr Falk to get the cardio perspective. We will hopefully know the new Ejection Fraction number and the left ventricle wall thickness. IF these are improving, that will be real cause for celebration. We have asked and been asked many times - "Can the heart repair itself ?" As I have stated before, there is no clear understandfing of this, no emperical evidence, but it has happend over time. If Chris keeps exercising hard, I personally am convinced that she will be living proof that her heart has strengthened.

Chris and I continue to read the other blogs of amyloidosis patients and relatives. Our prayers are with them and their families every day. I also continue to watch the clinical trials very closely. The two that are of most interest are Pomalidomide (CC-4047) and what is reffered to as V-MD which is a cocktail of Velcade, Melphalan, and Dexemethosone. They each seem to have very positive results. For those interested you can search on these terms with amyloidosis and see much of the updated information.

Thanks again for all the interest.

Day 2 Visit - 1 Year Anniversary

2009-07-28T20:20:00.002-04:00

Today went relatively well but a little disappointing for Chris and I who remain very optimistic generally. We have some of the numbers back and the important one, the lambda free light chain is now at 40. The last 4 readings have been 42, 41, 32, and today 40. Normal is 15-26 and Chris was close to 400 at one point. So the good news is it remains close to the norm vs where she was a year ago (the 94% response). The disappointing news was that her numbers did not go down to normal range. Effectively she has "plateaued" at the 40 level. Her 24 hour urine measurement is still going down, and that is good, but it also is not within the normal range - but again it is still trending down which is good. So we have come the realization that at one year out she did not get a complete response, which is what we had been praying for. But 94% is pretty close. So the big question - What do we do now? The doctors all say she is doing great clinically (direct observation) and clearly she is gaining weight, has much more stamina, and is feeling very good. The Pulminologist, Dr Burke, is very pleased overall with her lung improvement. She has no fluid in the lungs and the overall "definition" from the x-ray is very good, so he was happy. He commended her on her progress in exercising and gaining strength and her overall fitness. He did say that she has a "generous heart" which figuratively means that her heart has enlarged in order to compensate for it's reduced function over the last year. Yet we know that her heart has actually lost some of its thickness and he considers this "outstanding".

Chris also had an echocardiagram today which of course we will not have results until later in the week. We are looking for improvements in the ejection fraction (EF) from it's current reduced capacity. With her reduced BNP number and the reduction in her wall thickness, we might possibly see overall EF improvement, but that may be wishful thinking on my part. Dr Falk, the amyloidosis cardiac specialist has told us that there is no direct evidence of improved EF after Stem cell/high dose chemo treatment, but with so many positive signs we are optimistic. We meet with Dr Falk this coming Monday, with all of our tests results and a DVD of the echo. He will then give us his perspective on any improvement.

We discussed with Dr Burke the potential as to whether Chris might be a candidate for a second stem cell transplant. Of course he was non-committal (as he should be), effectively saying that we should cross that bridge when we come to it.

The normal Tuesday noon support group meeting was another reconfirmation of my now solidly held beliefs:
-Overall the medical community is grossly unaware of amyloidosis
-Having an aggressive patient advocate is critical, you can't fight this alone
-The only way to beat this thing is by working with the best in the world, such as BU and Mayo

We heard many stories where patients complained of symptoms for as long as a few years and they were all being treated for the symptoms, not the cause. If they ended up with an Oncologist they were treated for a blood disorder, if they ended up with a Cardiologist they were treated for heart conditions, if they were treated by a general practitioner they were diagnosed with fatigue, menopause, of just general aging. There was anger in the room as to the amount of misdiagnosis. Yet who is to blame? Doctors can't know everything, they are specialists at what they know, that is good. The issue is the general lack of awareness or the unwillingness to explore outside of their comfort zone. Every patient in the session today said effectively the same thing, they told their doctor that "something is wrong", in some cases many many times. Chris used to say it all the time until diagnosed and it was only the amazing luck of reading the article about Jen Gershman that Chris was diagnosed. Jen was committed to improving awareness and she was highly successful. Chris is now focused on the same thing. But again, I restate what was confirmed today: be aggressive, find a strong and organized advocate, and work tirelessly to improve awareness of this disease.

So for Chris, she has another day of test tomorrow (kidney and liver) and then we wait for the famous phone call on Friday. Yet I already know what the next step will be - wait and watch. My prediction is that the BU staff will want her back in November/December for more data points and trending (blood and urine tests). In the meantime, Chris' most important role is the same as it has been since last August - get as strong as possible. Exercise, lift weights, improve cardio conditioning, etc. If she needs another round, she will have to be very strong as this will give her the most number of options.

So overall Chris feels great, is getting stronger, and is generally living a very good quality of life. Yet it is clear we did not get the complete response we hoped for. Chris fought a very good battle and beat the enemy severely, but it is not dead yet. We don't know how badly these enemy proteins are wounded and what they are doing back to Chris, so we may need to go to battle again. So as of July '09, we wait and watch carefully for any signs of enemy movement. We will get the Chiefs of Staffs (the BU Team) back together again at the end of the year and reassess the situation. In the meantime Chris has to continue to strengthen he defense posture. (Sorry, I must have watched a war documentary recently with all these war analogies!!!). Chris and I know the war doesn't end until we get a complete response, so we can't declare success, but without any doubt in my mind, I am sure that Chris will be the victor. She is that tough.

More tomorrow, thanks for caring.

Day 1 visit - 1 year anniversary

2009-07-28T07:38:00.002-04:00

A few things to provide updates on, all overdue. First an update on Chris' trip to California last week. This was a first big test for her as to how she would be able to handle the travel, the crowds, the time zone, etc. and overall she did amazingly well. We were heading out for Stephen's Junior Elite Triathlon Championships in Oxnard - 60 miles north of LA, for 4 days, and despite incredible troubles getting there, she did great. We finally got to bed at 2:30 am (5;30 eastern of course) and while I thought she might be exhausted from the delays, rerouting, and general trouble, she was up the next morning and taking long walks along the beach. I think she even surprised herself. She enjoyed getting away, we had a great suite right at the triathlon site on the beach, and enjoyed the great company of the other athletes and parents who also came out. For me, it was proof that she really has come a long way to getting back to normal and can easily deal with the normal bumps in the road - something we could not even fathom a year ago at this time. Chris continues to walk daily and go to the gym or yoga almost every weekday. And with three kids "mostly" home this summer, she is very busy trying to keep order in the house.

So this was the status going into our first day of three at the BY Amyloidosis Center for her one year check up. We have been so busy we really didn't spend much time preparing ourselves - for either good or bad news. We know in general she has been doing very well, but as stated, we need to know what the numbers say. Day 1 overall was reconfirmation of what we thought, but these were all based on conversations. She had her blood work done (11 vials) and her 24 hour urine collection, but we will not get those numbers back until later in the week. We are of course looking at the free light chains in the protein in the urine as the critical bio-markers.
We met with two doctors including Dr. Sanchorawala. Both evaluations were very positive. Chris has no major issues that can be observed other than an occasional bout with GI problems that occur randomly. She explained that she generally feels great, exercises often, is gaining weight. She was told last summer when she was around 92 lbs. that her ideal weight she should target is 115. Yesterday she was 114.9 - and thus she felt good about getting a Dairy Queen Blizzard on the way home (doctors orders!).

Melissa joined us all day and found the whole process interesting, the discussions with the doctors, the conversations with the other patients (including the brand new, clueless, dear in the headlight folks - we used to be them), and all the testing they do. As a graduating nursing student next summer, it was another opportunity to see more medicine in action.

So we finished the day with two major realities. First is that unless we see surprising good numbers later in the week, it is clear that Chris did not get the Hematologic Complete Response (HCR) that we were hoping for. Dr. Sanchorawala ultimately decides at the one year mark whether a patient had an "HCR" and she told us that she doubted she would be able to identify that as the result for Chris. Her numbers all need to be within normal range and as stated in previous posts, they aren't. Yet they are close. Normal for free light chains is 15-26 and she is in the high 30's (from over 400 last summer). As such, she had a 94% response and as discussed earlier, the big question is "what is the tipping point" for action? If it was a 99.9% response, probably we would d nothing. If it was a 40% response, we would jump on the next course. But what do we do at 94%? Of course I asked that question many times and it is becoming clear that the answer is frustratingly the same as before - "let's wait and watch it". I told Dr Sanchorawala that I would predict the course of action prescribed at the end of the week - I predicted "come back in 3 months for more tests and evaluation". She smiled and nodded as she knows that is exactly what we will here. That however is frustrating but also good. Frustrating in that we want to take action, "out damn spot" as Lady Macbeth would say, let's finish this thing off. Yet good that right now no action is required - "no news is good news". Thus we have the raging battle of bad quotes.

The other major outcome of the day was a new awareness or insight that her heart is getting better and stronger. The thickness of the left ventricle wall (septal) has decreased (less stiffening) from 17 mm to 12 mm. This is really surprising and good to hear. Also her BNP number (used to measure the amount of protein produced by the heart) is way down (this is good), but still above normal levels. This is heading in the right direction and is confirmation that her heart is getting stronger - no doubt to her commitment to exercise. Yet the most surprising element of this improvement is that unlike what we had heard before, Chris might be able to tolerate a second stem cell transplant. We had been told earlier "no way" as her heart would not be strong enough. Yet now, that may in fact be an option. Of course we have no idea what the future plans are until we get the numbers back later in the week and the BU team discusses options.

We will go back today for more evaluations and the famous support group discussion every Tuesday. Based on who we met yesterday, I think many people will be looking at Chris and saying "wow, she looks great" and will get a dose of hope and optimism they need.

More updates tomorrow. Thanks for being interested and caring.

One year ago today - stem cell harvest #2

2009-06-12T17:08:00.003-04:00

Just a quick note that a year ago today- June 12th, 2008, Chris had her second day of stem cell collection where she gave up the last of her approx. 10 million stem cells before they would be wiped out by the melphalan. It is tough to read the old posts but amazing to see the progress. Chris continues to feel very good and has gone on notice that no matter what, she is "going to really enjoy this summer", having lost hers last year. You can read the posts from last year by going to the right of this page and hitting the arrow (you have to hit the triangle) >2008 that will then open up each of the months. Open up >June and you can see my posts of a year ago.

Sometimes it seems like eons ago that we went through that hell, other times it seems like yesterday. But in all cases we know we were truly fortunate to get the diagnosis, the treatment, and the support that we had so quickly. As I have said, we are not out of the dark, scary cave, and we don't know what lies ahead, but we also know that Chris has made incredible progress. We have a virtual army of supporters (all of you) who have made all the difference in the world.

Chris will be back for the next verdict (the next big turn in the cave) on July 27th. We have six weeks to enjoy the present and hopefully not worry about what we find out then.

It's been about 1 year...

2009-06-08T07:53:00.002-04:00

Chris and I moved into Boston for the start of her treatment one year ago this week. It was right during all the high school graduation parties for Abby and it is a bit strange as Stephen's have been going on all week (he graduated Friday evening) and it seems like deja vu all over again. We spent our first night in Boston at the new apartment on June 8th and she had day 1 of the stem cell collection on June 10. What a year.

This past week Chris has been doing the normal circuit of graduation parties and for many people, they have not seen her since last year. The comments are always, "you look great" or "wow, you have so much energy". A year ago we were both completely scared as to what would happen in the next few weeks, Chris was miserable with edema, shortness of breath, and overwhelming anxiety, and I was going into learn, advocate, and prepare mode (and also had overwhelming anxiety). Chris had been confirmed with amyloidosis, was almost rejected for the stem cell transplant due to heart damage, and we were walking into a dark cave where every turn was new to us. I certainly know the cave walk is not over, as we have many twists and turns and dangers ahead that we can't see or predict yet, but as we sit here today, Chris is doing great. Her edema is completely gone, her lungs are clear, her energy level is amazing, her blood pressure is good, and her weight is slowly gaining. All positive. She does have bouts with nausea (last week she lost everything, in all directions, for about a 5 hour period) but this seems to happen very rarely, about every 6-8 weeks. We can't figure out exactly what causes it, but she can't blame it on my cooking as she hasn't touched that since we left Boston. Chris' spirits are generally very good, except she struggles with 2 emotions: 1) sometimes she feels like she prayed so hard to make it through the kids high school graduations that now comes the payback and 2) she feels like she is a member of a community of amyloidosis patients (which she is) and she feels guilty when other community members don't do as well as her. Another patient (from the Pittsburgh area) passed away in the last few days having been to weak for a stem cell transplant. This is the third time in the last year that Chris has known or felt an attachment to someone who has lost the battle to amyloidosis. Each of these losses is a different story and each of them are tragic, and Chris feels so much pain for the families. She wants to feel good about her own results so far but knows the pain others are enduring.

Yesterday Chris drove down to Cape May NJ to attend the 8th grade graduation of her sister Debbie's son, Tommy. She and her parents are staying in a beautiful old hotel on the beach and it will be the first time Chris has travelled without me in over a year. She did not seem nervous at all, but we know that for some reason, when she travels, she seems to get very ill. I'm hoping this trip goes very well of course as we are heading out to LA next month. It is great that Chris is able to go down to NJ as Debbie spent the summer taking care of things back in Holliston. She gave up her entire summer last year helping us and Chris was determined to get down there for awhile this year.

We had some great news from our friends Tim and Connie from Ohio. Tim, who had the stem cell transplant last summer and has been on oral chemo since last fall has had some real improvement in his numbers. I spoke to him Sunday and he sounded very upbeat (despite a blood clot in his foot) and the doctors told him they expect even more improvement (at or within normal levels) by his next trip back to Boston in September. Tim has been on a roller coaster, but this is really exciting news.

Chris received a beautiful hand made necklace from Jen and Steve Gershman this week celebrating one year. Jen has been such an incredible inspiration to Chris and we both feel we owe so much to her and Steve. They were (and are) our mentors, our guides, and our friends. They are ahead of us in the dark cave we are in and they keep yelling back to us, "keep going, it's alright".

Our next milestones are: reliving some of the key days of June of last year, getting Melissa home from 6 months in Australia, getting to the triathlon in LA, and then getting the full one year evaluation at BU the week of July 27th. This will actually be 13 months, but we have had a number of tests throughout the year so we are ok with being a bit late and letting Chris enjoy July. In any scenario, Chris won't start any further treatment until September. We are hosting a long overdue party at the end of August with some of our best friends up in Maine and it is very important that we make this happen this year.

So that is the latest. Chris has more graduation parties over the next few weeks, and it is exciting to see her back being social, energetic, and spirited.

I have had many people tell me over the last week that when I don't update the blog for awhile, they get nervous, so I will do better. I also need to post some of the pictures from Stephen and Aislinns' visit to BU. I'll post as soon as I get them sent to me (hint hint).

Thanks for all the support. More soon.

Long Overdue Update -lots of new news

2009-05-29T17:19:00.003-04:00

Well, I will start off by making my regular apology for the long delays in updating Chris' status. There is no viable excuse other than too much going on. I guess by definition that is a good thing. Yet I also know so many people have been so supportive for over a year now that it is my responsibility to keep the supporters informed. There is a great deal of news to pass on, so I will do it in my normal bullet fashion. Let's start off with the most important:

-Chris continues to do well and in my opinion is getting stronger every day. She has recently been saying with great conviction she "feels better now" than she did in January of 2008 when things started going downhill. I am amazed at her energy and it even tires me watching her. She is bound and determined to enjoy this summer after losing all of last summer. As such, I need to almost drag her into the house at the end of a weekend day after working on her gardens for 10 hours straight. She is exhausted when she gets into bed, but it is a great exhaustion, not from feeling ill but from working her rear-end off. She continues to go to the gym regularly, multiple times per week. Rarely has she been feeling any sense of nuaseau, and really has had no significant side effects from the coumadin (blood thinner). She still takes a fist full of pills every morning and evening, but rarely does she complain about that. I think it is just part of her routine and in the end she knows it is good for her. Chris recently had a bone density test as we know that the chemo is so strong that it can do damage to the bones, such as the hips. The test came back as borderline with some minor weakening and thus she has been told to increase her calcium intake, e.g. lots of milk. I hate to admit it, but I have been telling her this for years, she just does not get enough calcium (I know what her two grandmothers looked like in their late eightees), and since I plan on being with her for another 40 years, I don't want her to be bending over too much (but I'll take it if that is the biggest problem she has).

-Chris had some tests (blood and urine) done a few weeks ago at BU. The markers continue to improve as indicated here:

Lambda free light chains 7/2008: >760, 1/2009: 39, 5/2009: 32 Normal: 6-26
This shows further reduction from January to May, great news as still heading down

Lambda/Kappa Ratio 7/2008: could not calculate, 1/2009: .29, 5/2009:.30 Normal: .26-1.6
This is within normal range

24 hour urine protein: 7/2008 3700 mg/Day, 1/2009: 2800 mg/day, 5/2009: 2200 Normal is under 150, so we have more to go here, but it is still heading down.

So overall everything keeps heading in the right direction and when you combine this with her overall general health, we remain optimistic.

-Chris continues to read the other blogs of amyloid patients. She wants to reach out to them and tell them how important hope is, and show them that there is light at the end of this long tunnel, but she doesn't want to insert herself either. Some are having very tough battles, others doing well, and others have lost their loved ones. The latter is the one she wants to reach out and help the most, but she doesn't know how to. We both pray for all.

-Our friends from Ohio, Tim and Connie, have been making a tremendous, unselfish contribution to others less fortunate patients by creating a home in Boston for Amyloidosis patients who need additional support. They have provided and furnished an apartment close to BU and have been out raising awareness by contacting the medical community with some outstanding information pamphlets. I cannot overstate the passion they have to help others in the midst of Tim's own fight, which he is winning. Secure Harbors will help so many who need the help to win this battle. Here is their web site: http://www.secureharbor.org/goals.php
Chris went out today to furnish it with some of the funds Connie and Tim have already raised. It will be a much better option than living in a bad motel for many weeks or months. This is another way for Chris to give back. Connie ands Tim are in Boston this week and will continue to get the new apartment set up. This is really exciting.

Chris really struggled with whether she could go to visit Melissa in Australia in June. She wanted to badly, but in the end was worried that travel seems to still take a toll on her. She was also invited with some great friends to Paris but turned that down as well. Now her objective is a trip to California for one of Stephen's Junior Elite National Triathlon Championships in July.

Chris is due to go back to BU for the one year evaluation next month, possibly July. It will be her one year anniversary from the stem cell treatment this month and they want to do a full evaluation on their patients at that milestone. It is not scheduled yet, but will happen late June or July. As always, they will do the complete head to toe and organ examination. We honestly have no idea what the diagnosis will be and hat the treatment plan will be. Anything from another stem cell transplant/high dose chemo to a 6 month regiment of oral chemo to doing nothing.

The really exciting news is that Stephen and Aislinn were asked to come and present their senior project to the doctors and researchers at Boston Medical. Dr. Skinner and Dr. Seldin, who run the program asked them to come and make a presentation about their fund raising efforts, their ride from Florida to Boston, how they planned it, etc. It was really great to see two high school students in front of some of the worlds leading experts, explaining their senior project. It was a great presentation, they were both very comfortable in front of a large group making a powerpoint presentation. and it was very interactive. The doctors asked questions about the ride, who they met, the good and the bad, etc. They wrapped up with an update on the total amount raised: almost $58,000. This is against a goal of $10,000. So many people gave so much support, whether in dollars, hosting parties for fundraising, sponsoring the ride, letting them stay in their homes. The support just continues to be amazing. Every single dollar raised has gone directly to the Amyloidosis Research Foundation. Stephen and Aislinn were also invited back to do some "shadowing" with some of the doctors and possibly discuss internship opportunities. After the presentation, they were given a tour of the research center. Filled with graduate students, interns, etc, it was an exciting place to see. The tough part was seeing the station where they do the test to determine amyloidosis. This is where tissue samples from all over the world are sent, to be stained with congo red and placed under a polarized light looking for apple-green birefringence (effectively a green glow seen under the microscope). I am sure this was the exact station inside the lab where Chris' cells were first confirmed as having amyloid deposits. To me, my own personal feeling, was that this is almost the place where many peoples lives are determined. Do they have amyloidosis or not? Well, just look into the microscope and we will know one way or the other. Yes or No. Hundreds of peoples lives: patients, spouses, children, family members and friends of all types will be affected by what shows up in that microscope. Very strange for me to see where Chris' diagnosis was confirmed. I should have asked the question but did not: How many are postive for amyloidosis vs negative? My guess is that because this disease is so hard to understand and the awareness is so limited, that the vast majority are positive. Patients are well down the road before anyone does a test. In fact, one would hope that through greater awareness, more testing for amyloidosis would be done earlier and the ratio of positive vs negative would go down. In other words, testing for this was more routine, thus lowering the percent of positive diagnosis but more importantly catching this earlier by being more aggressive with the testing.

So this month of June, 2009 will be an important one for Chris and all of us. It represents a real milestone - 1 year since treatment, and we will know this month or next what the future course of action holds. I never assume this thing is beaten, and I worry that people start taking her progress for granted. This will be a lifelong fight that we can never let our guard down against. Being an aggressive advocate, all the time, never easing up, is the name of the game in this fight. It is easy to forget that there is no cure for amyloidosis. It does not exist yet. Until there is a cure, the research, the support, the aggressive advocacy all have to continue.

Thanks for reading. I'll post more often as some very important milestones are met this month.

Update on Meeting with Dr. Falk

2009-04-09T08:38:00.002-04:00

Chris and I met with the cardiologist whom we like and believe to be the world expert on cardio impact from amyloidosis. He basically told us a few things:

-Chris continues to look good "clinically", i.e. through direct observation. No edema, no lung issues, good overall appearance.

-Her heart really has not changed much in the last 9 months. No reduction in ejection fraction, no further wall thickening, no other major changes. He did confirm that echocardiagrams were highly subjective and he is becoming more convinced of that over time. I found that very interesting.

-We discussed at length the options going forward. I asked specifically what would be the plan if Chris stabilizes at this 94% response (vs a complete response). He did not want to step into the role of the hematologist, and was really stating that BU needs to decide that next step with us. He did say, that the chance of a relapse is very high if in fact there is no complete response. This could happen soon or it could be a few years, nobody really knows. So this confirmed my strong belief that we need to continue to be aggressive with treatment as we try to get a complete response. We have all come to the conclusion that her heart is not strong enough for another stem cell transplant. This leaves oral chemo as the options, either Velcade or Revlimid. Dr. Falk indicated Velcade seemed to have a good success, but was a little more toxic. Again, he is going to leave the go forward strategy to BU.

-We also discussed the option of a defibrillator being implanted. He had mentioned this in an earlier discussion and has now ruled it out as not required. This of course is good news.

So overall, things remain stable, no further decline in heart health, but as we still have an abnormally high lambda light chain number of 40 (vs 26 as normal), there is still work to determine how to get a complete response. Chris goes back for another round of blood work in May and we will see what those numbers look like.

A few other happenings:

-April 4th was one year since Chris received the phone call from her doctor while she was playing tennis. Her doctor came in from vacation to meet with her personally and explain that something was wrong. At that time we did not know what we were facing, but we were very concerned. We heard all these words that we had no idea what they meant: mgus spike, Waldenstroms, Multiple Myeloma, amyloidosis. I was visiting Hartwick overnight and spent the entire night searching the internet for what I could learn. The worst part was reading that the average life expectancy for newly diagnosed amyloidosis was 13-15 months. I cannot describe the feeling and I pray I never experience it again. It would take a book to describe all my thoughts, blurring by my head at warp speed, and the complete fear I had. I got home and Chris and I cried all night long. So one year later, I feel as if we have dodged a bullet. Shots might still be fired, but we ducked one so far. We are still in the combat zone, yet we are running fast to find safe haven (a complete response). I know how lucky we are, and I can clearly state that we are grateful everyday. There are so many other stories of courageous battles fought by others. Some have been won, but the ones that break our heart are the ones that have been lost. We heard so many stories from people who donated to Stephen's ride. People wrote, "my brother succumbed to this", or "my husband was not diagnosed early enough and lost his battle". Just as I pray and am thankful for Chris' progress, I also pray for the strength of those who have lost loved ones to this disease. In many cases we know your names, we read the blogs, we see what others are going through, and our prayers are with you.

-I pushed Chris very hard to go skiing on April 4th. I really wanted her to get back into a normal sort of life. Get out, enjoy the snow, get some sun, and have some fun. I thought this would be an emotional milestone for her on her one year anniversary. She could conquer another roadblock. Walking up the driveway was a milestone. Driving a car by herself was a milestone. Helping others with a need was a milestone. I was hoping she would really enjoy a few hours of very slow, careful skiing, which she and I have enjoyed all over the US and Europe for years. Unfortunately it did not happen. The snow was very heavy, it was spring skiing with some bare spots, and she did not feel comfortable doing it. I really wanted her to push herself to do this, but I understand why she chose not too. Hopefully next year.

-One area that Chris now has to focus on is her cholesterol. This has risen over the last few months above normal. She has been trying to put weight on and has indulged in some great but bad foods. Kim's brownies, Caroline's chocolate mousse, unlimited ice cream - all with the intent to put some meat on her bones has now raised her cholesterol (and put pounds on me as well). So now we have the difficult task of reducing cholesterol while gaining weight. The food brought by friends has been the best. These people can cook!!. Yet now it is back to reality on the food selection. I take this as a good sign. Before, the BU doctors were saying that cholesterol was the least of her problems. Now, as she needs to focus on it, it means that we are thinking longer term. This is good.

-I will not steal Stephen's thunder on his fund-rasing efforts, but I will say that he, through all of you, has blown away the objective he set by an order of magnitude. It is beyond amazing. The letters, the donations, the inspiring stories have all been just incredible.

-Chris and I hope to get a short vacation away soon. She did not get to go to Florida, and did not get on the ski's, so now we will try to do some fun travelling and look back on a hell of a year.

Thanks again for everything.

Lung Improvement

2009-03-30T07:39:00.002-04:00

Chris had a CT scan and a number of doctor appointments last week to have her severe coughing looked at. In the end, it seems as if she had a case of both bronchitis and some pneumonia in her left lung. As an infection, she could have picked this up anywhere, but the stronger antibiotic seems to have made a difference over the weekend. She has almost stopped her continous coughing and thus is not coughing up blood either. Her doctor and the CT scan showed something called "ground glass" in her lung, which is basically scarring. In the end, this does not appear to be a long term problem and should resolve it self shortly.

Unfortunately Chris was unable to go to Florida to visit her parents. She really deserved a few days sitting in the sun, relaxing, warming herself up after the winter, but better safe than sorry. It is always a tough decision, the balance between being cautious and living your life. We can't hide in a cave the rest of her life, and she deserves to enjoy a normal existence. Yet that comes with risks of infection, and other issues. I am encouraging her to start getting back to normal, with travel (short distances for now), normal activities (hopefully some easy skiing this coming weekend), and continued daily exercise. She is anxious to get out in her gardens and I know she is looking forwared to summer, which she had none last year of course.

Chris did get a front page picture of her hugging Stephen in the local paper last week from his arrival home from the bike trip. She of course hates it, but it shows her great happiness and strong emotion as she greeted him after his incredible ride. So many peopl have been inspired by this story. Stephen received a very powerful letter, with a very nice donation to the Amyloidosis Research Foundation from a woamn who's husband passed away primarily due to lack of a diagnosis. As she stated, they kept treating the sympoms and not the cause, thus delaying possible treatment. By the time he was evaluated at BU Medical, he had lost an opportunity for aggresive treatment. This is exactly why awareness is so important. With the funds Stephen has been raising, donated by so many incredibly generous people, it is his intent to help prevent these stories from happening. Of course, like any rare disease, it will be a continuing battle.

Hopefully I can get Chris out on the slopes this weekend, and hopefully my plan to hit a few tennis balls with her comes true soon. She has been through an incredible year, and it is time to expand the horizon a little bit before we go back for the big 12 month evaluation in June.

Thanks for your ongoing support, interest, and well wishes to Chris. It means a great deal.

An amazing day....

2009-03-23T11:44:00.004-04:00

Yesterday, March 22, was a great day for many many people. It was a great day for Chris and I, and our entire family, it was a great day for those impacted by and working to cure amyloidosis, it was a great day for an incredible community of supporters, and it was a great day for Stephen and Aislinn.

Stephen and Aislinn arrived home after a grueling 1500+ mile/15 day bike ride from Jacksonville, Fl. to Holliston, MA. It was a once in a lifetime experience for them and all those who participated. They had good days and tough days, they met many great people, they promoted awareness, and they raised a staggering amount of money. Every dollar they raised, over $40,000 and still climbing, is going directly to the Amyloidosis Research Foundation. All expenses were covered by sponsor support, or frequent flier points, or the generosity of hosting families, so not a single dollar raised will go anywhere but directly to research.

There was a crowd of over 100 people when Stephen and Aislinn arrived yesterday. Friends from Holliston, schoolmates,faculty, and staff from Worcester Academy, family from PA, NJ, NH, fellow patients, and leaders in the Amyloidosis community such as Mary O'Donnell and Dr. Comenzo, and fellow bike riders from MinuteMan Road Club. They were both stunned at the reception and had no idea how many supporters across the community they had.

Stephen and Ailsinn learned many valuable lessons that they will post in their blog when they catch their breath. The biggest lesson I think we all have learned has been the incredible generosity from so many. It is mind-numbing. In this economy, with so many people worried about their financial stability, their savings impacted, and their future unknown, the number of people who made a contribution was just incredible. Every single donation meant a great deal, whether it was $5 from a child to an incredibly large, and anonymous donation (I do not know who that was, but I pray you walk with a personal pride of your great generosity and contribution). The community that we live in, all of our friends, all of my colleagues at IBM, all of those who gave becuase they suffered or lost loved ones from this disease, all of those who gave in any way were incredibly generous. As I have posted many times, just as "it takes a village to raise a child", it takes a village, an extended community, to fight a disease. Chris so far is very fortunate, but others have lost loved ones to amyloidosis. A year ago the vast majority of us had never heard of this disease. Now we are battling it on many fronts, but with an incredible village of loving, caring, supportive, and generous people with us.

I am anxious to read Stephen's and Aislinn's postings, but I am also anxious to say THANK YOU to everyone who has helped with Chris's journey to health.

Thanks to all of you so very very much.

Home again...

2009-03-22T06:27:00.002-04:00

Chris was sent home from her night in the hospital late Saturday afternoon. She has a combination of bronchitis and early pnuemonia caused by a viral infection. She has prescriptions for a very strong antibiotic, an inhaler for opening the bronchial tubes, and the old standby Robitussin with Codine for her cough. Basically she got a bad viral infection that we did not treat quickly enough and it got into her lungs. Her coughing has irritated her bronchial tubes so much that she was coughing up blood and the coumadine blood thinner did not help. She felt well last night and is sleeping soundly. Hopefully Chris bounces back quickly as she plans on visiting her parents down in Tampa for a few days this coming week with Abby. The sun, some sitting around the pool and some sleeeping in should help her feel better.

One interesting phenomenon which I test all the time is whether the doctors seeing Chris know anything about amyloidosis. Neither the emergency room doctor nor the internist on the floor knew anything about it other than "maybe" hearing about it in a lecture in medical school twenty years earlier. In fact the ER doctor stated that Chris really has Multiple Myeloma and not Amyloidosis which we know is completely false. I cannot blame the doctors, they can only know so much, but raising awareness is so critically important.

The lesson learned here is that when she starts to feel a cold or cough coming on, it needs to be treated. She has had such a cold free winter and I think we got comfortable that her cough would pass. Obviously a big mistake that put her in the hospital for a night.

Stephen also has a homecoming today as he completes his 1500 mile ride from FL to MA today. We expect him to be arrving at the Holliston Congregational Church at approx. 2:00 this afternoon with a good crowd to welcome him home. Abby and here friend Kim left earlier this morning to take 3 others out to join Stephen in Windsor Locks and others will be joining the ride as they get closer.

His commitment to raise funds and awareness has been nothing short of spectacular. Yet the help he has received along the way has also been amazing. Donations, sponsorships, blog postings, etc. have have all been very motivating for him. He gets to do the thing he loves (riding his bike) and helps others who have suffered or lost a loved one from this disease. His is a small contribution towards what needs to be done, but he knows he can make a difference by raisng awareness. Today will be a very special day in his life. He is very tired, he aches everywhere, he is mentally spent, but he made a plan months ago and he will complete it in great style. His friends will pull him home today, but figuratively he will be pushing research and awareness. He has met so many people and heard so many stories in the last few weeks, that he is riding with a purpose that will sustain him all the way to the finish.

I'll keep the updates on Chris coming. Getting her to Florida for a few days is the next goal. She has earned it.

Thanks for all the ongoing support and interest, it means more than I can say to Chris.

Back in the hospital...

2009-03-21T07:05:00.003-04:00

Chris has not been feeling well over the last week with a very bad cold/cough and a very congested head. She really started feeling poorly with this bad cold more than a week ago and unfortunately delayed contacting her primary care physician. She finally did last Tuesday and was put on an oral antibiotic, but unfortunately that has not seem to do the trick. Yesterday she went for an evaluation with with her primary and they decided to go ahead and get a chest x-ray. Chris had suddenly started coughing up blood yesterday (Friday) and this and the x-ray caused her to be admitted last night to Newton-Wellesley Hospital. There is nothing serious right now as it is more for observation than anything else, but they wanted to give her a stronger antibiotic and they need to balance all the medications with what she is currently taking.

Her fantastic friend Sally spent much of the afternoon and evening (and even into early morning) with her until I was able to relieve her. I drove back from Hyde Park where I was traveling along with Stephen and Aislinn on their FL to MA ride and got to the hospital around 12:30 am.
Chris seemed to be comfortable, but was tired and still coughing up some blood. I am glad we have her being watched, as I strongly believe in the motto "better safe than sorry". We fully expect that she will be released sometime time today and that the stronger antibiotic kicks in.

Chris has been planning Stephen's return home to Holliston for Sunday afternoon and has things pretty well under control. I know she will be very excited (and relieved) to see him pulling into downtown Holliston on Sunday. Both Aislinn and Stephen have had a fantastic ride, probably a life altering trip, and have met Amyloidosis patients, raised awareness, raised funds for research, and had the adventure of a lifetime. What a journey for them!!!

I'll keep Chris' status updated here, but I do expect her out of the hospital today. I know that nobody could keep her out tomorrow.

Update from BU

2009-03-18T07:43:00.002-04:00

Chris and I heard from BU yesterday and we heard much of what we knew as well as some new good news. Chris' biomarkers continue to go in the right direction, but as I have posted, we are not over the finish line by any means. I won't repeat all the numbers, but they are all looking good. The frustrating one is the lambda light chains kind of stuck at 40. We really want this down the the mid to low 20's and we will feel much better. The good news is that (and this is a very subjective read) is that the her left ventricle wall thickening may have reduced. BU is a currently doing research on the relationship of left ventricle wall thickness and whether there is evidence of actual reduction as patients stop further amyloid protein deposition. In other words, can the heart repair itself? We have asked doctors the various cardiologists we have met with that exact question and the answer is always something like "there is some evidence of that but it is not proven" or "that is exactly what we are trying to understand". Chris' ejection fraction came in at 37% on this reading. We have seen some readings much lower, but 37% is a number she can live a good life with if it can be maintained. What is important is that it does not get worse. Thus my aggressive pursuit of getting to a complete response, even if it means an oral chemo regiment this coming fall. Chris goes back for more blood work on April 28, the full 3 day analysis in the end of June, and then we will know much more.

In the meantime, she has a terrible cold and has had some bad GI issues the last few days. Visiting our friends in DC, she was really very disappointed as she had to lie down and was sick to her stomach often. We are trying to figure out what may have caused her digestive problems, but I am becoming convinced that the Quarter Pounder with fries on the NJ Turnpike did not help. Twice in the last month she has become ill and both times she hit fast food restaurants. This is my fault as we have learned that she can't get to a point of hunger ans when she does, we have to jump on the first food we find. We have been caught off guard when we travel and not prepared with good food. I'll get better at planning.

Her cold is very bad. Certainly the worst she has had since last year. She now is on an anti-biotic, and hopefully this will help soon. Her coughing and hacking and runny nose are very bad, but she is fighting through it.

Chris rode with Aislinn in the car from DC and was amazed at how coordinated Stephen's ride is. Aislinn has this all under control. Yet I know how hard this all is in terms of navigating Stephen's ride. You miss one turn and you can end up being miles off target. I think Aislinn is the unsung hero as she has everything ready to go, thinks ahead, plans for problems, etc. These two teenagers are learning things about managing a large project that I did not understand until well into my business experience. This ride is a potential logistics nightmare, and it is going along almost flawlessly. The focus should be on the awareness and fund raising that they are focused on, and not the ride itself. Aislinn's job is to allow that to happen without problems and she is doing it incredibly well.

I personally plan on riding with Stephen a few more times this week. The ride in DC went well ( no orthopedic surgeries required) and I think I did pretty well at keeping the pace going. The worst part was riding through a tunnel, on a 12" wide sidewalk, against oncoming traffic, with my shoes clipped in to the pedals. As Bill said, "at least I didn't soil myself". Other than that the ride was beautiful and a great deal of fun to be out riding with Stephen.

So overall, some good news from BU. Chris is heading in the right direction, more waiting to do. She needs to focus on exercise and building some muscle mass and conditioning which she will continue when she gets home. We have to get rid of this very bad sinus infection first and hopefully the anti-biotic will kick in today.

I'll post this weekend after we get Stephen and Aislinn back home. Thanks for checking in and caring so much. I can't tell you all how much everyone's support means to Chris and me.

Newspaper article - more awareness

2009-03-13T16:56:00.003-04:00

Here is a link to a newspaper article that was published today down in NC. Aislinn, Andy, and Stephen met with Judi Collins and a reporter and this was the result.
http://www.jdnews.com/news/stephen_62967___article.html/collins_disease.html

One note of interest, Stephen normally does not ride in socks and sandals. I am sure he takes his bike shoes off as frequently as he can.

Day 3 Return Visit

2009-03-11T20:38:00.002-04:00

We met with the Nephrologist (kidney specialist) today and went through some of the numbers that were available. Based on what she had seen come in, Chris' numbers all look good. She went through a good analysis as to how the amyloid proteins limit the kidney function and how they allow protein to get into the urine. Protein leaving the body is generally bad and an indication of amyloidosis, along with other causes. One of the indicators of protein in the urine is "foamy" urine that is very bubbly. It can also be an early indicator of diabetes so it should always be analyzed if there is foaminess (sorry to be so focused on urine tonight). The other biomarker is the amount of creatinine. Creatinine is the waste by-product of muscles and if too high indicates renal damage. Chris' number is .64 and the normal range is .5 to 1.0. So again this is very good. The doctor also expected that the 24 hour protein number would continue to decrease over time.

What we need is the total package of results which we will not have until next week. The normal procedure is that we get the call on Friday afternoon with the next steps. For this time, we will not hear until Tuesday or Wednesday.

They do want Chris to do another set of blood test (not the doctor visits) and she will do that on April 28th. Then she has her 12 month evaluation at the end of June. What we heard today was that it would not be until the June meeting that they declare what kind of response it was (complete or partial) and what the next steps are. So this is going to be slower than I would like but they need the full year to understand the total affect of the stem cell replant and chemo.

So, as I said yesterday, we are running in the right direction, but we do not know if and when we will cross the complete response finish line.

One thing that is really good is that the Director of the BU Amyloidosis Research Clinic asked us to meet with her in her office. She said that there had been a lot of talk about what our son was doing on his bike ride around the center and she wanted to tell us how amazed they are and supportive they want to be. They have invited Stephen and Aislinn to come and get the "VIP" tour of the center, meet with the doctors, see the labs, etc. I am wondering if maybe they might give a quick presentation to the medical staff on a Friday when they all meet. They will have great pictures and video's (and will get more there) and it would be a great dry-run for their presentation back at Worcester Academy.

So no real news until next week when they call with their final packaged results. We are pleased with the direction but would like to have all the biomarkers at or beyond the target. We are not there yet.

Your donations to the Research Foundation keep coming in. Thanks for all your support, your continued calls to Chris, your prayers and well wishes. We both believe that they are making a huge difference. In the meantime, Aislinn and Stephen march north. They have some great stories to tell, so their blog will be much more interesting for the next few days than mine.

More next week.

Day 2 Return Visit

2009-03-10T19:19:00.002-04:00

More tests today and some relatively good news. Chris had another echocardiagram today ( I think this is about the 8th one in the last nine months). We have learned that reading these is clearly very subjective so each person that analyzes the echo measures, analyzes, and sees different things. Thus it is most important to a) have progressive readings over time and b) have the same person do it. In our case, we are most comfortable with Dr. Falk as he has so much experience and has written so much based on his own research. We will have todays test sent prior to when Chris meets with him in April.

We also participated in the weekly support group meeting. I have written about this before. There are many stories told around the table; people who had a stem cewll transplant six years ago to those who are being evaluated for the first time. I have attended many of these and I know all too well the feeling of being overwhelmed by the words, the science, the numbers, all this stuff that people talk about and as a new participant you just sit like a log with an internal fear that is eating you up. There has to be a better way to help the newcomers than exists today. Just like we were, they are scared to death, they are trying to understand what on earth is going on, and they bombarded with all these tests and words, and complex things that just spin around and want to make your head blow up. Chris and I were shaking as we drove home after our first few days. This can be done better, I am sure. It is very clear that the strength, the willingness to learn, and the organization of the caregiver/advocate is probably as important as anything. I have seen caregivers who love their spouses or family members dearly but in no way are they capable of navigating the maze. They are conmforting, they hold their spouses close, but they are not prepared to be advocates, at all. Yet today I saw others who were ready for the battle. They asked a ton of questions, listend very intently, asked more questions, and learned from the others who went first. They are right on top of things, keep every test result, and do their own research. They will be both great caregivers AND great patient advocates.

More specifically for Chris, here are the numbers we have and my thoughts:

Lambda Light Chains: June '08=> 600+, December '08=> 40, March '09=> 40

This means no change in the amount of lambda light chains in her body. Good news is no increase, bad news is we are not at the target yet, which is under 21. This could be better, and needs to be.

Protein - 24 Hour Urine collection: June '08=>3800, December '08=>2800, March '09=>1800

This is clear continued improvement and of course is trending in the right direction. It is expected that this will continue to improve over the next few months. The target is about 150, so while there is clear improvement, there is more to go, which should happen.

Kappa/Lambda ratio: June '08 =>N/A (off the chart). Dec '08=> .2, March '09=> .29
This is very good, the target is above .25 and she is there. There are many ways to analyze the ratio, but when the lambda number is going up and the ratio is going up, this is a good thing.

BNP: June '08=> 269, Dec '08=> 286, March '09=> 204
This is the measure of congestive heart failure (CFH). Anything above 100 is a sign of some CFH, but Chris' is going down. This is good. The higher the BNP, the lower the cardiac output and the lower the blood volume pumped. This is trending down so we are pleased.

There are just some of the many tests, but here is how I sum this up:

Things are progressing in the right direction, they are generally getting better, but we are not over the finish line of a Hematologic Complete Response (the famous HCR). We want to reach the HCR objective. Tomorrow we do some more test, primarily kidney function, and then we will get our final report next week. They have already told us that they will want her back for blood work (not all the evaluations) in 4-6 weeks.

I have told them that when they think of Chris, they need to think of one word; aggressive. I do not want further protein deposition and we know how important getting an HCR is to life expectancy. I get the sense that some of the doctors at BU are almost saying "it's really good, stop worrying". Not me, I will only be comfortable when I hear the doctors say " congratulations, you have a complete response".

If Chris does need another round of chemo, we all agree that it would be after the summer. We lost the entire summer last year and we want to enjoy one this coming season. Chris would start in September. But, let's see what the final report says when we get it next week.

Another update tomorrow I hope. Thanks for checking in, it means a great deal to us.

Day 1 at Boston Medical return visit

2009-03-09T21:20:00.002-04:00

Things went well at BU today, for the little we learned. Chris had the 24 hour urine collection, lots and lots of blood taken, and a chest x-ray. We did not get all the results of course, but the chest x-ray results were very good. For the first time in over a year, Chris did not have any fluid in her lungs. This is really encouraging and we were glad to here it. We did meet with two doctors, one for a general review of her overall health, and then the hematologist, Dr. Sanchorawala who we both have a great deal of respect for and who runs the stem cell transplant program. She was very positive on Chris' progress, but we still need to see the results of the lambda light chains. This is the big number we are waiting to see as it will be an important determiner of the next plan of attack.

We discussed all of the various chemo options: Velcade, Revlimid, and possibly a combination of Velcade and Melphalan with dexemethesone. There is a possibility that we may have to go back in a month for more blood work to just track the data points. That may be when a final decision is made. I of course want to be as aggressive as possible and I have told every doctor I see that same thing. I am not happy with the 94% response, and Chris agrees, so we need to get 100% and pursue all potential options. We just won't know those options for awhile I guess.

The doctors were all fully aware of Stephen's ride. They have been very impressed and tell us that we must be very proud. Of course we are. There is a computer at the center and everyone was reading his web site and congratulating us.

We were very glad to spend some time with our friends from Ohio. Tim went through the stem cell transplant a month before Chris and is doing very well. He is now on an oral dose of chemo and we are learning from him all of the good and the bad. It is clearly tough on the body, but Tim is soldiering through it very well. Tim and his wife Connie have started a foundation where they will own an apartment in Boston and give it to Amyloidosis patients who are in need of financial support for their living expense while staying in Boston. Connie has this all planned out but is in need of some organization/coordination/management support from someone in Boston. Chris is very interested in helping as she has had such great support from so many people here in Holliston and donations from people all over the US. This could be a really win/win /win situation. Connie and Tim start a fantastic foundation, those in need of help get it, and Chris gets to give back (or pass forward) all the help she has received.

So bottom line, we don't know much yet, but hopefully we'll know a bit more tomorrow. Thanks so much for your interest and incredible support.

Some important days coming up....

2009-03-08T19:51:00.002-04:00

Chris and I go in to Boston tomorrow for three days of tests tomorrow. Effectively, we will learn two things, 1) what is the current status of her amyloidosis (specifically her amyloid protein production) and 2) what the course of treatment will be. For the first question, it could be anything from worsening, to being stable, to improving. Since her response was a 94% reduction back in January, it would be great to see a total response. This would mean no second round of chemo, no further treatment other than continued medicines she is on now. If there has been no improvement, then I am sure she will have to do a second round of chemo this spring.
The process will be the same as before. Three days of tests on her heart, kidney, liver, lungs, etc. then a medical team conference on Friday followed by the famous call to us saying what the prognosis and course of action is. Chris and I are both getting a little nervous and it will hit us hard when we get there in the morning. As I have said many times, we will pray for the best, plan for the worst.

The outpouring of generosity to Stephen's quest to raise awareness and help further the level of research has been absolutely amazing. I do not know hardly any of the details of the contributions as Stephen is keeping this close to the vest, but I can tell you that many many people have been more than generous. Stephen has been amazed at the response. He is keeping a list of all of the donors and will be sending a thank you note upon is return. Everyone has been just incredibly generous in this economy. People have donated signs, shirts, biking equipment, housing, and have even hosted cocktail parties to raise money - just amazing.

I have spoken to a few people who have asked how he is doing. He has finished day two with great progress and many stories already. He and Aislin will have some incredible experiences to share when they get back to Worcester Academy. He does update his blog late every night at
www.chrisjourney.com and he is also sending quick updates during the day form his phone at www.twitter.com/chrisjourney . We can actually track his progress real time using a combination of igoogle and a blackberry. One thing that I have learned from his ride is the value of technology for communication. Between blogs and Twitter and igoogle, it is really easy to stay connected.

Chris and I cannot thank you all enough. The Holliston community, Worcester Academy friends, IBMers, people who have lost loved ones or have suffered themselves, the prayers, the love, the generosity, it is all beyond belief. It is impossible to properly thank you all.

Please continue the prayers for success this week, both for Chris and Stephen.

On their way to raise awareness......

2009-03-06T20:29:00.005-05:00

A great send-off from the Worcester Academy students, teachers, parents, friends, and family. Many signs, hugs, and well wishes as they headed off. I won't post many pictures here as Stephen will be posting on the http://www.chrisjourney.com/ site with his daily updates.

Chris, Stephen, and Aislinn at the party. It meant a great deal to us to see such a great turnout.

Clicking on the picture should give a better view.

More Awareness for Amyloidosis

2009-03-04T18:41:00.004-05:00

As I have posted here earlier, one of the most terrible things about amyloidosis is that it goes undiagnosed in so many people; they suffer the terrible consequences due to the lack of awareness. Chris was made aware from the pure luck of reading the newspaper article written about Jennifer Gershman, who was diagnosed a year before Chris and was also treated at Boston Medical. Jennifer promoted awareness and Stephen has continued the march with his east cost bike ride from Florida to Boston this month. Interestingly, this project that he and Aislinn Shea are doing for their senior project has benefited from some "viral marketing". That basically means that the story is spreading through word of mouth or other media without any organized or intended approach.

Today was a good example. The NBC affiliate in Boston, WBZ, interviewed Stephen and Chris today and ran a story on the news this evening. Here is the link:
http://wbztv.com/video/?id=73734@wbz.dayport.com

Here is their web site posting:
http://wbztv.com/health/east.coast.ride.2.950441.html

Also, the paper in Worcester picked up the story and ran it in today's Telegram and Gazette:
http://www.telegram.com/article/20090304/NEWS/903040449/1011

Chris is a bit worried that this is getting bigger than she wanted, but if one person is made aware or one doctor thinks of testing for this, then it is worth it all.

Stephen and Aislinn leave tomorrow for Florida from Worcester Academy. I'll try to post some pictures if at all possible on Friday.

A good and quick explanation promoting awareness

2009-03-02T17:59:00.002-05:00

The Amyloidosis Foundation has sponsored a 5 minute video that was developed to explain Amyloidosis to physicians. It gets a little technical, but the key elements are understandable. Chris has Primary AL type, and you can hear some frightening statistics about life expectancy and number of sufferers. As the video points out, the single most important factor in successful treatment is early diagnosis. Yet diagnosis is unlikely given the lack of awareness by the medical community. As stated, often the patient suffers from many different symptoms and numerous tests are often performed without any clear pointer. In the case of amyloidosis, the only real test is a fat biopsy with a red congo stain.

Here is the url for the video:
http://vimeo.com/3358025

In the meantime, Chris is doing well but has had a very bad cold and some GI issues in the last week. Unfortunately we are learning that we just can't grab over the counter medicines to treat her symptoms as everything needs to be analyzed against the Coumadin. This slows everything down and she suffers a bit longer as a result. We want her to be as healthy as possible next week as we go in for the 3 days of evaluation.

Stephen continues to train hard for his ride. He and Aislinn leave on Thursday directly from school and will arrive in Jacksonville Friday evening with the ride north starting Saturday morning from the Mayo Clinic. They are pretty well organized, but I am sure they will have some bumps in the road. His plan is to update his blog each evening with a quick status of his location, his adventures, and his progress promoting awareness. He remains amazed as to the outpouring of support for his ride to promote awareness.

Again, thanks to all!!!

Some Good Milestones - mid February

2009-02-23T08:25:00.004-05:00

A long overdue update. My only defense is that when there is not any significant news, I don't want to bore anyone with the mundane. Yet, I know there is so much support and interest that providing updates is important.

In the last few weeks, Chris has really "broken out" as they say. She has been going back to her personal trainer at the gym she went to prior to her diagnosis and has started a series of sessions where she will focus on her strength, building back some muscle mass, and some minor conditioning. This was an objective of hers as part of her recovery and I know that she is very happy she is back doing this. When you combine this with her regular walks and her yoga classes, it is clear she is really getting back into the swing of things. After a few more months, the next physical activity objective is to get back on the tennis court. That will have a special meaning in many ways as it is where she was when her doctor called with the bad news. I have always said we need to be back hitting balls prior to that anniversary, and I am sure she will do it.

Another milestone was getting back to church. Chris has tried to be very cautious about being in large crowds where people have potential colds and flu's and lot's of handshaking. With no immunity system, this has been a concern. But she really wanted to go and was prepared for any bugs she might pick up. Of course when she got there she was given lots of hugs by many people who were so excited to see her and she was shaking many many hands. She got home, washed her hands, and never got a thing. She was very glad to get back there for lot's of reasons.

This gave her some confidence to go to NY this past weekend for Abby's NCAA swim meet championship. We drove to Syracuse and went to the meet every morning and evening with a very large crowd, all packed in tight, with many people using door knob's, hand railings, etc. A few months ago Chris would never have attempted this, as the risk would be to high, but she did great, meeting many people. I in fact had a bad cold and did not want to risk giving it to others so I did not shake hands, while Chris was doing it for me. What a great role reversal. By the way, Abby did fantastic and was very happy.

Melissa has been calling from Australia and giving us the updates on her new escapades. Chris loves to hear the stories of her adventures in New Zealand and getting settled in Perth. I know Chris is anxious to start travelling again and hopefully will get to Florida in the next month or so after our visit to BU Medical in a few weeks. By the way, Melissa loves Perth.

So while I have been trying to stay out of the way for Stephen's bike ride, I can attest that it is coming together well. His friend, Aislinn Shea has been organizing the routes, the houses and hotels they will stay at, and the overall logistics. Stephen has been focusing on getting into shape with long rides on weekends and many hours in the basement training. Doing 100+ miles every day, for 15 days will be an incredible challenge and take a real toll on his body, but he is up for it. Aislinn will be critical in keeping the momentum going once they get started. Chris and I will be at our three days of tests in Boston while they are on week 1, so it will be a busy week for amyloidosis activity.

As I said, Stephen and Aislinn do not let Chris and I get very involved in his fund raising efforts for the research foundation, and thus I know very little. What I do know is that many many people have donated and it is incredible to see. I do not know the amounts and that is not important to me, but what is important is that he has many individuals contributing. Friends at IBM have been fantastic, friends from across town and nearby, friends of Stephen at school, it is just amazing. However, the ones that Stephen does tell me about are the ones with a letter attached to the donation. Many of them are from families who have lost loved ones to amyloidosis, or who have personally fought the battle themselves and are doing all they can to help find a cure. The stories are gut wrenching sometimes, but that is why Stephen is doing this ride. He knows he had a very ill mother, and he knows she has done very well so far, but he is painfully aware that others have lost husbands, fathers, mothers, wives, sisters and brothers. Creating awareness and finding a cure are what he and Aislinn doing this trip for.

In the meantime we will prepare for the BU appointments March 9, 10, 11. We could get great news (normal lambda light chain levels) or very bad news, but we will go in praying for the best and preparing for the worst.

I had not realized how many other amyloidosis patients read this blog, but based on the donations and the links to other sites, it is clear there are many. I can tell you, although you have not met Chris, she reads your blogs as faithfully. We both pray for you all, Amy, Kevin, Debbie, etc. We share your losses, your progress, your success.

Promoting Awareness

2009-02-03T08:31:00.002-05:00

With amyloidosis being such a rare but brutal disease, the fact that it is so misunderstood and diagnosis is often delayed beyond treatment means that promoting awareness is critical. Chris and I remain incredibly lucky that she read the news article about Jenifer Gershman in the newspaper. http://www.metrowestdailynews.com/archive/x1853598606 It pointed Chris to the diagnosis and allowed us a strong treatment. Everyone we speak to who suffers from this disease is amazed that Chris went from her first significant symptoms in January to diagnosis in April, to stem cell transplant/chemo in June. We were very aggressive about testing and treatment, but very lucky as well. Jenifer made a commitment to promote awareness with the hope that she could help others with an early diagnosis, and with Chris she succeeded. Now it is time to help Jenifer, and that is one of the reasons Stephen has decided to do the 1500 mile bike ride. This will be an incredible accomplishment, but I am sure he will pull it off, even if he rides in snow storms in the night. But even more incredible is the response he has received from so many people. It has blown him and us away at the generosity and willingness to help to raise this money for the Amyloidosis Research Foundation. He will be updating the status of things as this is completely his initiative, but suffice it to say the outpouring has been fantastic so far. He has tremendous support from sponsors from diverse as bike retailers to cookie companies to landscape architects. The sponsors then help him raise the money by supporting the cost of the ride itself. Stephen will of course give 100% of the donations to the foundation. Those donations are coming in daily in the mail or on the foundation web site. I cannot tell you how very much appreciated these are. Some are small and some are big, but Stephen just can't believe the number of donors. Even his friends at school have been raising money through various events. Just amazing. Stephen is as much interested in the number of donors as the amount, as it means that someone was willing to spend the few minutes it took to help promote awareness of Chris' disease. Thus, even small donations are very appreciated as it means one more person helps fight the cause.

Stephen has had some good press on his campaign. The regional newspaper came by and interviewed Chris and Stephen for their Sunday edition. Here is the link:
http://www.metrowestdailynews.com/archive/x1556828605/Spitz-Son-is-riding-for-moms-life

Chris continues to work hard at regaining her strength and fitness. Unfortunately the bad weather does not encourage much outdoor exercise. Her aerobic fitness is very important to her heart, so she tries when she can, but still feels very winded with her reduced heart function. Her cocktail of pills continues morning and night, but she has not complained about it. I am becoming more convinced based on conversations with doctors and my own knowledge of the disease that Chris will be doing oral chemo this spring or summer. We need to attack the remaining proteins (lamda light chains) still being produced and prevent any further deposition in her organs, especially her heart. The March visit will be the point where we decide the next steps. Chris keeps saying, "I just want to know where I stand", as we feel like we have more to do but no definitive action plan. But in about 4 weeks we will know, just as Stephen starts his ride.

Again here is the link to his site: http://chrisjourney.com/

Thanks to all.

A very brief photo history: To Hell and Back

2009-01-30T21:26:00.012-05:00

Chris and her sister Debbie in August of 2007 in NH. We had no idea what the next 12 months would bring at this point.

June 2008 - Getting ready to start the chemo. It took about 15 minutes to take it all. Notice the "pic" line inserted in her chest. The chemo was given via the line. The chemo is so strong that it can burn through veins close to the skin, thus Chris had to keep ice packed in her mouth for about 20 minutes to avoid severe sores. This is the equivalent of 6 months worth of oral chemo in two direct shots over 2 days.

The actual chemo, melphalan, which was given via the pic line (catheter) that had been inserted into her chest for about 6 weeks. This is the stuff that almost kills you but in the end is your best friend. Chris took the maximum (but highest risk) dose.

One of two bags, each containing 5 million stem cells. These are collected in a process similar to dialysis and separated with a centrifuge. The stems cells are collected prior to the chemo, then replanted a few days afterward.

They very carefully wrapped her stem cells up and transported them in this box to the blood bank for freezing. They can be stored for 10 years. In her case they replanted half of them back into her bone marrow. The other half, about 5 million are still at BU.

July in Boston, about 21 days after the chemo. Chris was under 95 pounds and unable to walk more than about 10 feet at a time.

On the deck in NH in August. Very frail, but happy to be done with the treatment and very slowly starting to feel a bit normal again.

Chris with Stephen, January 2009.

Another Echo today

2009-01-27T19:12:00.003-05:00

Chris had another Echocardiagram today with Dr Falk to get his most recent perspective on her heart status. He compared the results with the November echo and saw no change, either good or bad. He is waiting to get the May echo from BU and then will compare that to the one today to see what has really occurred over the last 8 months or so. This will give us the widest possible spread of dates and provide an indication of how things have changed. The May echo was her first. So really not any significant news other then we remain concerned about the overall status of her heart and its ability to keep up with an active lifestyle.

Chris did go to Yoga again and she really feels like this is good for her. She complains that she feels very tight and yet she is pleased with her strength. Hopefully she will keep this up, as well as more cardio exercise. The weather has been very bad here and this limits the outside walks of course.

Last week I was in Rochester, MN for a business meeting and I made a trip to the Mayo Clinic which is really the only other amyloidosis center in the world, or at least the US. Rochester is a very funny place as it is about 90 miles from Minn./St Paul and really has two major employers: Mayo and IBM. The Mayo clinic of course is world renowned for it's expertise and incredible staff, but you have to wonder how it ended up in Rochester. The facility itself is amazing. I am not kidding when I say that the lobby is a beautiful three story atrium and there is a man in a tux playing a grand piano. I felt as if I was in the Four Seasons hotel. It must be hard for those without health insurance or needing to cover ongoing medical bills to see this use of money, but it certainly was an incredible place. What I really wanted to see was the Amyloidosis Center, just to see what it looked like compared to BU. We had heard of many patients who had been to Mayo for clinics or evaluation and thus it would be interesting to see it. I asked at the front desk and of course they had never heard of amyloidosis and had no idea where to direct me. Then I asked where the Hematology dept was and they said the 10th floor which I then proceeded to go investigate.

I was looking for a picture of the Center, just a nameplate or a logo or something that indicated Mayo and Amyloidosis as I wanted to give it to Stephen for his fund raiser web site (http://chrisjourney.com/) A woman that worked in the Hematology dept asked if she could help (as I wandered around aimlessly) and I explained what I was looking for. She went back to the inner sanctum of the hospital and returned saying that she had asked one of the doctors, Dr. Morie Gertz, if he knew of a sign or poster or something that I could take a picture of and the answer was simply "nope, nothing like that". Oh well. Interestingly as she mentioned Dr. Gertz' name, it hit me that I had read many many publications of his in my quest to be the expert on this disease. I brazenly asked if I could quickly meet him, just to say hello and tell him how appreciative I was of his work (and maybe get a quote for Stephen's bike ride). She went back to ask and returned with a curt "he is unavailable". My intentions were very honorable, but oh well, it just did not work out.

So our next major step is to hear about the comparison of May's echo with the one today with Dr Falk. Ideally we will get some important insight into the real level of her ejection fraction. This is a big frustration to Chris and I as we really do not have consistent answers. Hopefully we will learn more in the next few days.

Chris has recently heard from a number of friends who had not heard of her illness. She is really glad to hear from them and as always I encourage everyone to please feel free to call her. The support from all just continues to energize her. Thanks to all who have reached out in so many ways.

Brief Update on the Cardiologist meeting

2009-01-23T12:06:00.002-05:00

Well, it is hard to say that Chris and I walked away with some incredible earth shaking news from the appointment with Dr Falk yesterday. We spent almost an hour with him reviewing his analysis, what the results show, next steps, etc. At the end, I asked him to summarize all of our discussions in just a few words. and he said, as he counted the words on his fingers: "I think she is doing very well".

Chris and I don't know exactly how to react to that. He did indicate that there has in fact been some decline in the heart function, as measured by the ejection fraction. He does not believe it is nearly as bad as the most recent BU analysis says, but he thinks there is some detioration since May. He did not know if that was due to further amyloid deposition in her left ventricle, or possibly the result of the high dose chemo itself. What confuses him (or as he says is "a contraindication") is that all of her other organs have responded so well to the treatment. Like Dr. Sanchorawala at BU, he says she clearly has had a very good partial response, but not the complete response we were all praying for. As such we asked him the likely next steps and he laid out pretty much the same 4 options we knew. 1) Another stem cell with high dose chemo, 2) oral Velcade, 3) oral Revlimid and 4) do nothing. Of course option 4 is not an option for me, so we ignore that. He doubts that any incremental benefits of the stem cell/chemo approach would be worth the risk due to her heart condition (the benefit being negligible vs a higher risk for cardiac involved patients). He thought the the oral Velcade would be his choice but he was clear to point out that he trusts BU and in the end that is a call they (and us) need to make.

He does want another (yes, yet another) echo next week. That way, they will have one more data point. One from BU in May, Dr Falk in November, BU again in December, and Dr Falk again in January. As echo's cause no radiation, I think getting more history for analysis is the right thing to do.

We did discuss mitral valve leakage and he stated that while there is probably some there, it was not a huge issue and there would be no action to take even if it was, as surgery would not be viable.

Chris asked the burning question "Can I ski ?". He was not very positive on that as he was afraid that any potential low blood pressure might cause her to faint and hit a tree. It was not a concern about stress on the heart from skiing, but a concern about fainting. Nevertheless, Chris and I did agree that on a beautiful sunny, non crowded day in March, we will in fact go ahead and ski. Chris enjoys skiing, is very good at it, and the hell with it, if we take it easy we can have a great day. So look for us in March on the slopes. I am not denying her of this and we set it as a goal.

Chris will get the results of next weeks echo right away, so we will have new news next week. I also need to add pictures here as I promised (but Stephen has beaten me to the punch on his 1500 mile ride for amyloidosis web site). Thanks for checking in and caring so much about Chris. As the doctor said, "She is doing great", but it just does not seem like we are yet able to close the book on this journey to good health. We are getting there however, I am very optimistic.

Please feel free to call Chris, she would love to hear from you and it is important for her to talk.
Pictures soon.

A small minor point: I am writing this post from Rochester MN, home of the other Amyloidosis Center. If I get a chance I may stop in to see the center.

1500 Miles (click here)

2009-01-19T10:20:00.004-05:00

All of our children have been the best possible supporters of Chris throughout her journey to health. They have sacrificed a great deal to ensure that they can help Chris however she needs it; around the house, giving up activities, running errands, and keeping things going at home while Chris went through her Chemo in Boston.

Fortunately, our 17 year old son Stephen has a unique opportunity to help in another way. During a 2 week spring-break in March, he and some of his close friends have organized a fund-raiser with all of the proceeds going to the Amyloidosis Research Foundation. He and others (sponsors, friends) will bear all of the expenses ensuring that every dollar that is donated will go directly to the foundation. His ride will take him from Florida to Massachusetts over a 2 week period. This will mean he will need to cycle an average of over 115 miles everyday for 14 days - an incredible challenge. His goal is to raise $10,000 during this ride while promoting awareness.

A link to the web site he has created is on the right side of the blog. He is anxious to get a strong start toward his goal, and has already lined up sponsors who will support the ride. Any donation of any value will be greatly appreciated. This is fully tax deductible and will represent a very important donation towards the fight against Amyloidosis. Chris has been extremely fortunate that we have the medical care, the financial resources, a supportive community, and amazing friends who have all been an important part of Chris' journey. What Stephen wants do to is to help others who may not be so fortunate in their fight. Chris continues to fight, and we are sure she will win, but their are many others who can be helped by awareness, research, treatment, and support. This is his objective - not bad for a 17 year old kid.

Feel free to contact him with questions at: stephen@chrisjourney.com and please follow his blog as he prepares and then completes the ride. Chris and I thank you very much.

Doing Well - Waiting for 1/22 Cardiac Appt

2009-01-17T12:07:00.002-05:00

Chris has had a good couple of weeks. She has been busy with various trips to Vermont and NY, putting away all the Christmas things, getting back to yoga, etc. She really was glad to start the yoga again and she said she felt stronger than she expected. This was encouraging and I expect she will continue this and soon get back to the gym where she was a regular less than a year ago. The yoga is also important for her mental health (or so she tells me), but I hope I don't come home some day and find a Yogi humming in our living room.

We received the full report from our BU visit back in mid-December. The entire report is 40+ pages as all of the results are included from the lung, heart, kidney, blood and liver tests. I will map the data against the trend charts as a measure of ongoing improvement. I thought I would include some comments from the summary here as an example of the results. These are all quotes:

-On physical examination, Mrs Wright looked well. She was aferbrile with a standing heart rate of 104 bpm and a corresponding blood pressure of 92/61 (mmHg). She was not orthostatic. HEENT exam revlealed no submandibular gland enlargement.

-Cardiac auscultation revelaed a regular rate and rythm without murmer.

-Pulmonary auscultation revelaed mild bibasilar crackles.

-Mrs. Wright underwant numerous diagnostic tests during the course of her eevaluation with us. Serum immunofixation revealed a polyclonal distribtion with a persistent, but fainter IgM lambda band. A pevious M-spike of 0.42 has now essentially resolved.

-Transthoracis echocardiogram notes worsening of left ventricular ejection fraction at 35% with the suggestion of worsened mitral regurgitation and persistent biatrial enlargement. Dr. (I will leave the name out here) noted her echo findings seemed contradictory to her improved functional status with decreasing need for medication.

-Dr. Sanchorawala noted that Mrs. Wright has had a great partial response of her underlying plasma cell dyscrasia and evidence of protein response with a reduction in proteinuria, BNP, diuretic requirement, and improves tolerance. Dr. Sanchorawala advises no additional treatment is needed at this time; however, she did request that Mrs. Wright return to clinic in 3 months (March 8-10) for reevaluation to include serum free light chain analysis.

Imagine 40 pages of this. So what does this all mean? Here is where I think we are as of Jan. 17.

Chris clearly has had a very good response to the chemo therapy. The amyloid proteins in both her blood and her urine are down significantly, yet she has not had the Complete hematological response we were all hoping for. Yet, she is 95% there. She is really feeling very good and her stamina, attitude, and outlook are all positive. She is getting herself into a normal routine and really feeling good about things. The worry however is clearly the heart. She is formally in congestive heart failure (you would never know it) and we have the entire spectrum of numbers around the ejection fraction - ranging from 50% to 20% ( normal is 55%-75%). As such, our next major gate is meeting with Dr. Falk, the amyloidosis cardiologist specialist on Jan 22. We have sent him the actual CD of the echo from BU and he will review that vs his own offices tests and the one done at Brigham and Women's and see what the heck is the real truth.

Chris has restarted the Coumadin (blood thinner) and has had no adverse reaction, unlike the first attempt. I now blame that reaction to Thai food (so thankfully we won't be going back to that restaurant soon). We'll see what Dr. Falk says about the implications for using this for the rest of her life, but I anticipate that is going to be the reality as it is needed to prevent a stroke from a thrombosis in her heart.

Stephen has been working very diligently on his required senior high school project which is to raise funds and awareness for Amyloidosis. He has 2 weeks off from school in March and his plan is to ride from Florida to Massachusetts. He is developing a web site, mapping the ride, creating a budget, etc. He has contacted the Amyloidosis Research Foundation as well as local sponsors - all on his own. Chris and I are very impressed with his passion and determination. He has asked for no help at all from us as he really wants to do this on his own. I am asking if I can at least join his support crew - he'll "think about it". His plan is to do this in the first 2 weeks of March, so time is now critical to get the plan locked, but I am sure he will make this happen. I will be posting a link to his web site in the next week or so.

I also have permission from Chris to post some pictures of her over the last few months. Many of her distantly located friends have been asking for pictures and she has always said no, but now she feels as if it might make people more aware if she can show them how you can come back from the hell of the high dose chemo. So I will look for some and post them here in the next week. She will of course maintain editorial control she warns me.

Again my apologies for my delayed blog updates. I have started a new ( and very exciting) job at IBM and am in the middle of reshuffling organizations. Yet job #1 remains Chris. I will certainly post an update after the cardiac appointment and hopefully post some pictures in the next few days.

A million thanks for your ongoing interest and support. I can't tell you how important it is.

Lessons Learned from Chris' Journey through 2008

2009-01-04T21:25:00.000-05:00

With 2008 gone, and our optimism for a much better 2009 as high as ever, I have thought for awhile now that it would be important to share my insights, perspectives, recommendations, and warnings for others who may be face a similar circumstance, I will try not to be too verbose (yeah right) but convey the essence of the thought. Some of these may be only applicable to Amyloidosis, but I am trying to think of them more generally. So here goes:

-If your family member, colleuege, neighbor, whomever says, "I'm just not feeling right" or "something is wrong but I can't tell you what" and this goes on for an extended period (more than a few weeks), get it checked out. Don't write it off to old age or work stress or a low-grade fever. I am convinced the body sends the mind clear messages that attention is needed. If something isn't feeling right, then do somehting about it. We all want to fight the battle, keep moving, go to work, etc. We don't have time for the Doctor. Lesson: If you don't feel well for more than a few weeks, see a doctor. Time may be very important. Many Amyloid patients miss their opportunity for treatment because they were not diagnosed early enough. They die from a lack of a diagnosis.

-Get a second opinion on anything more than the basics. As I have stated many times, medicine is an art not a science. What one doctor misses, the other may see. Every doctor has a different set of experiences and training. They use their judgement based on what they know. The experts based at the BU center believe that >95% of primary care physicians have never diagnosed nor treated Amyloidosis and would not even think of testing for it. Maybe they read about it in Medical School. This is why the majority of sufferers die from the disease before treatment. Doctors don't see it, they don't know what too look for, and they don't even think to test for it. A great book to read if your looking for something on this topic is a book I happened to have read about a year ago. It is very interesting reading for a caregiver or advocate:
http://www.amazon.com/How-Doctors-Think-Jerome-Groopman/dp/0618610030 (sorry I don't mean to promote Amazon). I strongly recommend it before a crisis hits and I am sure it shaped my own thinking as Chris' advocate.

Have an annual physical with complete blood and urine tests. The body is filled with bio-markers which are indicators of chemical balance. I had no idea how complicated the chemical make-up of the human body is until I looked at a 5 page blood analysis. We have every metric on Chris since May and each one tells a story: glucose, potassium, protein, lambda light chains, albumin, creatinine, etc, etc...... Always have complete blood work and a urinalysis at every checkup. and always have it if you are going in if you don't feel well. Be aggressive about getting these done, they are not needless tests, they are critical chemical indicators. Chris had a urine test, which then led to a 24 hour urine analysis, which then led to an indication of an immuniglobulin M-spike, which then led to a bone marrow biopsy, and then a fat-pad biopsy that was tested with the famous red congo stain confirming Primary Amyloidosis. Without the first urine test, the whole sequence would have been delayed and the diagnosis may never have happened.

Become an expert. Whether it is heart disease, lung cancer, pneumonia, or any strange disease nobody has ever heard of, the resources are there to become an expert. The more you know, the more you become a participant in the discussion with the medical team vs an observer to the process. You can challenge, ask intelligent questions, seek out specialists, manage the drugs, demand tests, and potentially avoid problems. I now know drug interactions, I know all clinical trials by drug companies, I know the latest testing techniques, and I have read almost every paper written in the last few years on the subject. I use Google Alerts to scan the Internet for all mention of Amyloidosis as well as any publications from selected experts in the field. I get a weekly update on all new items about the disease, including new trials, published studies, conferences, etc. I suggest reading all recently published works. In our case it comes form magazines such as Blood, Journal of the British Hematological Society, etc.

Document and get a copy of everything. I have a copy of every single test result Chris has had. I have a copy of every report written by every doctor. I have the daily blood tests results as well as the graphs of the trends over the entire period. These are documents that are your right to have as they are about you or the patient you are advocating for. I used to think there was some special underground/behind the scenes data that only the medical experts had access to. In fact you have rights to everything. There are many benefits to this:
-You can become smarter by looking up every term used, understanding every test result, and getting a very well documented position of the doctors opinion.
-You can make these available to other doctors who need to know the background, especially those providing a second opinion.
-You might be able to avoid tests. Chris has had many many lung x-rays. I am concerned about over use of these. With a copy of the x-rays, I simply hand them to another doctor and avoid the tests. As I have learned, hospitals operate as members of competitive organizations. They often network well inside of their partnership, but not well outside to other partnerships.

Hospitals are very infectious places. One surprising lesson I have learned is how hospitals are really filled with germs. Chris' immunity system was completely wiped out, and they did not want to have her stay in the hospital for fear of infection. Some hospitals do the stem cell transplant and high-dose chemo as in-patient. Boston Medical insists that all patients live off-site, but close. The doctors strongly believe that they have a lower mortality rate simply due to the number one cause of death for these patients - infections. I am not advocating that patients avoid the hospital, but I was surprised at how many stories I heard about people getting very serious infections in the hospital such as MRSA, staff infections, etc.

There are two critical roles: Caregiver and Advocate. These can be the same person or two different people, but they both need to be in place. The caregiver is focused on the patient and his/her comfort. All seriously ill patients need a caregiver, even if at the hospital. Nurses have many patients to worry about cannot give the level of comfort or attention that an ill patient really needs. The caregiver needs to do a virtual brain transplant and think in the mind of the patient. What would make them more comfortable, who might they be worrying about, what are they feeling? The caregiver needs to swallow their pride completely. As Steve G, husband of Jen (the couple whom we read about in the paper back in May) told me," be prepared to wear a loincloth, you will become a slave and will need to swallow all pride and be a servant". This is a tough and frustrating job. You may be asked to get something very small from a sound sleep, you may have to clean up some really nasty stuff, you may be sent to the store to get something only to find it was the wrong thing. My suggestion is to just acknowledge that you are there to serve. It is not about you, it is totally, 100% about the patient. You get very little sympathy, very little acknowledgement, and few people understand what you are personally going through. Suck it up, be tough, and focus only on the patient. You will have to run on the internal pride you will develop that you are making a difference.

The other key role is the medical advocate. This is the person who needs to be the brains to the caregivers heart. Write everything down, understand all medications, understand the rationale behind all tests. If there is a need for medical attention, go get it. Get the best you can, even if you have to fight a battle. Question everything -not to dispute it, but to understand it. Bring a notebook to all medical appointments and listen in on all medical rounds if in the hospital. Listen very carefully and then question for clarity. Since medicine is an art, not a science, there is no simple algorithm that doctors use to come up with the answer. The advocate needs to be a part of the decision making process if the patient cannot be. Don't let things happen around you with others making the decision, be a part of the decision. Be aggressive, actively representing the patient. This does not mean being a pain in the ass. I have asked the doctors at BU if they thought I was overly aggressive and a negative in the process. The answer is always: "active participation is always better than benign neglect". Yes, I asked alot of questions and yes I knew what I was talking about (sometimes not as much as I thought I did), but they respected me for being involved. I witnessed some spouses who did not go into an appointment with the doctor, could not even properly say the disease, had no idea what was going on, and thus could not advocate. The advocate is not about making the patient comfortable (that is the caregiver), but it is about fighting the required battles in the complex medical system we live in. It is very hard to do both, but it can be done. Just think of them as two different roles and understand which one your in. The caregiver has no pride and a big heart, the advocate has a big brain and a strong enough ego to go into battle if required. (As I right this, I know it sounds so obvious to most of us, but I witnessed such distant participation and bad care giving by family members that it shocked me).

Hospital chairs are terrible beds. It is a very small price to pay from what Chris has been through, and as the caregiver in the hospital, Chris wanted me there at night. But, these chairs are torture devices. The interesting thing is the difference in the patients. I had a very big back operation a year ago and was in the hospital for a week. The absolute last thing I would want is for Chris to be there overnight. In fact her being there for more than an hour was tough. When I am sick, just leave me alone, don't ask me how I am doing, just let me be and I will let you know when I am better and I'll call you to come pick me up. Chris is the opposite. She wanted me there every night and all day. I had to just accept it (the caregiver has no rights to personal comfort as the lowest form of life on earth) and sleep in a chair. Forget the previously cured back, but that is another story. The nurses were always very good about finding me a blanket and pillows and sneaking me a muffin or a soda, but all I can suggest is try to avoid these chairs as best you can. Again, a very small price to pay and probably my only complaint worth sharing.

Rely on religion, meditation, poems, psychologists, or denial - whatever works. The human mind is sometimes the patients worst enemy. Chris and I spent many many nights up all night, scared to death of our future, what would happen to our fantastic life, will we ever get through this, etc. Many tears, night sweats, all night pacing, it was horrendous. We both needed help, some kind of guidance. Chris reached out to many alternatives: our minister, prayer books, meditation, Reiki, massage, acupuncture, lucky bracelets from the kids, etc. Each worked a little and helped in the total picture. One thing that I found interesting was when we met with the Psychiatrist at BU as part of the normal regiment. He told us a very effective coping mechanism was the use of denial as a way to deal with this. Deny that anything could go wrong, deny that this is really happening, deny that there will be any changes in your life. He told us that this is how many patients cope. To me, it is a dangerous approach, but probably very effective. Chris found her prayer book very useful as she went to bed. It focused on hope, life, and love. She also got a bracelet from a good friend that said: Expect Miracles, Have Faith. She wears it daily and really believes that with faith, miracles can happen. One point I think is important to make is that while outwardly a sufferer and and their family may appear calm and collected, inside they are torn apart. They are scared, depressed, angry, and confused, but they fight hard not to ever show it. I was amazed at how Chris held herself together at all of Abby's graduation parties in May only to get home and breakdown. We all need to know that when others are going through this, what we see is far from the reality.

Family is critical. Both Chris' and my family have been fantastic. They have cared for our kids (with Chris' sister Debbie giving up her entire summer without hesitation), driven up numerous times from Pennsylvania (her brother Tim and sister Sally) and called almost daily (see comments below on blogs). Her parents have been fantastic. My family has also been great, always asking what they can do to help, sending love, being there when we need them.

It takes a village. I have written about this before, but more than anything this was the single largest lesson learned for Chris and I. We have been blown away at how our extended community has reached out to help us. This village, made of neighbors, life long friends, family, church members, colleagues at work, and even unknown people, have reached out to help and offer support. I cannot say enough times how valuable the meals, the rides to the doctors, the phone calls, the cards, the prayers, the small gifts offering hope have been. A great best friend coordinated all meals for many many weeks, to the point we had to say thanks but no more. Another has called up after dinner and said, "you did not walk today, lets go now", even though I know she was tired. Another drove her to Boston for entire day medical appointments on their only day off. Friends from her high school in Belgium have called and left many messages of support. Friends of our parents from all over the US have sent cards. Friends at IBM from Singapore, Sydney, Paris, all over, have sent their well wishes ( including a colleague in NJ who herself has been very sick and sent a great note of inspiration). Friends from UNH, Univ. of Delaware, a student doing a semester in New Zealand. On Chris' 100th day, we got cards from people we do not know, including a great card from a gentleman in Philadelphia who gave Chris some words of encouragement. It all has been absolutely beyond anything I had ever thought possible. For Chris, she has been so pleased to hear from friends, it means alot that people call to check on her. At this point, Chris now feels very good when she can give back, or "pass it forward". She did the breast cancer walk, she has been helping others in need, and she is really looking forward to getting back involved in much of the charity work she did prior to getting sick. It takes a village to take care of a very sick person, and this village of ours ( a worldwide village) has been the single most amazing thing for us in helping.

Communications. This is a hard one as it is complex. When we first entered this surreal world, we had no idea what to share, what to hold back, how to tell people, how to talk to our kids. Based on what I now know, the best approach is always one of complete and open communication. Tell anyone who wants to know the whole story, the good and the bad. There is no sense in hiding the reality. Some people will be very interested, others have their own issues and will listen and wish you good luck. One of the toughest decisions we had to deal with is what we would say to our children. We let them ask the questions but also tried to explain it all. We did lessen the degree of risk of the transplant/chemo. For someone with severe cardiac involvement like Chris, the chance of death during the first 100 days was about 20%. We knew this very well, but we probably hid that reality more than anything else. Maybe this was selfish on our part, but we did not want to deal with the emotional panic of our children while we needed to be focused on Chris. We wanted them to live as normal of a summer as possible, so we tried to give them only the positive. In retrospect, that was the right decision. The other lesson is the power of the blog. It has been invaluable. It has helped me give updates to hundreds at a time, it has been therapeutic for me, in fact very therapeutic, and it has let me share this ordeal with others who are or will go through it. One thing that was very hard for me was to answer the phone all evening long when Chris was unable to talk, giving an update on how she was. We would have very long days at the Center, I would make Chris a dinner and then clean both the dinner dishes as well as the food when it came back up, and then the phone would ring. I was exhausted and just wanted to rest and focus in getting her comfortable. Yet the calls were mostly from our families and I knew they were calling with their love. The blog has allowed me to give updates and people can read them at their convenience. One decision I made that I strongly feel is the right one was not to open it up for comments. This would have put pressure on me to respond, and more importantly I felt people would feel obligated to say something every time. We knew just by reading it that people cared, but by not allowing comments, I believe it was easier for both sides.
I have been told by many people that they really appreciated the blog and found it very helpful. I am not a natural writer and have never done any writing like this, but I have found that the best way of communicating my thoughts was to write from the heart. This was the therapeutic side of it for me. Probably the biggest mistake I have made is getting too focused on the medical elements - the details of the blood tests, the description of the disease, the medications, etc. Chris has read very little of this, literally a paragraph or two on a given day. When this is all over, I will somehow package these posts all together, from the beginning, and let her read them. She remembers very little, but I think she will find it very interesting. I occasionally have gone back and looked at random days. It has been an incedible ride and I am glad I have documented it. My hope is that as new patients enter into this craziness, they can at least see the details of one story. Chris and I were incredibly lucky that we had Jen and Steve Gershman to help guide us through, but there is very little documented, maybe this will help some. WHEN we are successful, then those scared to death like we were can maybe see actual hope.

Friends. Never ever try to watch the entire 10 year series of Freinds. Trust me on this one. There is clear evidence of acute brain damage to those who try. Yet as I write that, it has been our Friends who have done so much for us. Friends have been the single most important element of Chris' success and yet here I am warning people to avoid watching Friends. Not being a writer, I don't know if that is a juxtaposition, a contradiction, or the antithesis of each other, but it is at least interesting to me.

I think that at this point I have more than tried the patience of the readers on my "Lessons Learned". I apologize, but wanted to get it documented. I know I have editorialized, complained, over-described, and bored some to death with this blog, but I really appreciate your reading it. We are not done by any means with Chris' journey to health. We see Dr. Falk in 3 weeks,, we go back for a three day evaluation at BU in March, and Chris continues to work to get her body back to normal. We are trying to live a normal life now, and feel pretty optimistic. We went out for dinner last night with friends and ran into many other couples from around town. To me, it was almost like the old days (i.e. Jan. '08). We spoke about our kids, the town, real estate, jobs, etc, and little about Chris' illness. She has been through absolute hell and and here we are, less than a year from diagnosis, and trying to get back to a normal life. I look at her when she is talking to others and I feel so much pride and amazement.

OK, sorry for the far too long note. More in a few weeks, thanks for checking, please remember to look again down the road. It means so much to Chris when people call and say hello. Please feel free to call, send a note, give a prayer, or just wish her well.

Way overdue update- The Holidays and Cardiologist

2008-12-31T17:15:00.003-05:00

My apologies upfront for those checking the blog, your interest and support are great and I feel terrible that I have not kept things as updated as intended. There has been a lot going on and I will try to provide the latest.

Christmas was hard on Chris. Not physically, but emotionally. She loves the holiday and to her it represents the best family time of the year. So while she was thrilled to have everyone home, she was worried how many more of these she would have. She was switching between the joy of having everyone around with the fear of the future. It was the most negative and depressed I had seen her in months. We had my entire family as well as her parents over for Christmas Eve (23 people) and she worked her tail off to make it happen. It was fantastic and she pulled it all off. I played a surprise Santa for the neighbors next door (they did not even know it was me for a long time) and we all had a good laugh. Yet when it came time to go the midnight service at Church, she was too exhausted to go. She loves this candlelight service and it really puts the holiday into perspective for us all. But we went without her on her urging and returned home to her being very upset and depressed. It was the lowest I had seen her since July. I know that holidays can be extremely tough on people, and Chris wanted hers to be perfect. Yet I completely miscalculated how difficult it was on her emotionally. She was so tired, and had such conflicting emotions around the family all being there while being so scared of the future. It was a tough night for her and I blame myself for not preventing this. A lesson learned.

Christmas morning was fun and Chris did well. We opened stockings and presents and had a good breakfast with a very beautiful white snow outside. She seemed to have lost her sadness and was glad that her parents spent the night. We then went to my sisters for dinner. It was a blast (she knows how to hold a party!). The dinner was great, my Aunt joined us as well (so now 24 people) and the food was fantastic. Chris enjoyed it and really had a great time. Yet, she did not join the "dance party" where the adults and the teenagers went into a big time party mode. We had the music cranked and the place was jumping. What a blast. Chris never has been much of a dancer, but it would have been nice to see her rocking out. So Christmas Day was a very good one after a tough Christmas Eve.

The following Saturday (the 27th) was Chris' birthday. We all were up at her parents in NH and all of her siblings (Tim, Sally, and Debbie) made it up from NJ and PA to help her celebrate along with all but one niece/nephew (who was in Denver). She had a good time, a good dinner, and received some nice gifts. Again, I think this may have been a bit tough emotionally as well, as these milestones often cause one to think about the future.

Today, 12/31, we had the long awaited meeting with Dr. Falk - the cardiologist in Boston. Despite 8 inches of snow here, we made it in. Chris did the stress test that she did poorly on a month earlier. This one went much better. We then met with him and discussed the results - briefly. To make it simple, and to reconfirm my now strengthening hypothesis - Medicine is an art, not a science. We took Dr Falk through the BU results from early December and basically he dismissed them saying it was not possible to see such a reduction in the Ejection Fraction. The BU cardiologist had it at 25%, and today we heard "not possible". In fact, he was very upbeat, saying that based on her stress test results and his clinical observation, she was doing very well. He gave her the green light to start yoga, continue daily moderate exercise, and potentially start an even more aggressive exercise routine. We clearly have conflicting results from two different doctors. This is very frustrating, and of course we believe the more positive results. He wants us to have BU send the actual CD (not just the report) to him so he can see them directly. As I have stated earlier, I am not sure how doctors in different organizations work together, but when I see them dismiss anothers perspective, it is very disconcerting. We have to make our own decisions as to whom we believe and who's direction we follow. Clearly an art, not a science.

We will have the actual data sent from BU to Dr Falk and we will meet with him in 3 weeks - Jan 22nd to be exact.

So we walked out of the office today feeling very positive. We asked about the potential for a second stem cell transplant/high dose chemo OR the oral chemo. He said he would wait for the March 3 day analysis to give us his perspective.

So to wrap up the year, it has been a hell of a ride. Chris started what we thought would be a normal year last January, in April we heard the word Amyloidosis for the first time, in May she was confirmed with it, in June she had all her stem cells in her body removed and was given an almost lethal dose of poison (chemo), and in July she returned home 30 pounds lighter, no hair, no immune system, no strength, and in a distant stupor. She sat listless, never smiled, never did anything on her own, and watched life go by. Yet, she has worked relentlessly to get herself back to a normal life. She walks daily, is very independent, and actually yells at me like the good old days. I can't believe I am saying this, but it is so great to here her say "Steve, stop that" or "slow down" or "fix this". Of course she usually says it nicely, but I will take it any way she gives it. Chris has also seen both friends and fellow sufferers pass away from this terrible disease. It scares the daylights out of us, and in fact sets us way back emotionally after we work so hard to rebuild our confidence and optimism.

But now, on the last day of December of 2008, we end the year very optimistic. Chris told the doctor today feels better now than she did a year ago. That is progress, that is medicine, that is the power of prayer, that is the result of hundreds of well wishers all genuinely caring about Chris. She has reconnected with old friends, she has experienced more love than she ever thought possible, she has leaned on many people and they have supported her and she has verified that her family and friends are the most valuable people in the world.

For me, I have learned more than I thought possible. I have learned about blood disease, about medicine, about hospital practices, about how doctors think, about clinical trials, support groups, drug research, mortality rates, bio-markers, and how the human body rejects the poison of chemo by attempting to force it flying out of all ends of the body. And I have learned how to clean that up after that as well. But more important than the medical side of things, I have learned an amazing amount about the human side of this. I have seen what complete fear of death looks like, I have seen how a community has given all they possibly can to help a friend in need, I have seen a sibling give up months with her own family to take care of ours, I have seen my colleagues at IBM offer their help and support, and I have seen a very brave and strong woman fight a killer disease like a prizefighter. She may get knocked down, but she gets right back up again and goes the next round. She is amazing.

I have owed my Top 10 lessons learned for months now. I think I first promised back in July. My hope is to write those this coming weekend. I pray nobody ever has to go through what we have been through, but there has been a great deal of learning and I owe it to everyone to share.

I wish you all a very Happy New Year. Drink a toast to both Chris and yourselves as you usher in the new year. I will drink a toast to all of you!!!

And again, my apologies for my delayed posts. I know how many people check it regularly.

Very Tough Week - Physically and Emotionally

2008-12-21T04:41:00.004-05:00

The week before Christmas has been a pretty difficult one for Chris unfortunately. She has had two bumps in the road - yet remains stalwart. Actually to call them bumps in the road minmmizes them - let's call them detours.

Wednesday was my third day down in NY attending some very tough meetings. I had flown down early Monday for 4 days of meetings while Chris and Stephen remained home. All was going very well so Stephen left Wednesday afternoon to go to Maine skiing with friends. At about 10:30 PM while I was dragging myself to bed after a very long day, my phone rang. It was Chris. She was breaking out in hives with rash across her body, her lips swelling, incredible itching, etc. This came on relatively suddenly. She called a friend who had been giving her some shots - the blood thinner coumadin. As a nurse, her friend has been very helpful as Chris hates needles (imagine the hundreds and hundreds of injections she has had this year). Her friend suggested she take some benadryl and call the doctor. Of course we had none in the house ( bad luck on our side ) so a scramble of calls started in the neighborhood and some was found (good luck except for those getting phone calls late at night). Her doctor called her back and said, "get to the ER - STAT". So without hesitation, our great friend Sally said "grab a coat, lets go". No kids were home, I was not home, and here is Chris heading to the ER at 11:00 at night. I won't bore you with the details, but Chris has had a very severe reaction to ==> we have no idea! 3 days letter, she is still breaking out with a very ugly rash across her torso, on her neck, under her arms. Big, red, raised welts. The ER treated the symptoms (prednisone, more benadryl), but did not identify the root cause.

This could have been caused by the coumadine, it may have been due to a new pill (same prescription, just different manufacturer) she started to take, possibly a couple of injections she needed prior to the coumadin, or even that Pad Thai she had for lunch. She got back to the house just before 3:00 AM and Sally spent the night in case of any further problems. Here is Sally getting into her own bed and 5 hours later she is getting back from the ER to sleep in another house. She was amazing - as she always is. I was pretty much useless other than irritating phone calls to Chris as she spoke to the ER physician. For so many months I have been her caregiver and medical advocate and here I was 200 miles away in NY - not a position I like being in.

So she is still suffering from the rash. She woke up Saturday morning with it all over her, wanting to itch everywhere, literally jumping up and down wanting to get out of her own skin. Large doses of benadryl and the prednisone help, but if we don't stay on top of it, she breaks out really badly. This all of course has just added to her frustration. As we drove to Burlington VT to pick up Melissa at the end of her semester, her frustration really showed. She is "so sick of being sick". "Why is this happening to me?", etc. I know well when she is on the verge of wanting to just breakdown and let it all out, but she allows herself to complain, maybe a tear or two, and she pulls herself together. She is a rock.

We will continue to look for the cause of the reaction. We have been in contact with her primary doctor, her cardiologist, and Boston center and they are working together to determine the next steps. In the meantime she is not taking any coumadin (or pad thai for that matter).

So Saturday, Chris and I drove to Burlington in the snow to move Melissa out of her house (in preparation to a semester in Australia starting next month). It was a very long day with about 6 hours of driving in the snow, packing her up, etc. We got home later than expected, yet ran to a party we had RSVP'd to earlier. We got there late and stayed relatively late. Upon our return home, she checked her mail before going to bed. This is the emotional detour, or crash I could say. As I have mentioned in earlier posts, Chris has been reading other peoples blogs who suffer from this terrible disease (yet still does not want to read this one). She reads the updates, prays for them, we talk about the differences with her care and their unique situations they all are going through. With me, she calls them by their first name, although she has never met them. "Paul had a good day today, Kevin is getting his heart transplant". The one blog she has been following since early fall has been a man in NY (Paul) with 2 young kids who went through the stem cell transplant and high dose chemo a few weeks after her. His wife and caregiver has been faithfully documenting his journey and Chris has felt very attached to the story (and Paul). He went home, she was thrilled, he had dialysis and she felt bad for him. Chris read yesterdays update and it threw her and I an unexpected shot to the head - Paul died of cardiac failure that morning. Two boys, 3 years old and 9 months old, a young wife who had been his caregiver, advocate, emotional support, etc. are left behind. They fought this battle as Chris has, with fortitude, optimism, many many tough periods, and with an incredible determination to beat this and get back to a normal life. Chris is devastated. Such a long, difficult, yet courageous battle only to have lost it in the end. Chris is scared, angry, confused, and very very sad for the family. This has thrown me for a loop as well, a big loop, as it brings the reality home. I can't share my emotions with Chris when she needs strength to lean on, but damn. DAMN THIS THING!

If you want to read a very tough story, here is Pauls blog:
http://gettingpaulhealthy.blogspot.com/2008/12/very-sad-day-indeed.html

Chris and I will get our act together and face the holidays with the joy of the season. We will have my entire family together and see almost all of hers in the next week or so. She has had a hell of a week, but she will do great. Keep praying for her. I'll give another update before Christmas.

Mixed News - as predicted

2008-12-13T13:47:00.006-05:00

We heard from the doctors at Boston yesterday afternoon and it is not great news but more importantly it is not terrible news. In the end, it is progress, but will require us to continue the fight as it is not over. The results are what we actually had been predicting, as we are now smart enough to read the test results, ask the right questions, and know the options as the various meetings with doctors occur. I don't need to wait for a big report when I know the individual elements themselves. So, what I will describe is what we knew for the most part.

Lambda light chains: Normal is a number of 5-26. Chris it turns out was over 700 at one point and 660 when first diagnosed. She is now down to 39, and this represents a drop of 94% or so, but unfortunately still above 26. This means the amyloid proteins are still being produced, albeit at a significantly reduced rate. A 94% response is a long way there, but not across the finish line.

Plasma Cells in her bone marrow: Normal under 5%, Chris was over 10%. Now after the high dose chemo, she is back to under 5%. This is very good.

Serum IFE (immunoglubulin): This was down to zero, a very good story.

Flight Light Chain Ratio (Kappa/Lambda): Normal is .2-1.6. Chris is at .2, again very good.

So she still has the lambda free light chains being produced, but the biomarkers are all looking very good.

Cardio: Here is the difficult part, for both the doctors and for us. They cannot explain why the hematologic response has been so good while there continues to be further damage to her heart. We just got the analysis from the TEE (the echo through her esophagus) and it shows "severe left atrium enlargement", and "the right atrium is severely dilated". Further, there is "moderate to severe mitral regurgitation." They show an ejection fraction of 20%-25%, where normal is over 50%. Thus, as stated in earlier posts, our focus now shifts from the blood to the heart. We fully expect that there will be more work to do on the amyloidosis, as we need to stop the continued protein depostion, BUT, job #1 is now to understand and execute a plan that protects the heart. So the next activity is to meet with the Dr. Falk on December 31.

For those of you interested and looking for some technical and potentially mind-numbing/sleep inducing reading, here is a great paper written by Dr. Falk:
http://circ.ahajournals.org/cgi/reprint/112/13/2047.pdf .

I suggest only the very curious spend the time to print and read this, but it gives a detailed overview of the new battle front.

So enough of the technical stuff, lets talk about the most important element, Chris herself. Overall, she is doing very well emotionally and physically. She has been on the treadmill every day - this is her full time job, to ensure she is in top physical condition with the constraints she has. She has been very active, decorating the house, going to parties around town, shopping, etc. Outwardly, you would see little difference than the Chris you would have seen a year or two ago, except about 20 pounds lighter (she is up to a whopping 109) and very, very short hair (which is really starting to come back). She is upbeat, she is active, she is feeling good. However, there are times when we are both in the dump a bit. The worst time is late at night, when the mind has time to think a bit more. We both worry, about the future, about implications, about options, and about whether there is more we can be doing. I personally continue to ask myself; what else could we be doing? Are we missing a test? Are there better doctors to talk to? Am I prepared for all situations and outcomes? I hope so, but who knows for sure.

Overall, we are very fortunate. I can tell you when I first heard the word "amyloidosis" on Friday April 4th while touring Hartwick College with Abby, I had no idea what it is. Since then, Chris has had a stem cell transplant with high dose chemo, been "as close to hell as you can get and still being able to come back". returned home, and is living an active life. Melissa has written papers and made presentations on Amyloidosis at college, Abby has been an amazing help, cheerleader and supporter to Chris, and Stephen announced last week that for his required Senior Project at Worcester Academy, that he will be raising money for Amyloidosis research by riding from Florida to Holliston during his 2 week spring break - 1200 miles (more on that in future posts). So our family has been great. Yet, the most amazing thing has been what has occurred outside the family (which I know I state too often). Never ever, in my wildest dreams, did I ever expect a community to come together and help a friend like people have for Chris. It is the most amazing thing to see. Beyond anything I can describe. From the smallest things like a phone call to ask her how she is doing, to cards, to daily walks (sometimes in the rain and late at night in the neighborhood), the brownies, sauces, soups, meals, the errands, the interest, the listening, the learning, and most importantly the prayers and positive thoughts. It is beyond belief. There are no words strong enough or big enough to thank Chris' sister giving up her entire summer with her family to watch our family. I can't describe the incredible strength Chris has had in this fight, and I can't begin to express how optimistic I am for our future. We are going to fight two battles now (heart and blood), but I know the alignment of my tough wife, our amazing support (all of you), and the best medical care in the world will end up with success.

I'll try to update the blog occasionally over the next few weeks, and will provide a complete report after the meeting with Dr. Falk. Thanks for your continued interest.

Waiting for Godot (really Sanchorawala)

2008-12-11T20:06:00.003-05:00

Chris has been very busy today and not had much time to be idle and thinking. That is a good thing. We are waiting for the big call from the medical center tomorrow afternoon where they will tell us what the indicators are for the degree of response. I know hundreds of people are waiting for the big HCR to be claimed, but based on some of the results, while I do not think we are there, I think we are close. The question I have been asked frequently, and a good one is: "At one point do they claim victory?" Is 95% close enough? Is 98%? The answer is pretty binary as I understand it. Your either have a complete response or you don't. If your body is still producing the amyloid proteins ( and as you know, this means the lambda free light chains), then we have to stop the production and the resulting deposition of them in the organs. More proteins mean more damage, and thus there is still work to be done.

My best guess is that they are going to say - "we are pleased, there is a good response, enjoy the holidays, come back in January and we'll retest and decide the action plan then".

As I thought a bit more last night about how to describe Chris' status, I would break it down into two areas: 1) Hematologically things are really looking good. 2) Cardiologically (a new word I just invented I think), things are OK but need a great deal of attention. I remain positive that if we can stop further heart damage, then Chris can move forward with a great and active (but not too active) lifestyle. Her positive attitude will make a huge difference going forward.

Chris did sleep very well last night. She has carried such a tremendous burden of stress that I know she felt as though she completed a major milestone yesterday. As she slept soundly this morning, I swear there was a smile on her face. This meant the world to me.

More tomorrow night or maybe early Saturday morning. I cannot say it enough - Thanks to all of you who care so much.

Return Visit - Day 3

2008-12-10T18:44:00.002-05:00

OK, a deep breath so I can think how to best make this understandable. Bottom line is two key messages: 1) all the test are not in yet, 2) the tests that are in show very mixed and confusing indications/results. Let me start with the bad and finish with the good.

Chris has a serious heart problem that has become worse since the spring. The number one issue, amongst many, is a significantly reduced ejection faction (EF). In simple terms, ejection faction is the % of blood pumped out of the heart with each heart beat. Normally this is in the range of 55% to 58%. In May, Chris' EF was 49%, not too bad. Today it is 35%. This has clearly worsened over the year. This is not due to the Chemo, but amyloid damage. This reduced EF is a symptom of the hardening of the left ventricle wall, which with reduced muscle elasticity has less ability to pump efficiently. There may also be some valve leakage between the atrium and the ventricle. What this all means is that Chris continues to have congestive heart failure. However, do not let the term 'failure' scare you as it did me. Failure in this case means that the heart is just not pumping at normal rates, so failure does not mean stoppage, it means reduction. The reduction in the pumping means that as her body calls for more oxygen as she exercises, climbs stairs, whatever, she will not be able to keep up with the demand. She would become easily fatigued, out of breath, and maybe have chest pounding. The cardiac story is not a good one, but she can function with normal activity and if the damage stops where it is, she can live a normal life while being careful. Nobody knows what further heart damage will occur going forward. I asked the question a hundred times, but nobody knows.
We see Dr. Falk (the most knowledgeable cardiologist in the world on this) again on December 31st. He now becomes an even bigger part of the game plan going forward since heart involvement becomes the single biggest issue we face.

OK, so now the better news. The other organs all are doing well, in fact her kidneys, which were problem number two after the heart, has actually improved since the chemo. There are a potpourri of blood and urine tests to measure kidney effectiveness. Almost all of these came back with improved results. Her kidneys are doing what they are supposed to be doing, they are seeing less amyloid proteins then they did before, and they are flushing them from the body better than before. One of the key tests is the amount of protein collected in a 24 hour urine sample (Chris hates carrying the jug around, but has now become a pro at it). Her Mg/24 hours were over 4000 in May, now they are about 2800, so this is a big drop and indicates a downward trend. But, to put it in perspective, normal is about 150 Mg/24 hours. As they explained it all today, the reduction will take as long as two years. I of course asked where we were on the reduction curve, ahead or behind what they would have expected for a good response and they said "6 months ahead of the curve". Obviously that is good.

The other key metric is the amount of lambda free light chains. Before, Chris was 660. Today she was 39. This is the single best news we have heard. This is a direct result of the chemo and indicates that the free light chain proteins that are created by her plasma cells and are floating around, combining and becoming insoluble in her organs have reduced considerably. This is about 94% reduction. Better than no reduction, better than 50% reduction, and better than 93% reduction. BUT, not a complete reduction. Normal is 15 -26. There are 4 elements that make up the criteria for a hematologic complete response (the famous HCR). Getting down to 26 or lower is one of them. The others are the Bone Marrow plasma cells at under 5% (she was at 10% in May), a Kappa/Lambda ratio between .26 and 1.65 and a good serum Immunofixation (IFE) test (too hard to explain here). The only test data we have at this point is the amount of lambda free light chains, and that is very good. The others we will get on Friday.

So now, the big question: Did we get a complete response? the answer is we do not yet know. We probably need to wait another month to see if the lambda's continue to reduce. To be honest, based on the discussions today, it is not clear if a complete response is possible. I seems to be unlikely at this point. The next big question is: what if it isn't complete response, now what?

Four options:
1) A second stem cell replant with high dose chemo (all over again), probably next spring/summer
2) An oral chemo, using a drug in trial called Revlimid
3) an oral chemo, using a drug in trial called Velcade
4) do nothing and watch.

Chris and I were very clear (more me than her) - we want to continue to be very aggressive. If they heard one word today it was "aggressive". Chris survived the pure hell of the high dose chemo this past year, and will do it again if its poison (and that is what it is) kills the rest of the bad plasma cells creating the proteins.

So here is the bottom line:
Chris has very bad heart damage, which we are seeking world class medical help to address. She will most likely be placed on an anticoagulant to prevent clots in her heart from causing a stroke.
The test results from this week are not all in, and thus we do not know what the prognosis is. The ones we do have are positive, but not where we need them to be. My guess is that they will ask her to come back in January and we will jointly decide the course of action.

So there is a lot we don't know.

Here is what I do know:
-Chris is tough as nails, an incredible fighter who is fighting the fight of her life
-We have found the best medical care possible - no second guessing on a single thing we have done
-We have the most amazing family, friends, colleagues (IBMers), and doctors we could have ever wished for
-The power of prayer, good wishes, concern, good meals, cards, phone calls, walks in the woods, etc have been the single most important factor in her success to date. All of you reading this blog are responsible for her progress to date. Thanks to everyone of you!!!

Return Visit - Day 2

2008-12-09T18:31:00.005-05:00

Not a great day today. I'll keep this short, as I really am not feeling like going into great detail and I am hoping that tomorrow will be better. Today was two sessions:

1) the support group. I have now been to five of these and each f them is different. Today there were a variety of experiences/stories. A woman from California was waiting on a heart and liver transplant, a man from NC had his transplant and high dose chemo 4 years ago and was doing great, another man had just localised impact in his stomach. Many different stories, but generally all good ones. So we walked out of that feeling good about these stories.

2) met with the Boston Medical cardiologist (not the one we saw two weeks ago). To be brutally honest, neither Chris or I like this guy, and we think it is more than just the message he gives. He has the sympathy of a rock and the heart of a piece of cold steel - just brutal. So here the news was not as positive as we were hoping for. There is continued decline in Chris' heart function, specifically her ejection fraction which is a measure of pumping effectiveness. There are some "biomarkers" or measurements of heart effectiveness that were not as good as they were previously. It was not a positive story and Chris and I walked out about as low as we have felt in the last 3-4 months. Tomorrow we will get the larger picture from the doctor we trust the most, but today was a huge stab in the back.

Despite the heart results, Chris continues to feel great. She honestly has a great attitude, has alot of energy, and is very positive. I think how she feels trumps what the chemical indicators show, but for me, it has been a very tough day. I'm hoping for a better day tomorrow.

Return Visit - Day 1

2008-12-08T18:21:00.002-05:00

Our first day back at the clinic in a long time, but it is very familiar to us and we are comfortable being there, strange as that sounds. We know may of the people, we know the building, we know the procedures, and we know all the terms. It is easy to feel like veterans having been through this previously. As before, and as we once were, there are people there that are going through this for the first time (the deer in the headlights look) and they have no idea what to expect. They have read that this is a fatal disease on the Internet and the average life expectancy. They don't know anything about the disease, the treatment, the doctors, they are just scared to death. Chris and I relate to this so well. We tried to start up conversations with the others going through their first three days and attempted to reassure them that they have found the best place to be. On the other side of the coin, we met a man today who had been diagnosed 4 years ago at the age of 70 and he also went through the stem cell transplant and high dose chemo and is doing well. So we are both givers and receivers of the all important "hope" that is the required ingredient for success.

Day 1 consisted of the following:
Blood Work and Vital Signs: Chris has many many vials of blood drawn today. They will analyze every possible aspect of this over the next 24 hours. Her blood pressure was a bit low (91/74) but not too bad. They will check her red blood cells, her white blood cells, and about 90 other unique tests. The lab results we received last time were 12 pages long.

Chest X-Ray: Chris has had many of these and to be honest I am getting a bit worried about all the radiation she must be getting. I would say she has had over a dozen x-rays in the last year on her chest/lungs. They are looking for pleural effusion - basically water in the lungs or surrounding chest cavity. This is an indication of congestive heart failure and was a problem she had pretty badly this spring. As with the blood test, we did not get any results.

Bone Marrow Biopsy: This is the dreaded big thick long needle inserted into the pelvis bone marrow. They remove a few large needles full. They are primarily looking at the % of plasma cells and the degree that their a light chain proteins in the plasma. Again, no results today.

Echocardiagram: This is the heart imaging procedure where the measure size, wall thickness, efficiency, etc. It is about a 40 minute test that they record to a DVD. Between the echo she had a few weeks ago, and the esophagus one done last Friday, they will have lots of data to look at.

Examination: This was the only meeting with a Doctor today. They asked her about a gazillion questions about her health, her medications, her activity, her emotions, and just about everything else. They were surprised to find out we went and saw the other cardiologist, but were interested in seeing his analysis. We had not met him before as he is a "fellow" new to the center, but he seemed like a good guy. I think I amazed him a bit with my knowledge of the disease, the tests, the "biomarkers", etc. I asked him questions about her BNP, her lambda numbers, her degree of left ventricle hypertrophy, etc. ( I have done a TON of research). He was a bit taken aback but acknowledged that much "homework" had been done. Nevertheless, we got no answers, only a "wait until Wednesday" response.

So the day was uneventful. We have no further insight, no good or bad results, just an ongoing level of nervousness about what we are going to hear later in the week.

I know many of you prayed a special prayer for Chris this week. It is not possible to tell you how much this means to us, but trust me, Chris feels the amount of love and good wishes being sent her way.

More tomorrow night after Day 2.

Heading into uncharted waters

2008-12-06T10:04:00.002-05:00

This past week and this coming week are very strange for us. We really do not know what to expect, how to properly be prepared, and how to react to different scenario's. This past week has been a busy one for Chris on the medical front, but of course next week is the big one.

On this past Tuesday, Chris had a stress test in Boston with Dr. Falk, the cardiologist. The test was short as Chris has had a bad cold that she really has not been able to shake. Lot's of coughing, stuffiness, general malaise. She said she felt tired on the treadmill after 5 minutes, and her blood pressure dropped a bit, which concerned her. She generally walks about 45 minutes straight everyday, so it was a bit disconcerting to not do as well on the stress test as she would have expected. I think we all think(or hope) this was due to her cold. As such, she has been rescheduled for another stress test on December 31 - what a way to celebrate the end of a tough year.

After this appointment on Tuesday, she met with her primary care physician who put her on an antibiotic to try to get rid of this cold. That was Tuesday and having heard her cough most of the night last night, she has not seemed to have broken this yet. I am hoping today is a much better one for her.

Yesterday, she was at another Boston hospital having a transesophageal echocardiagram. They had to put her out for this as they put a device down her throat to look at her heart more directly. The whole intent here is to look at possible damage to the right atrium and determine whether there may be some kind of an exposure with the proteins that could possible cause a stroke. The treatment is to put her on a blood thinner, possibly for the rest of her life if stroke becomes a concern.

Chris' spirits have been a little lower in the past few days. I am not sure if this is due to her cold, her having to go back to the hospital for more prodding and probing this past week, or the uncertainty of this coming week. We both know that in less than a week our lives will be different than they are now. We have many questions, concerns, hopes, etc. If there is a 50% reduction in the free light chains ( a 50% hematological response) does that mean another high dose chemo next summer? )Below in the last paragraph of this post, I will explain "free light chains). What if it is 90% or 10%. Is there a direct relationship between the percent response and life expectancy? Have there been any new medicines or clinical trials we need to pursue and consider since this past summer? Do we take immediate action or wait for her to get stronger? Clearly a lot of questions running through our mind.

Many of our friends have called to provide words of encouragement in the last few days. Those are all well appreciated. I'll try to accelerate the pace of these posting over the next week as I know how much concern and genuine interest and support exists out there.

Free light chains: We all have a small number of the protein elements that are not attached to the normal plasma protein. Our bodies produce two types of these unattached protein molecules: kappa and lambda. We have small amount floating in our blood and there is a normal ratio between them. A normal range for us is between 3 and 19 mg/L of kappa light chains, 6 and 26 mg/L for lambda light chains, and a kappa/lambda ratio of 6 and 26. Chris' kappa light chains were normal, but her lambda light chains were 660 mg/L. Thus she had more than 33X the normal rate of these lambda light chains floating in her body. These are what end up connecting to each other and forming the insoluble proteins that get lodged in the organ. Our hope is that the chemo has taken these lambda light chains from 660 to under 26. That will be a Complete Hematologic Response (the famous HCR). In a nutshell, that is what we are looking for this coming week - elimination of the lambda free light chains in her body.

Sorry for the medical lesson, but I know many have asked for a little more detail.

Have a great weekend and watch for updates next week. Thanks for your prayers this weekend.

Giving Thanks

2008-11-28T15:39:00.002-05:00

This Thanksgiving week has been an interesting, and good one. With all the kids home, Chris has been energized and she really has a very positive attitude and great spirits. She has enjoyed doing lots of activities with them (cooking, errands, visiting friends, etc.) and will clearly miss the girls when they leave at the end of the weekend. I have watched and am very pleased to see how them being around has made a difference in Chris' outlook. We had Thanksgiving dinner at my sister Judy's and Chris did great with a long day, no signs of tiring and a good appetite.

On Wednesday, Chris and I had an appointment with a cardiologist in Boston who is the world renowned expert on amyloid damage to the heart. He used to be part of the Boston Medical team but has since switched medical partnerships. Nevertheless he remains the "go to guy" on this subject. Again, yet another advantage of being in Boston where the world class health care and expertise just doesn't end. Dr. Falk has led the medical field in clinical analysis and we sought him out to get his perspective, validate what we were hearing at Boston Medical, and satisfy our own need to ensure we have engaged all possible expertise. I am not sure how the Boston Medical program will react to us seeking other opinions, but I am sure they will respect our need to leverage all options.

The appointment went well. Chris had an exam including an echocardiagram, had blood work done, and we spent a long time answering questions - providing descriptions of the history, the treatment, and the period back home. This has led to two next steps. On Tuesday, Chris will have a stress test on the treadmill as they measure blood pressure, heart activity, oxygen levels, etc. This will give us an indication of her level of fitness and her ability to increase her daily amount of exercise. If she shows that she can maintain her blood pressure (and not get light-headed) while she exerts herself, this will be a good sign. If on the other hand she gets winded easily, her chest hurts, and her BP drops, this will probably indicate the heart damage is more than maybe we had thought. Tuesday will be a big day.

On Friday, Chris is going to Brighams and Womens Hospital in Boston for the next procedure. Here, they will insert a camera down her throat and somehow (Chris and I have been trying to figure this out) will snake it to her right atrium in her heart where they will look at the heart wall. They are afraid of hardened proteins deposited in her heart wall and want to see to what extent they are there. These can actually break off as a clot and cause a stroke. To prevent that from happening they are considering placing her on a blood thinner such as coumadin.

So next week will be another important week for Chris' journey to health. This of course will be followed by the week we have been trying to patiently wait for. Chris and I are both clearly getting nervous as we get closer, knowing that what we hear in the next 2 weeks will impact us more than can be written. Good or Bad, our lives will be forever changed. I could write for hours as to what I mean by that, but suffice it to say we are hoping for the best, preparing for the worst.

No matter what happens (in my heart, I know a miracle is going to happen), Chris and I are both thankful for the incredible love, support, friendship, and compassion everyone has given. We had an amazing Thanksgiving as we are more thankful than we have ever been in our lives. We are thankful for family and friends, the fact that we have been able to find the best medical support in the world, the fact that we hopefully caught this early, and the fact that we have been able to be very aggressive in treating this disease. We all have much to be thankful for.

Just waiting

2008-11-16T14:50:00.002-05:00

As we get closer to the big 3 day evaluation back in Boston, it is becoming more difficult to wait, at least for me. Chris and I want to know what direction things are going and we will not know for sure until we get a final analysis the end of the week of December 8th. I am sure the week before will be one of much anxiety.

Chris is generally continuing to do well. She stays active, is very alert, and usuallykeeps a positive attitude. Yet, she has yet to gain any significant weight, which has been an ongoing issue for months. Despite a great deal of trying hard to eat high calorie foods, she is still only at 105 pounds. This week will be 5 months since her stem cell transplant, and yet in all that time, she has gained little weight. Some of this may be due to her long walks everyday, but we will see what the doctors say when we meet with them.

Chris actually went to the hair salon last week with her mother. While she was there, she had the back of her next cleaned up a small bit. The minor act of sitting in a salon chair seemed to have pleased Chris. She was happy to brag that "she got her hair done". In reality, it has really started to come back well. It is still very short, but at this point you cannot see her scalp anymore. That said, she continues to run into old acquaintances and many of them don't recognize her. She is very thin and she wears a scarf on her head, so she gets strange looks from people and she needs to say "Hi, yes, it's me".

So we watch the calendar inch forward. As we get closer, each day seems to take longer.

In the meantime, Chris is still getting cards of encouragement and an incredible amount of support from friends, friends of friends, distant family members, etc. Thanks to all of you for that.

A Reality Slap To The Face

2008-11-08T07:05:00.002-05:00

A very strange week with some great highs and some tough lows.

Chris and I went out to Hartwick to visit Abby on parents weekend last weekend and it was very good. Chris did great with the travel, the two hotel nights, walking all over campus, going out for dinner, shopping with Abby, etc. A very busy couple of days, requiring stamina with little down time. She never complained about being tired or not feeling well. On Monday night, we had agreed to meet with some friends from Ohio for dinner in Boston whom we had met at the BU Clinic back in June. He went through the program about 4 weeks before Chris. We had not seen them since then and they were back for their tests after 6 months. It was great to compare notes, horror stories, medication regiments, exercise routines, etc. He had grown a fair amount of hair back and was walking daily and even playing golf. It was encouraging for Chris to see the improvement in energy, confidence, and demeanor since we saw them last. It was fun to go in to Boston and enjoy a great dinner - something fairly normal that we don't take for granted any more. We received a few notes from them as they went through the various three days of testing during the week.

The rest of the week was very busy as well. Between voting, doing her daily walks, keeping the house organized, Chris was busy and really feeling confident. Wednesday night she told me "I am really feeling "normal" again". These are just incredible words to hear.

On Thursday, she drove up to NH to see her parents. She drove by herself (2-2.5 hours), went to dinner with them and raked leaves and did chores around the house on Friday, driving home late Friday afternoon. A few months ago she just started driving downtown and now she is driving to NH by herself to help her parents do jobs around their house. I know I keep saying it, but I am amazed by the energy and confidence.

After she got home from NH last night, we went to the H.S. football game. We stood and talked to good friends during the game and Chris was laughing and really enjoyed being out, even after a busy few days. Getting home, she stayed up and did more things around the house. Overall, an extremely busy, very hectic last week between NY, NH, and activities at home. Life as we want it.

Then reality set in, quickly.

We received an email Friday night from the friends in Ohio who had returned home from their three days of evaluations. They were devastated at the results that they had just heard. They, like us, thought all was going amazingly well - the ability to play golf, the daily exercises, the alertness, etc. Yet, after he showed so much visible improvement, their results came back at a reduction of the proteins of 65%. Obviously that means that his body is still producing 35% of the defective proteins - not the Hematologic Complete Response (HCR) that we all pray for. In my eternal optimism, my naivete, and daily indicators of her progress, I am afraid that we have been slowly lulled into a false sense of confidence. As posted way back months ago, the chance of a complete response is 40%-50%, a partial response from 25%-40%, and no response or fatility (the 100 days) from 10% to 25%. I think in both of our minds, we have convinced ourselves that she has has had a complete response. That is what we pray for daily. Yet, the reality of our friends results cannot be ignored. He had stated earlier that he "will never, ever, ever do chemo again". Under no cicumstances. However with only a partial response, he has no choice. The tougher news is that they did not collect enough stem cells to do a second transplant and high dose chemo back in June. He will have to do oral chemo for three weeks every three months. He'll lose the hair, have no energy, get all the sickness again, etc. I am sure this second "kick", he will beat the proteins once and for all, but for us, it is reality slapping us in the face. We have no idea what Chris' response will be when we go back in December. It could be 100% or it could be 10%. We have said we need to hope for the best, plan for the worst. But we have allowed little of the "worst" to creep into our heads. False optimism, denial, foolishness - I'm not sure. It's carried us this far, but we need to be prepared for all outcomes. Reality time is coming soon.

Keep up the prayers, this isn't over yet. Thanks to all of you.

Doctors Appointment Today

2008-10-30T21:41:00.002-04:00

Another checkup with Chris' primary and things are still progressing well. The good news:
- her lungs were very clear, best in a long time, hardly any fluid at all
- blood pressure has remained very good
- no measurable edema
- increasingly more stamina

She gets good grades for all her progress.

Needs improvement:
- weight gain. Is stuck in low 100's (103 pounds this morning) despite an incredible valiant attempt to eat early and often. Her doctor believes this may indicate some intestinal impact but that is not clear.
- still some red marks showing on her skin. These come and go, but are marker that should stop after awhile.
- cold head!! Chris can't believe that men walk around all day long with their head so cold. She is starting to really get a scalp full of coverage, but still is always complaining that her head is so cold.

Her walking has continued with daily treks. She now tracks her distance with a pedometer (thanks Paula) and can ensure that she really improves her speed and distance on a regular basis. I just remain amazed at the difference since this summer when she was at the other end of the spectrum. Going back and reading the posts from late June/early July and the stark improvement jumps out.

There was an interesting article in the Wall Street Journal recently indicating the need for a family member as a patient advocate for someone in the hospital. I could not agree with this more. I can cite many examples when staff turnover, general inattention, sloppy procedures, and unclear direction added an undue level of risk into Chris' recovery. As I have said before, Boston Medical Center was outstanding, beyond any expectations of a world class facility, but no hospital is perfect and no individual totally focused. The value of a patient advocate, whose sole focus is that of caregiver in the hospital cannot be overstated. Watching, questioning, learning, explaining, documenting, and questioning again, even if overzealous, is so important.

Chris and I will go and visit Abby for parents weekend tomorrow. This is another major milestone. Abby is doing great, we'll be glad to see her, and Chris will certainly get some exercise on the hills.

All the time, I keep looking at the calendar. We are getting closer and closer to December. To be honest, I get more nervous every day, but I think how incredibly lucky and blessed we have been so far.

Update on Chris

2008-10-20T17:36:00.002-04:00

Chris continues her forward momentum. She has been getting out even more than before, has been very busy with normal activities and is really getting back to running things around here her way. My brief assignment as head of the household has been unceremoniously ripped from my clenched fists. Clearly my turn at the top of the family food chain has been deemed as a nice experiment, but in Chris' mind can easily be considered a weak attempt at organization. Without a doubt she is getting stronger!!

Yesterday Chris walked 5 miles (with some good hills) as part of a Walk for Breast Cancer fundraiser. She was hesitant to do this as it would be at a good pace and no opportunity to really stop and rest. She completed it with no issues at all. Here she is diagnosed with a very serious disease that she is battling everyday and she goes out and does this event. We are all very proud of her. This was both a physical and mental challenge and she did it with a smile on her face. I asked her if she thought she could have done this a few weeks before any of the chemo or stem cell transplant and she thought she could not have. In other words, she feels better now then she did before the incredible undertaking she went through this summer. This to me is a testament that something is going well. We don't know what is happening on the inside (we won't until December). but without question, good things are happening on the outside.

Chris still continues to be extremely thin (104 pounds) and has got to now focus on muscle strengthening. She wants to go back to yoga and her gym, but is afraid of all the germs, so we are kind of in a no-man's land; needing to workout with a trainer but not able to go to the gym where all the equipment and her friends are. She will probably soon get frustrated enough to just go ahead and start the workouts and yoga, while ensuring she is maniacal about washing hands, etc.

She has started to see many more of her friends around town as she gets out more. Some recognize her immediately and rush to give her a hug, while others just nod at her and have no idea who she is. Then they do a double take and are thrilled to see her. I am sure that when we get some weight on her, she loses the always present head scarf, and she gets a little color, that she will look as good as new. In all cases, everyone is thrilled to see her and are eager to tell her how they are of her. I know we all are.

Friday - October 10

2008-10-10T17:03:00.002-04:00

Things continue to get better and better with Chris. She has more energy, is moving fast, keeping very busy, and starting to really grow some hair back. Nothing long, but definitely covering her scalp. It is really amazing how she is falling right back into her normal life after such an incredible summer. She is up to a whopping 103.5 pounds, yet she continues to eat very well. The food from the neighbors keeps coming, with everyone trying to fatten her up - but of course I am the one packing it on.

Chris feels she can travel relatively comfortably, so we will spend some of next week looking at colleges in upstate NY, VT, NH, and Maine with Stephen and me. Last weekend she went to the local town fair and ran into many friends she had not seen since prior to all of this. She was very glad to see them and vice-versa. She is more confident getting out in public, going to restaurants, etc.

She has had a little bit of edema in her legs, just a little, but we will watch it and increase the diuretics as required.

Chris has been in touch with another patient from Ohio who went through the treatment just prior to us and we hope to meet them for a dinner in Boston when he comes back for his 6 month evaluation. There is a small community of patients, and it is important to talk, share progress, give each other support, etc. Chris has also been watching another patients story unfold down in NYC with a blog like this one. As I have said, she has never read this blog, but really is interested in the others. This particular patient was in the hospital for the entire period, was allowed to go home after 5 or 6 weeks, and returned to the hospital after 2 hours at home with an infection. He has been in the ICU with severe kidney issues, but the most recent blog update indicates significant improvement. She prays for him like many of you have prayed for her.

Chris told me today that she is going to live her life as normally as possible between now and December 8th. She is "tired of being sick" and knowing that things are going to get very strange as we get closer to that date, she is going to just be "normal". Hopefully in a month or so she will be confident enough that she can go out without wearing a head scarf, and nobody will even know what she has lived through.

For me, I know that I have promised to post my own "lessons I have learned" list. To be honest, I really want to be in the right frame of mind when I do that. I have thought for many many hours what I would share in terms of my experiences as Chris' caregiver, medical advocate, husband, best friend, researcher, cook, driver, and bodily fluid cleaner upper. I am not sure how insightful I will be, but it will be a personal catharsis for me if nothing else.

As Sarah Palin would say, "let me give a big shout out" to all the incredible number of friends, family, and worldwide well wishers who continue to support Chris. You all get "extra credit".

Thanks to all you. The incredible number of cards sent to her blew her away. She is still getting some and they mean a great deal to her. I thought at one point they would have to back a truck up to the house. THANKS!!!!

p.s. my apologies for the slowness in the updates, but I will keep them coming up to and through our visit in December.

Thanks again - I can't say it enough.

Day 100 - Thanks to all of you

2008-09-29T07:35:00.002-04:00

Just a quick update (I will write more in the next day or two), but I wanted to say THANK YOU to all of you who took the time to send something to Chris. It was absolutely amazing to see the outpouring of love, the incredible comments of support, the variety of people, and the resulting stunned reaction from Chris. She was so pleased to read each and every card and realize that so many people were rooting for her. From high school friends, colleagues at work, neighbors, friends of parents and siblings, friends of our daughters away at college, the cards were fantastic.
Chris also received cards from people she did not know, some of whom have been following her journey on this blog, some of whom are going through the same journey themselves. Of course, the cookies, the cakes, the balloons, and the anonymous flowers (beautiful) also were well received and greatly appreciated by Chris. She honestly was shocked at how much people cared and are praying for her recovery.

Chris had a great day. To see the progress from as little as a month ago, would surprise many people. Just 3 months ago, Chris would eat a half slice of toast and a small glass of OJ and 30 minutes later she would lose it. She could not walk 40 feet without having to stop and take a rest, and she was almost in a deep sleep while listening and watching things going on around here. I was honestly scared that we would never get to see again the incredibly strong and fun-loving Chris that we knew just a year ago. Yet, she is really coming back strong in the last few weeks.

So today, we start a new chapter in this journey. The focus now is to:
-get strong and conditioned and feeling physically able to do almost anything
-get back to a normal routine and lifestyle
-get mentally prepared for December when we go back to Boston Medical and come to the next crossroad on this path.

As I have told her many times, her new full time job is to focus 100% on herself.

I cannot thank you all enough. You have been amazing and you have made a huge difference. Trust me. THANK YOU!!!!

Quick Update

2008-09-26T09:45:00.003-04:00

Things are getting better with Chris all the time:
-She walked 3 times yesterday, combined over 6 miles. Lot's of exercise is critical and she is doing a good job with it.
-She is driving alone regularly, running errands, etc.
-She has been sleeping better and better - probably due to more exercise.
-Her blood pressure has been remaining strong (concern is that it is low) so she may be able to reduce some of that medication. The pharmacy warehouse of medications she has been taking since June has been reduced considerably, but still about 15 pills a day at this point.

Chris' sister Debbie, who managed the home front this summer while Chris and I were in Boston, is flying up for the weekend. She has not seen Chris since the end of August, so it will be a good comparison point.

The cards have been coming in - Thank You! They put a smile on her face everytime she opens one up. We are going to enjoy the weekend, celebrating the 100 days. Your cards are part of that celebration.

Day 95

2008-09-23T21:15:00.002-04:00

The support group meeting in Boston this past weekend was very interesting. Chris and I met about 6 other couples who were a combination patient/caregiver, each with their own unique story and road travelled. We met people who had a stem cell transplant with High Dose Chemo 3 years ago, with great success, to another man who had undergone two transplants with no success but is now undergoing an oral chemo regiment, to a third man who was rejected for any transplant due to advanced progression. There was a guest lecturer, a neurologist, who described the symptoms when this disease manifests itself in the autonomic nerve system. This "neuroprathy" is very debilitating where the proteins effectively kill the nerve cells. It does not take much imagination to understand the consequences of what this does to the body. Chris has has no signs of any nerve damage at all (only heart and lungs).

We both walked away from that session with some reconfirmations of what we knew before:
-we were very early in identifying this disease compared to many who went from doctor to doctor with no diagnosis
-Chris was in the majority of sufferers. The normal patient is male over 60 years old, so she is clearly unique. Doing the math, with only 2000 cases diagnosed annually, being a much younger female probably makes her one of 300-400 in the entire US who has this.
-As with most diseases, there are very passionate individuals who lead national advocacy organizations, including lobbying activities, fund raising, tracking clinical trials, etc. These people do unheralded work but make a tremendous difference. They are well respected by the doctors treating this disease as they work tirelessly to fight the battle beyond the hospital walls.

Chris has continued to increase her walking, going a good 5 miles every day. She comes back very tired, and her legs ache, but it is just incredible to see the difference from just two months ago. She has to fight the urge to just relax as her body screams "enough already", but she has been diligent. This is her full time job right now - get herself as strong as possible. She also had a very busy day today as she drove 3 1/2 hours (7 total) to visit Abby. She was the passenger, as her great friend did all the driving, but it was all worth it to go and let Abby see her. Just in the month since Abby has been at school there has been so much progress. it was important for both of them as Chris was unable to go to the drop off back in August. She got to see the dorm, the room, meet the roommate, etc, and this was very important for Chris. I am sure she will be very tired for a day or two, but again, all worth it. Thanks to Sally!!! It meant a ton to Chris.

We now have another incentive to get very health very quickly. Melissa has applied and been accepted for a semester abroad in Perth, Australia. She will go in January and attend classes as part of an Australian nursing college. Chris has always wanted to go to Australia and I would be thrilled to take her there. I have some great work friends in Sydney who have hosted me in Australia many times, and have also been very interested in her progress, and it would be great to get together with a very healthy wife. Much to plan, but a great objective.

Today is Day 95. As Chris has never read the blog, despite our original intent to have her post to it, it will still be a great surprise to her if you could all send a congratulations card that would arrive on Saturday, Sept 27 ( or thereabouts). A simple card whether from Holliston, NJ, Texas, Nevada, Europe, or Asia, just to let her know how much support she has had would be a fantastic gift. It would give her an incredible strength that she will need to get to December and the very scary results of the hell she has gone through. Just wish for a "Complete Hematological Response" (HCR) and I know it will make a difference. The address is in the post below this one.

This woman is made of steel, and has fought an incredible battle. She is both stoic and scared, as we all are, but with the amazing support she has received from all of you, she knows she has hundreds of people personally rooting for her. At day 100, a major milestone, she will have successfully climbed a very steep mountain. From that peak, we can all pray that she can see far far into the future for many years. I'm looking forward to letting you know how surprised she is.

Thanks to all!!!!

Support Group

2008-09-20T08:08:00.002-04:00

Today Chris and I head back to the Boston Medical Center to attend out first support group meeting. These are different than the weekly meetings held in the center, but instead are larger meetings, almost conferences, with guest speakers, updates on clinical programs, etc. We have no idea what to expect, but it is important that we attend. I think all diseases have some sort of Advocacy or lobbying organization, and annual conferences, etc. This one is so rare that I believe the organizing body is very small. We'll know more when we get there.

Chris continues making forward progress, with some concerns. In the last few days she has really picked up the walking, going over 5 miles over two sessions. She comes back exhausted, but without question, she is reconditioning herself. It will be along time before she can get back on the tennis court, but I am sure it will be possible if she keeps her personal determination in place. She also has been driving by herself, running errands, etc. It seems so easy, but it really has been a major step for her. For almost 4 months, she has done almost everything with someone: her sister, her amazing friends, her parents, or myself. She is still very reluctant, to be exposed to a germ infected world, and has to wipe down everything prior to touching it, but clearly her ability and willingness to go it alone is a major milestone. Hooray!!!

I was in San Fransisco for a few days this week while Chris held down the house with no issues. We've come a long way from me having to sleep in that chair in the hospital many nights in a row. As I said in the last post, her voice, her stature, her demeanor have all improved. In fact, just like a newly seeded lawn, there is clear evidence of hair growth. It is thin and very short, but the evidence is clear - the hair is coming back.

Just another reminder/request. September 28th, next weekend, is Chris' 100 days of her new life. She has been through a life threatening period, that has taken an incredible bite out of her. Yet she has come through this very well so far. A note of congratulations from you would be just incredible. I have so many friends at work from around the world (Sydney, Singapore, France, Spain, UK, etc.) who have asked about her, her old high school friends from Belgium, friends of both of our parents, friends from her home town, friends here in Mass., it just is amazing. If I could ask you to send just a congratulations card from you, your spouses, your kids away at school, from every and anyone who is aware of this incredible battle she is fighting, it would give her a jolt of strength she is going to need to get to December 8th (the next diagnosis date). If you have never met her, but have been praying/thinking/watching/wondering. then it would be even greater that you could spend the few minutes to send a card. She has no idea (it is unfathomable even to me) how much love has her been sent her way.

Chris Wright
20 Rolling Meadow Drive
Holliston, MA 01746 USA

Someday I will figure out how to thank everyone, but for now I hope you have a piece of knowledge in your heart that you all, everyone of you, have had a hand in her success.
THANK YOU!!!

Clear Progress

2008-09-15T12:35:00.002-04:00

Almost everyone who has seen Chris recently has agreed that she has visibly improved during the last week or so. You can hear it in her voice, her stride is quicker, her demeanor more positive, everything is looking better. She has a new found confidence that just a few weeks ago did not seem likely. A few milestones:

-Chris drove this past weekend. No big deal for most of us, but a really clear indication of her mental alertness, her reaction time, her overall confidence. This will help unleash her a bit. She drove with me, so now we need to get her to drive alone.
-She has been walking more and more, and at a good pace. Today she did about 3 miles, with many hills. Just a few weeks ago she was doing an easy mile on the high school track.
-Chris and I went to Burlington VT this weekend to visit Melissa. As such, we stayed at a hotel, we went out for dinner and breakfast, ran some errands, all the time with Chris really feeling good about getting out. Of course she is really careful about avoiding sick people and not touching door knobs, etc.
-Friday night she and I went to a dinner at the Four Seasons in Boston - very fancy, live orchestra, the works. Chris looked beautiful. She was very engaged in conversation, she ate well, and to top it all off, she actually danced with me - albeit a very slow dance. A few months ago, I was not sure if I would ever have the opportunity to get to dance with her again, or even go out in public, but it is clear she has turned a corner. I can't tell you how happy I was to have the chance to spend a night out with her. It has been a hell of a summer, but the chance to go out on Friday night was so important, and she did great! My parents and siblings were all there and they were all excited to see the progress.

Chris' weight today was 103, still very low but up a pound. Her blood pressure has been pretty steady, averaging about 100/75, so we will reduce one more pill that keeps the pressure up.

As I have stated previously, Sept. 28th will be the magical 100 days since her stem cell transplant. This represents a major milestone. The biggest is that this is the end of the high risk period and when they stop tracking mortality from the stem cell transplant. The doctors have stated that any issues after that point are not related to the transplant and the chemo directly. Of course this has no relationship to the actual disease itself, which we will go back to Boston in December for that testing, but it does represent success that the high risk transplant and chemo were survived.

I am bold enough to ask any readers to send a Congratulations card to Chris so that it arrives on Sept 27th or thereabouts. (The 28th is a Sunday). A simple card with your good wishes would be fantastic. No flowers, no gifts, just a card. I do not think she knows how many people have been praying for her, have asked about her, have read the blog, but she would be amazed.

Address Info is:

Chris Wright
20 Rolling Meadow Drive
Holliston, MA. 01746
USA

I'll add this again in a later posting as a reminder.

I am also going to declare that Sept. 28 is the end of all of the amazing help from friends and neighbors. The meals have been absolutely incredible (trust me, I am the one gaining all the weight), the offers to run errands, the shopping, etc. -they have all been great, but we all need to get Chris back to complete self-reliance. By then she will be feeling very strong and she needs to know that she can live the life she had before she got ill. As well, although I know we all pray this is not needed, many of the patients go through this a second time 12 months later. We'll need your help then, so it's time to give you all a break.

So, to summarize, it is clear that the last week has been a significant upswing for Chris. Your help, prayers, and interest have been remarkable. Everyone has been a part of this progress - Thank You!!

Visit back to the Amyloidosis Center Yesterday

2008-09-09T17:17:00.002-04:00

Chris and I went back to Boston yesterday based on the concern of her primary last week relative to concerns with a lung X-Ray. It was a bit streange being back in the center as we had not been there since we left in Mid-July, after going daily for 5 weeks.

We met with Dr. Burke who is a pulmonary specialist who we had met with prior to the treatment. Chris had another chest X-Ray (she may be radioactive soon) and we went through the comaprisons with Dr. Burke. I was prepared for some bad news, that is how you start getting in this situation, and was afraid we might see worsening in the lungs. However, that was not the case. He said things looked "impressive". Of course we never now how they use these adjectives as we were confused about the word "unremarkable" a few months ago. Bottom line, he said things were progressing well, that she had increased lung capacity since June, and that he saw no troubling issues.

She also met quickly with the heart specialist and he indicated he felt very comfortable.

All in all a good report and encouraging. We also attented the weekly Amyloidosis Support Group and had some good conversations with new (deer in the headlights) and very experienced (multiple stem cell transplants and oral chemo) patients. We always learn a great deal from others and now feel that we can help others based on the progress Chris has made.

I am currently in Zurih with limited internet access, so will keep this short for today. Chris' Mom is at home with her. She also heard from a frind from High School today - which really made her happy. Thanks to all of you reaching out.

More updates to come based on test results!!

Some Bumps, But Moving Forward

2008-09-05T16:42:00.002-04:00

This week Chris met with her primary care doctor. We discussed a number of areas:

-there is clearly some fluid in her lungs that has caused an increase amount of coughing, particularly when laying flat. Her doctor had a chest x-ray done which confirmed the fluid. They also found a "patch" (some kind of dark area) on one of her lungs that they need to understand more. This could come from: 1) the chemo 2) some kind of a fungus or 3) something that has always been there but not noticed before. her doctor contacted Boston Medical and they agreed that Chris should come in and meet with the pulmonary specialist who first evaluated Chris as part of the three day diagnosis in May. They also put her on an anti-biotic, hoping it is something that just goes away after treatment. So, back to Boston for a day. We didn't think we would be there so soon, but as always we want to be very aggressive.

-Chris has also not been gaining any weight, and in fact has lost a few pounds (now at 102). This is troubling of course. She really has been eating well. Some of the weight gain a few weeks ago may have simply been fluid, which has now been flushed out with an increased dosage of the diuretic, but in any case she is not gaining weight. Her doctor told her to continue to eat high calorie meals (a complete change from how she ate before) and try to have an Ensure or Boost (high calorie drink) between meals (that has not been well received by Chris as she really hates that stuff).

-We did reduce some of the medications and are continuing to eliminate the original fistful of pills pretty significantly. Some she will take for many months, maybe years, but clear progress on that front.

-Chris' hair is slowly starting to reappear. You still need to wear sunglasses when you are outside with her as the sunlight reflects brightly off her scalp, but there is clear peach fuzz. This is progress.

-Her stamina is clearly increasing. Her walking is much quicker, she is hardly taking naps (it used to be three hours naps when we first got home), and she is generally sleeping well. I see clear signs of general improvements.

-We do have a big formal dinner next Friday in Boston and she had to go find something new and nice that fit her. I have no idea about sizes, but I think she said she is a size "zero" - but not sure how that works. But she did go shopping with her great friends and it was a sign of increased confidence and activity.

-I will also be gone for three nights next week (Europe) so this will be her first real lengthy period without me. For years she used to ask me "don't you need to go on a business trip or something"? when I was hanging around too much and cramping her style. This will be a good test of her getting back to some Independence (not to worry, her Mom will be here).

So we need to have this lung issue addressed, we need to get her to gain weight, and we need to get her to this dinner next week (a real big step). But as always, things are getting better and better slowly but surely.

Good Week, But Still Weak

2008-09-02T08:03:00.002-04:00

The past week has been both good and bad for Chris. She has been walking more and more and yesterday walked a portion of her old daily route at the state park with the dog and me. This was probably about 2 miles with some very steep hills and difficult paths, but she was very glad to get off the high school track and get back into the woods. This also allows the dog to do her daily swim and get some exercise chasing squirrels.

Chris' weight is at a standstill however. She was 103 yesterday and this is very surprising as she really has been eating very well (or so it appears). She has lost the nausea she had over the previous week and generally is feeling better, although frustrated at the lack of weight gain. This and her lack of strength are very frustrating to her. Yesterday she was unable to husk an ear of corn, and this really gave her an indication as to how weak she is. She will pick up a regular routine with the physical therapist this week and is hoping to get permission to go back to yoga class (with an understanding that she uses her own mat, wipes down everything, etc). I think this will help her out a great deal, just getting out and making progress on rebuilding her strength. One objective she has for this week is to start driving again. She has not driven since May and is concerned she does not "have it all together" yet to start driving. In my opinion she is ready and able, so I think it is more of a confidence thing than any other concern.

The past week was a tough one emotionally for Chris. With Abby going to school as a freshman and Melissa starting classes in Burlington, she really wanted to be there for both of them. She kept saying "what kind of Mom doesn't see their freshman off to college", but we all knew that the move required alot of walking, carrying, set up, etc and the focus needed to be on Abby. This was really tough on Chris, but it was in fact a very long day and we made the right decision. We will go visit both daughters as soon as we possibly can, while also taking Stephen on more college visits.

We see Chris' primary physician this week and are looking for more positive signs from the blood work and hopefully some reductions in her medications.

So overall Chris is getting stronger and feeling better. She still has bouts of much sadness and frustration, but she is anxious to get back into a normal pattern. The kids have been fantastic and the friends and neighbors just continue to help out so much with driving, walks, dinners, etc. These offers help Chris to focus on herself, get stronger, and at the same time stay socially involved. My hope is that by the end of September (the 28th to be exact), she will be totally self sufficient, able to drive, workout, run the house, etc. This will have only been possible with the incredible help of others. In fact, she told me the other day she can't wait to give back to others who could benefit from her help. This is a real sign of progress I think. Thanks to all of you.

Monday, August 25th

2008-08-25T06:40:00.002-04:00

Over the past week, Chris has continued to work hard at increasing her strength. She has been walking everyday at the high school track and then again in the evening after dinner. Each day she gets stronger with a general positive trend. She still has what most people would consider a very low energy level and it is an effort everyday to go perform an exercise that we all take for granted. Still, she is reconditioning herself with a passion to get stronger everyday. That said, I have learned to equate the day to day changes like a bull (positive) stock market. Some days things go up, some days they go down, but the general trend is up. On her down days, she has some nausea and some coughing due to what feels like some increased fluid in her lungs. We will call the doctors today and ask them what they think we should do about this new cough.

Her weight has kicked back into gear and she is now at 107 pounds, but some of that may be due to increased fluid retention. They told me at one point back in June that she may have been carrying around as much as 10 pounds of excess fluid in her body. This was primarily in her ankles, abdomen, and lungs. Her ankles and abdomen seem to be fine, but the lungs and coughing are a bit of a concern.

We go back to her primary physician next week for another check-up, and will see if we can reduce further any medication. As is easy to imagine, Chris is "really, really tired" of taking a fist full of pills throughout the day. She actually has reduced these significantly, but still has a large number to take. We sometimes have to remind her that these are good things, not bad, but it does become a daily grind.

Today is day 64. It really has been an incredibly strange summer. Just after school let out and graduation, we started this ordeal, and now we are down to a week before things get back into gear with Melissa starting school next week, Abby going to school on Friday, and Stephen starting next Tuesday. Chris has felt cheated out of her summer, but it is one we all pray will have been a small price to pay for a long and healthy life.

Chris hopes to try to drive in a few weeks. She is concerned that she doesn't have the strength or the reaction time, but she is anxious to get a little independence. In the meantime, her friends and neighbors have been great. They have taken turns at walking with her, still making some meals, and running errands with her. The daily walks are incredibly valuable. They get her out of the house, they give her some good conversation, and most importantly they push her to exercise and get stronger. I cannot thank her local friends enough as to how much this is appreciated by both of us. The combination of social interaction and exercise is very critical. Despite having their owns lives and daily burdens, the willingness to help Chris has been invaluable.

I have been thinking lately how this limitation of life must impact Chris' attitude. As I think back, Chris has not felt well in over 6-7 months. Imagine everyday waking up, with little strength, not being able to do what she wants, often feeling nauseous, and fearing the future. Most of us get sick for a few days at a time and start feeling normal again. Chris has not felt normal through much of this year. As such, I grow more and more amazed at the incredible internal strength she must have. Day after day after day, this tough lady wakes up, looks at her hairless head and thin body and fights a battle that few can imagine. As I have said before, she is a rock!!

Remember, September 28th is Day 100, a very special day. I will be bold enough to ask that everyone recognize her strength with a card on that day. More on that next month.

More updates next week. Thanks to all for the amazing support, prayers, and interest.

Chris Update - Monday, Aug. 18

2008-08-18T12:08:00.003-04:00

The past week has been a relatively good one for Chris. She has been up in NH for the last 5 days and enjoyed the opportunity to continue to walk regularly, eat well, and sit by the lake. A quick update on her overall status:

-Her weight remains the same at about 102, which is hard to understand as she has been eating well.

-She has increased her walking to about 1.5 miles in the morning and another brisk walk (but less) in the evening.

-Her blood pressure has improved and is now about 104/85 on average - this is very good.

-She does have a cough that troubles her at night when she lays down. This is indicative of some fluid in her lungs, which is what she had back in the month of May. I contacted Boston Medical and they are not as concerned as I am, but we will watch this over the next few weeks and determine if she needs an lung x-ray to understand the extent of any fluid build up.

-Chris' demeanor has been both up and down. Her sister and I have been pretty hard on her about pushing herself, taking her medications, not dwelling on the negative, etc. She usually has been positive, but as I have explained she lapses into a sadness every once in awhile

The big change coming is that we will get back to a more normal, and difficult world soon. Chris' sister Debbie will finally get to live a life where she focuses on herself, her husband and kids, her own life. She has been in Boston away from her own life since early June. Now she is heading home from NH tomorrow, with her 2 boys. The only way I can describe her is as an "Angel". She has been an angel from above who has come to help Chris, me, our children, everyone. She has been focused 100% on Chris or our children for over 2 months, without a complaint, without any frustration, and without a seconds hesitancy to help in any way. She has been an incredible gift. More on Debbie in a later post, but it goes without saying the impact she will have had on her sisters life.

One major milestone coming up is Sept. 28. That will be Day 100 from Chris stem cell replant on - her new birthday. From a medical perspective, this is a very important day as it represents the closing of the risk period for the stem cell transplant and the high dose chemo. June 20 was her "new birthday" and Sept. 28 will be her 100th day. I may ask all readers to hold that date, and send a card, give her a call, stop by if local, etc. I'll give address info later, but I really think it would be great to show Chris, at the completion of this milestone, how much incredible support she has behind her. So if you think about it, make a small note on your calendar.

With Melissa starting school in Burlington, Abby off to school on 8/29, Stephen starting back just after labor day, and Debbie back in NJ. Chris will certainly face a different reality than she has had all summer. She will have to walk daily, do some shopping, do some of the housework, etc. - all on her own. All steps in getting back to a normal life. She will often be exhausted, sad, and frustrated, but as we all know, she is a very tough fighter who is 100% determined to get her life back.

I keep trying to update the blog more often, but is has been a bit crazy between going back and forth to NH, Colorado, MA, NY, etc. I appreciate your patience as you all have been so supportive. Thanks for your continued patience and incredible love and support.

Update - Thursday, August 7th

2008-08-07T07:51:00.002-04:00

First my apologies for the delayed update. I realize how much support, and therefore genuine interest for Chris exists, and I have been negligent in providing updates. It was much easier back in the apartment when my focus was 100% on Chris, but back at home the normal family demands pile on quickly - Again my apologies.

Chris continues moving forward, with generally daily good days. We are back from NH after 6 days and while up there, she walked daily, ate well, slept generally well and kept busy. She took a few boat rides when we took the kids wakeboarding, went for a few drives around town, and visited my parents a few times. Everyone who sees her after not seeing her for a period of time says,"wow, you look much better than last time I saw you". She of course does not see this, and in fact remains very frustrated that she is not seeing the increased energy and alertness that she hoped she would have. She has been diligent about doing her exercises, but she still feels exhausted by the afternoon. Yesterday was a very good day for her. She did many chores around the house, got the kids organized, paid some bills, etc. She doesn't see her own improvement and as such her morale suffers sometimes. Her sister has been good about not letting her feel down and giving her a kick when she has a "personal pit party" as she calls it.

Unfortunately her weight has not really increased much. She remains at about 102 pounds, which is very surprising to me as she seems to have a good appetite. Compared to early July where she was unable to hold down a half slice of toast, she eats full, albeit not large, meals. Her edema has really been good, with very little swelling in her ankles and her abdomen, so she has lost this water weight which may balance out the increased gain from eating.

One thing that Chris is depressed about is her belief that she is failing as a mother. Our kids are the single most important thing to her, and she is unable to help them significantly. Three examples: I took Melissa to the emergency room late Tuesday night due to severe abdominal pain. To keep it simple, it looks like a gallstone which will be confirmed tomorrow. Chris really wanted to go with Melissa, but as it was 11 pm and we did not get home until just before 3am, this would not have made any sense. Yet Chris feels an obligation to be with her kids when they are ill, like all mothers. Abby is having some oral surgery today (yes, we are a medical nightmare of a family) and Chris wants to be there before and after. But, again, Chris cannot go into the highly infectious hospital environment, so I will take her. Chris has always been taking kids for medical appointments and now she can't - very frustrating for her. Finally, tonight I fly to Colorado Springs with Stephen where he will compete in the US Junior Elite Triathlon Championships. This is the US national championship title for 16-19 year olds and Chris, Stephen, and I had planned this trip since last January. Of course Chris can't make the trip and she was in tears about it last night. I keep trying to remind her that her only job right now is to spend the next few months focusing on getting back to 100% health, so that we can in fact do all the normal things for the rest of her long life. This is just a relatively short period of time when she gets to be totally focused on herself - yet despite my nightly words of encouragement, she still sometimes falls of the bandwagon and gets depressed.

But I don't want to paint too bad of a picture. Chris is getting stronger, more alert, and more active everyday. Her walks, physical therapy, meditation, and chores are becoming part of a daily routine that will position her for a few weeks from now where she will not have her sister and kids at home during the day. She remains a very tough and strong woman who is absolutely committed to beat this thing.

The many cards, phone calls, visits, and prayers have really helped Chris to stay positive. We are both incredibly appreciative for how much love and support has been sent her way. She is soaking it all up and that is what is making her stronger.

I'll do better on the updates!! Thanks to all!!

Monday - July 28

2008-07-28T20:42:00.002-04:00

Just a quick update as I have many requests to keep the updates coming. Chris continues to feel stronger and do more. She has been going to the track daily, has her physical therapy two times per week, and even speaks with a stronger voice and a bit more confidence. The medical team called today to check in on her and they went through a number of issues, including her weight (actually down a few pounds due to reduced swelling), her continued insomnia, and her mental state. The doctors response was one of guidance to be more patient. They reminded Chris that she went through an incredibly difficult and risky procedure and that she needs to acknowledge this reality. Her life will be different for another few months, and she needs to accept this. To me the big question will be how she is doing in September. Will she bored at home, unable to go into big crowds, will she get back into a regular routine, will she focus on nothing but building her strength back. I think the fear of not knowing what the future is going to bring is probably the most difficult thing to acknowledge for her and all of us right now.

We did hear some very sobering news this week. One of the patients going through the Amyloid program passed away as a direct result of the stem cell transplant and the high dose chemo. I have written about her earlier (she had been taken from her apartment in an ambulance) and had been in intensive care for the last few weeks. Her husband and her brother were her caregivers and I got to know them as we ate lunch together. Chris knew her and I broke the news to her on Saturday. We knew going into this that there was a clear chance of death that could result. The impossible job of the doctors is to make the tough call as to who goes through the treatment and who doesn't. The chance of a complete response is 50%, but the chance of death is also very real. But the impact of doing nothing is the one nobody wants to face. We were reminded of this harsh reality this week. So far we have been very blessed.

Chris' siblings have all been here the last few days. It has been great to have them all helping, keeping Chris busy, reminding her of how important it is to stay positive. She has also been getting many calls and cards. She is starting to realize the incredible support she has from an incredible network of friends. An amazing number of folks at my work ask about her regularly, have put her on their prayer list, and have sent an incredible amount of positive energy. Friends from my high school and her high school have reached out, some she has not spoken to in years. Neighbors who moved away years ago, friends of her parents, it just goes on and on. I cannot state how much this has meant to her and me. The unbelievable love fore this woman is almost impossible to comprehend.

I will get her up to the lake after a Doctors appointment on Thursday. I will provide an update then. Again, I have no idea how to thank everyone, but I have learned alot about being a good friend, a good sibling, a good colleague, and hopefully the best possible husband.

A special thanks to Amy who reads the blog to Tim regularly. It means a great deal to him!

Friday - July 25th

2008-07-25T19:23:00.006-04:00

Chris continues to get a bit stronger every day, with increased weight, increased stamina and less nausea. While she is still very weak compared to what she started out at, she does exercise daily with walks, stretching, and some light weights. Her personal trainer now comes to the house twice a week and gives her a series of exercises that she does daily. She is very tired after these circuits, but she knows they are helping. The good news (for me) is that the personal trainer is a 50 year old woman, not the stud I was worried about.

Chris is up to 105 pounds, some of which is edema. Nevertheless she is clearly gaining weight and being much more active. Her blood pressure is still very low and she is on many medications for this, antibiotics, nausea, etc. She is starting to really detest this constant potpourri of pills, but she has no choice.

There are two issues she is struggling with. She is not sleeping well at all and is now in the habit of waking up almost every night for a few hours. She falls asleep quickly, usually wakes up around 2 or 3 am, and then sleeps again until morning. We are not sure if this is due to the medication, too long of a nap in the afternoon or just what. She is dealing with it, but it does mess up her daily rhythm a bit. My guess is that she is going downstairs ans secretly watching watching season 9 of Friends.

The other issue is one that concerns me much more, and that is a general feeling of depression that she still feels. I spoke to the medical team about that, and they indicate that this is not unusual, particularly in women (sorry, I'm just quoting). Chris occasionally feels like a "victim" or asks the question "what did I do to serve this?" During these low periods, she feels less energetic, less mentally sharp, and sleeps often (typical of course). This may be compounding the sleepless nights.

This week we will get her up to the lake house in NH where she will be able to really relax and not worry about the stresses around the house. I know she is anxious to get up there, but we needed to make sure the ride was ok and she did not have serious nausea.

Overall she is still very frail, but without a doubt she continues getting better and better. We will work on mental state and get her enjoying the rest of the summer. Abby leaves for school in 4 weeks, so it will be important that she knows Chris is doing well.

The calls and cards are greatly appreciated, they mean a great deal to Chris, so thank you.

Finally to the mystery e-mailer who sent the attached picture - please identify yourself.

Upward Trajectory - Sunday, July 20

2008-07-20T15:05:00.002-04:00

After a week of delayed updates about Chris, I am well overdue to the many many people who have expressed their support, love, and prayers for Chris. My apologies.

The news is good, in fact I believe very good, although she may not agree. Over the last seven days, she has:
- gained 6 pounds
- walked every day about 3 or 4 times for 15 minutes or so up and down our driveway (with an incline)
- had an actual appetite to the point of asking for seconds. She has a complete bagel, some fruit, OJ, and toast for breakfast usually, and a complete, albeit light, lunch and dinner. A few times this past week she ate so much that she complained about being completely full.
- She has had some relatively good energy and can talk on the phone with a strong voice.
- The most telling indicator of progress was when she lit in to me for about 3 minutes for pushing multi-vitamins (which she is supposed to take), saying she is tired of me telling her what to do and to let her make her "own damn decisions". Now that is the Chris we know and love.

So overall, some real good forward progress in the last week. But to be honest, there have been a few bumps. She still has the swelling in her legs, her blood pressure is still very low, and occasionally she stills feels nauseous. These have all improved or stayed the same. The bigger issue is her occasional sadness or depression. She sometimes has bouts of feeling very down, mostly due to frustration that she doesn't have the energy or the drive that she would like, as well as the feeling of not knowing what will be the end result of all of this (we will not find out until December). Her sister Debbie and I have tried to be good coaches, supporters, encouragers, and for the most part she is positive. Yet, she has really had some down moments.
We were told to expect some of this, and she knows any negativity is in conflict with all the positive vibes she has received from an incredible array of friends and supporters. She has heard from high school friends from her years in Belgium, she has heard from friends of friends, and she has been in the prayers of literally hundreds of places of worship around the world. She truly knows how many people are fighting this with her and when she is reminded of that, it really turns her around.

We met with her primary care physician ("family doctor" for those outside the US), and she hardly recognized Chris since she had last seen her back in May. Twenty-five pounds, no muscle mass, and no hair will really change a person. Yet, her doctor gave her some really good words of encouragement and prescribed a home Physical Therapist to start getting Chris back into shape. Chris can't go to the gym or even yoga class due to too much risk of infection. Thus a visiting physical therapist will be coming to the house. I never thought I'd be happy that a young stud with bulging muscles give my wife a private workout session, but hey, if that's what it takes to get her back into shape, I'm all for it (I think).

This coming week will be a transition from a very sedentary week of staying low, to one of being more active, increasing the frequency and length of walking (hopefully the high school track), seeing more visitors, and doing some things around the house that she enjoys, e.g. gardening, maybe some simple cooking, and reading. She did listen to a book on tape from my aunt and that worked out very well.

It is nice to be home. I have been working at my home office and that sure saves on the commuting time. More importantly Chris is much more comfortable at home. As well, it is easier to care for her at home, although maybe that is simply because she is doing so much better. I will associate the Boston apartment with daily routines of cleaning up vomit and other things, (e.g. hair), and things are much less messier nowadays.

So overall, things are clearly going in the right direction. We need to get her out of her occasional funk, and that will come in time as she gets more energy, gets back into her routine, and gets some time up in New Hampshire.

I can't tell you how much the support you all have provided has been. The phone calls, the notes, the flowers, the dinners, and more importantly the prayers and good wishes remain beyond belief. Just reading the blog shows you care.
Thanks to all.

I'll provide another update on Chris next weekend, if not earlier, but until then, please know she is doing better and better each and every day.

Sunday - Uneventul Day (so far)

2008-07-13T18:08:00.002-04:00

Not much to post today. Chris had an OK night last night with some issues, but slept in and has done pretty well today. She walked up and down the driveway 4 times, had some food, and actually gained vs. yesterday.

She is frustrated that she is "going to lose her entire summer", but with a little prompting she remembers that this is a small price to pay if she can beat this thing. Hopefully we can get her up to NH in a few weeks and she can sit by the lake and just enjoy the views, nice weather, and relaxation. Her goal is to be strong enough that she can join me when I take Abby to college at the end of August.

I think I will take the next few days off in writing the blog, as I am not sure there is going to be much new news. I'll plan on providing an update next weekend with a full report as to how the week went for Chris.

Let me just say how thankful we are that she has so many people interested in her, praying for her, and sending their love. Everyday she gets stronger and everyday she tells me how lucky she is that so many people are rooting for her.

Thanks to all of you...

Quick Update - Saturday

2008-07-12T20:32:00.002-04:00

Chris is very very happy to be home and had a relatively good day today. She did well most of the day, but is easily fatiuged and not too interested in eating. She did get outside this morning and even spent a minte watering her gardens, something she really enjoys. The neighbors next door stopped by to say hello and it was good for her to spend some time interacting, but she is more of a listener than a conversationalist right now. The nuasea medicine makes her very groggy.

She did get sick once, but that may have been due to the fact that she ate more at one sitting than she has in a long time. Our plan is to slowly reintroduce her to food she enjoys and not give her too much that it makes her ill. She was still down at 97 pounds today, and her blood pressure is also very low, so we will have make sure we stay on top of that.

Chris has been getting many cards and she enjoys getting these. I even received a card today with the Friends theme song playing when you open it (thanks Jonathan)- it's now permanently etched deep in my brain.

I think this will be a very slow week for her, with lots of rest, some new foods, keeping her nausea down and her blood pressure and weight up. She is very happy to see and hear the family again. Her sister Debbie has been an incredible help - beyond words. She must be as tired as Chris and I are, she just nevcr stops helping.

It is a bit strange being way out here in the country. Instead of sirens and elevators and all the various city noises, they have crickets and birds and dogs here. Being the city people that Chris and I are (after 30+ days), it's certainly different. Chris had a craving for a rueben sandwich from a certain place this afternoon - 10 miles away and 30 minutes round trip. I jumped in my car, and got it, versus taking the elevator down to the food court and deli. It wouln't have been so bad if she ate a little of it, but oh well.

Overall a good day, where she is very comfortable, slowly getting into things at home, trying to eat anything other than food I have prepared. I don't beleive the nausea is all my fault, but many of my friends are convinced of it. We'll know in a few days I guess.

July 11 - Home, Sweet Home

2008-07-11T17:35:00.002-04:00

At 1:53 this afternoon, Chris got her walking papers to come home. It was a long day at the center, with lots of instructions, warnings, responses to my long list of questions, and of course some hugs. We got home 35 minutes later and it was a fantastic feeling. I can't describe the feeling of sitting in the house, with the family, with the pets surrounded by our own belongings. Chris could not get out of Boston fast enough, she didn't even yell at me to "slow down" like she usually does.

She has detailed instructions on diet, driving, pets, immunizations, alcohol, gardening, and even diaper changing. Clearly the risk of infection still remains. The metric for mortality rate is tracked for 100 days post replant, and we are on day 21, so we have another three months or so of being extra cautious. Limited exposure, no crowds, no babies, no cat litter, etc. She will start an immunization process over the next 12-24 months (measles, polio, rubella, etc.) that will slowly rebuild her lost immunity system.

We go back for three days of tests on December 8th. This will be very scary as it is then that we will learn what the hematological response was. Complete, partial, or none. As I said, we want HCR. I have received some notes and emails that said HCR stood for: Hooray, Chris Recovered or Hot Chris Reborn. Her numbers today were:

Measurement / Chris / Normal Range / Result
WBC (white) /4.7/ 4.0 - 11.0 / Normal
RBC (red) / 3.6/ 4.2 - 5.4 / LOW
HGB (Hemoglobin) / 11.5/ 12 - 16 / LOW
Platelet / 116/ 150 - 400 / LOW but getting closer all the time

I asked the team today about 4 dozen questions that I had written down. All kinds of questions about Chris' heart, her lungs, average recovery time, reduction in her heart wall, edema, appetite, exercise, etc. Suffice it to say I drove them crazy, but I walk out very comfortable that I know almost all a civilian could know about amyloidosis. I have read every research paper ever published by the BU team and have arranged email alerts for any new publications referencing them or the center or the disease. I also had them print out graphs of all her blood counts over the last 30 days. I asked for a digital version (on a dvd or cd) of all of her charts , but they told me paper only (I tried).

Chris got home, sat on the couch, and could not describe how happy she was to be home. She lasted about 15 minutes before declaring she needed a nap and wanted to get into her own bed. I have not seen her move that fast in months. She really needed to fell at home, and nothing does more than crawling into her own bed. Our dog went crazy when we got home, but Chris can't pet her, so it drives them both crazy.

I've avoided bringing up the subject of Friends the last few days, but at this point, I kind of consider watching the seasons like running a marathon. I'm at mile 21 (season 8) and I can't quit now. I've come this far, if I can just hang on a little longer. My body may not hit the proverbial "wall", but my brain is pretty close to exploding. Chris and I are the little engine that can: "We think we can, we think we can" finish all ten seasons, but trust me, this hurts.

My kids believe that they are masters at getting their teachers "off topic". They are so proud when they get their teachers to wade into the weeds in class and never get to their lesson plan. Well, they have accused me of getting "off topic" here on the blog, and editorializing on some issue not relevant to Chris. I agree that has happened, but this whole experience has just been an incredible learning process which I have soaked up like a sponge. The disease, the medicine, the interactions, the hierarchy, the personal touches, the surprising lack of a well defined treatment approach, it is all just amazing. In the next week or so, I will expound on my Top 10 (like David Letterman's) Lessons Learned. This will be for the benefit of my own mental therapy, but maybe someone can benefit from it. Chris benefited from Jen's reaching out (see the very first post here about the newspaper article) to the press and TV, and maybe one person will benefit from the insane ramblings of my lunacy. See there I go again, off topic, sorry.

So Chris has completed one leg of her journey to health. It was an extremely bumpy ride, with many potholes, speed bumps, flat tires, ground delays, etc, but she has reached this stage of the long journey successfully. Not a single infection, no mouth sores, no trips to the ICU. She does have incredible weight loss (97 lbs. /44 kilo's), but now our goal it to fatten her up, build the muscle tone she had, and get her back to a more normal life.

More updates (and possible meanderings) tomorrow, but for now, tonight, at home, all is good for this wonderfully tough ladie. As I have said before, she is my hero! And we are home, sweet home!!!

Thursday July 10 - Big Day Tomorrow

2008-07-10T20:06:00.002-04:00

Today was another day off from the hospital for us. Chris' numbers had been good on Wednesday, and they told us that tomorrow will be a big day. Thus Chris slept in, got a few walks in, made a few phone calls, and took many naps. She also suffered from nausea but only dry heaves, no lost meals. They gave her a new medicine that seems to push things through her stomach quickly before she can lose it. That said, she continues to get thinner and thinner. She has lost an incredible amount of muscle mass and to be honest, looks very frail. She has a great deal of work in front of her to rebuild herself. Yet, there is no question that she will - she is being tough, positive, and determined. Chris' goal is to beat me in tennis (hardly ever happend before any of this, but) in the next year.

Tomorrow we go through a series of tests, instructions, and a huge amount of paperwork. They will give her a final medicine protocol, instructions for frequent visits with her primary physician. schedule follow up appointments at BU, etc. Then Chris wants to get in the car at the end of the day, and go directly home. Home means back to Holliston, to her own bed, her own gardens, her own things. We may have to be back for another visit next week, but that would be a day trip.

We do have the apartment until next Wednesday, so the kids may have their friends into Boston for a night or two on the town. It would be great to walk to some great restaurants, sit in the park and read the paper with a coffee, stroll around the city, etc. Someday we'll come back for a month, with Chris totally healthy and celebrate the incredible success of her recovery.

I won't go into percentages here (I can quote them to the tenth of a percent), but what we are looking for is what is called an HCR - hematological complete response. This is where the light chain proteins (amyloid) are no longer being produced by the defective plasma cells. Partial response or no response are other outcomes, but all we are thinking about is HCR. This is my new favorite acronym, HCR, with C being the most critical letter. Everyone who has prayed, sent cards, flowed their positive thoughts Chris' way, have all made a huge difference. The message you were sending made the difference. Chris went through stem cell replant and the chemo with flying colors (and some flying vomit (sorry)). Now what we need is the same wish for HCR!! We will not know what the response was for another 6 months, but if we all think and pray HCR, it will be huge.

So, the journey continues, it doesn't end tomorrow, or in six months, as she will be tested always. But the journey has been a good one so far. We have a very tired woman, who feels beaten up, looks even worse, and yet she smiles for me everyday. The smile tomorrow when we drive into our driveway will be a great one to watch.

I know I have an incredible number of people to thank for their support. I don't know how and I don't know when to say thanks, but the list is amazing, I am awe-struck. Over the last week, over 960 people have read this blog. The love, interest, and support for Chris has been beyond comprehension and is a testament to the love she has been sent. Despite my editorial ramblings, poor writing skills and bad grammar, you all overcome that to check on Chris. Again, just amazing.

So tomorrow is a big day, I'll update the blog with how it went, and how glad she is to be home.
Have a great day, I know we will!!!

Wednesday, July 9 - Land Ho!!

2008-07-09T19:34:00.002-04:00

Without having to squint to hard, there is clearly a light at the end of the tunnel today. In fact it is big and bright, we can see it plain as day. Chris got the green light we have been waiting for and we are heading home this weekend. As Marv Albert would say: "YES"!!!

Overall things are good enough and Chris successfully lobbied for a discharge and she got it. So unless things change dramatically, we'll be home by Saturday. We most likely will have to come back for one day next week, but that works for us.
Her numbers are as follows:

Measurement / Chris / Normal Range / Result
WBC (white) /5.8/ 4.0 - 11.0 / Normal
RBC (red) / 3.2/ 4.2 - 5.4 / LOW
HGB (Hemoglobin) / 10 / 12 - 16 / LOW
Platelet / 102 / 150 - 400 / LOW but significant improvement

So the white blood cells and the platelets were the key measurements, and they are either within range, or rapidly going in that direction. Clearly, the stem cells have grafted into the bone marrow and Chris is now producing the blood cells on her own, after a long 18 days (clearly these stem cells took their time kicking into gear).

They also removed the chest catheter today. Very interesting. The doctor who did it said that I could stay if I promised not to pass out. I told her she must be kidding, as not only did I want to watch, but I wanted to actually do the removal procedure myself. I guess I have not fully earned my place on the medical team as I got a pretty direct "NO" to my request. The catheter is literally just pulled out. It is a 14 inch tube that went up to her neck and down her jugular vein into her right atrium in her heart. They simply pull it. I at least asked to keep it as a memento - no go on that request either.

So, assuming everything goes OK the next two days, we are "outta here". Five weeks have sometimes seemed like 5 months, but we have it so much easier than 99% of the patients who are hundreds or thousands of miles from their home and families.

So that is all good news. There is some bad news. Chris continues to experience vomiting. Nobody can get it under control, and she has no appetite. She keeps having a craving for something (raspberry sorbet, Hostess cupcakes, spaghetti), I run to the store to get it, and by the time I return and she sees it, it makes her vomit thinking of it. She is down to 101 pounds, and based on what she is eating and not keeping down, she will most likely be under 100 in the next day or two. The doctors explain that her reaction to the chemo is in her GI track and that this will eventually resolve itself. However, they also told her that she better start eating more or her recovery will be much longer as she won't have the nourishment to get into shape. Yet, as we all know, you can't make a nauseous person eat, no matter how much you plead. So, I guess the trick is to let the GI system recover on it's own, and not to push her. I do wonder if it is my cooking that is the problem here, and I am starting to take it a little bit personally, but in the end I know that no matter who cooks it, she just can't stomach it.

So we have a ticket for this weekend, her blood counts are up and we have prevented infection (the biggest risk) and now we just need to get her stomach settled and start the rebuilding process. We can't give a sigh of relief yet by any means, but we can certainly feel very positive about the progress. It has been long row to hoe, but I expect some blooming soon.

Tuesday, July 8 - Nothing Exciting

2008-07-08T21:26:00.004-04:00

Chris got the "day off" today from the trip to the hospital. As they saw some progress with the blood counts yesterday, I guess they wanted her to stay home and use the day to focus on getting some rest, getting a little exercise, and trying to get the nausea under control. I would say we were two out of three in meeting these objectives. Chris had some rest, with sleeping in and a number of naps. She went down to the courtyard and walked twice for about 15 minutes each time, getting a little sun and fresh air. She has to wear a hat (avoiding a sunburned scalp) and her surgical mask, so she really doesn't enjoy it, but knows she needs to push herself. Unfortunately, as if on schedule, she got sick again tonight. This has probably happened now every day for over two weeks in some form or another. Nobody should have to go through this like she is, but she remains stoic. To be honest, for the first time, I hear some anger in her voice. She doesn't let herself slip often, but I know this is really getting to her. She will fight through this emotional trough, but it actually takes energy to get yourself back up mentally, and she really doesn't have much energy. So tonight she went to bed early, just to get the day over with sooner I believe. But even after a tough day, she always says: "I know tomorrow will be a better day". So overall she remains positive, as she knows how important that is to beating this, but she clearly is getting some small dents in her armour.

One thing I have been trying to get smarter on is how the drug trials work. We were offered the opportunity to participate in a drug trial at the very start of her treatment, and we did some research and contacted some other experts (thanks Ron) and we decided against it. In retrospect, we think it was the right decision. But drug trials and the pharmaceutical industry are very interesting to learn about. Of course pharmaceutical firms are "for profit" entities, and they are vilified in the press all the time, but in the end, they do develop amazing cures. I honestly believe we take for granted the drugs that have saved lives across the world (think of small pox vaccines, hepatitis, influenza shots we all take in the fall) and some organization had to invest significant dollars to develop this drug, confirmed by a trial, produced with high standards, and distributed. So I will avoid politics here (again), but I wanted to use this off topic diatribe to simply point out a very interesting site online that lists all clinical trials for all kinds of diseases.

This is sponsored by the National Institute of Health:
http://clinicaltrials.gov/ct2/home

It shows all clinical trials going on worldwide that the NIH is tracking, and I believe it is a great example of a valuable government service (words I very rarely use together). You can search on almost any disease, e.g, amyloidosis, heartburn, bladder cancer (B, our turn to pray for you now), and even the heartbreak of psoriasis. If you are aware of a loved one who suffers from a disease, and are interested in learning about all clinical trials (closed, active or proposed), and their status, this actually is a very powerful site. I can assure you I know every single trial around primary amyloidosis, who is sponsoring them, etc.

OK, again, sorry for my meandering muses.

Chris and I are anxious for a very successful day tomorrow. I am counting on good blood counts, a rock solid stomach, and her smiles that let me know everything is all right. Nothing makes my day more than seeing her smile. Tomorrow I know I will see many of them.

Monday, July 7 - the saga continues

2008-07-07T19:27:00.002-04:00

рвота, κάνετε εμετό, vómito, Erbrechen,呕吐 This is Russian, Greek, Spanish, German, and Chinese for the english word vomit. I am tired of writing that word, I am sure readers are tired of seeing it, and most importantly Chris is tired of doing it. It just never ends. Last night after I posted the blog update and again this afternoon after getting back from the hospital she vómitoed again. She continues to lose weight and is now down to 105 lbs. Just 2 months ago she was 121. We spent a great deal of time talking to the medical team about this today. Of course we could not find a common denominator as to a cause (remember this is an art, not a science), but they have been very focused on it as they are truly concerned (although they explained that this kind of occurrence happens occasionally). Everyone has a different reaction to the toxicity of the chemo. Some people have kidney and liver issues, some have skin lesions or bone pain, and some have Gastro-Intestinal problems. So, the new protocol (medical speak for "course of action") is to add another anti-nausea medicine, increase some existing dosages, and change the diet a bit. Just listening to the team propose various options and debate pros and cons is a bit scary, but this is where the faith in their expertise comes in. I question everything, ask a litany of questions, read all about the prescriptions they are giving her, and challenge the team often. I do feel they they have come to respect my involvement as well as my perspective. Slowly I am earning my way into the decision making process, but never will I pretend I know more than I do (as is often the case at work my colleagues would tell you).

So we now have a new set of drugs and pills which we will try. The purpose of the new pill is to move things into her digestive system quicker and thus get it out of her stomach before she loses it. They have a pill for everything, we just don't seem to have the right ones yet.

I met with a family today where their relative has been in the ICU for 4 days now, with very serious issues. I keep hearing these horrific stories and I think how lucky we are that we are not in that bad of shape. Chris also spoke to her friend Jen today (the one who the original article about amyloidosis was written about) who recently went through her second treatment. She has a fantastic attitude, has a ton of energy, and serves as an inspiration to Chris. So Chris sees the bad side of this and thinks how lucky she is, then she sees the good side and knows there is light at the end of the tunnel.

The doctors said there were three things Chris needed before she could get the green light home:
1) stop the nausea 2) get the platelets over 100, and get the edema under control. They do not want to pull the chest catheter until both the nausea and the edema are balanced, as they use this catheter for directly giving her the medicine. This way she can't Erbrechen (see the first line) it.

Her CBC numbers today were:

Measurement / Chris / Normal Range / Result
WBC (white) /3.0/ 4.0 - 11.0 / LOW
RBC (red) / 3.1/ 4.2 - 5.4 / LOW
HGB (Hemoglobin) / 9.8 / 12 - 16 / LOW
Platelet / 67.0 / 150 - 400 / LOW

Chris and I asked them what they would guess the next 10 days might look like. They believe that they will solve the рвота by Wednesday (optimists maybe?), pull the catheter by Friday, watch her over the weekend, and let her go home the middle of next week. So in the worst case, we are here until Wednesday of next week.

The one issue that is starting to come up nightly is the recurring nightmares. Mine, not Chris'.
I keep having these dreams that these two guys names Chandler and Joey are my roommates and we live across the hall from these women who, well never mind. Just suffice it to say that season 10 cannot come quick enough. I am so close to κάνετε εμετό every time I watch it.

Chris continues to really appreciate all the support, prayers, love, and positive vibes being sent her way. She knows that an incredible number of people are thinking of her every single day and she knows that you are all praying for her. You have no idea how much it means to her that people from around the world are pulling for her. I think of it as a 24 hour vigil of hope for her successful recovery.

Thanks to all!!

p.s. apologies for those who don't have international language support enabled on their PC's. I have no idea what will show up when you read this on your PC.

Sunday, July 6

2008-07-06T21:53:00.002-04:00

Today continued the slow weekend approach with Chris sleeping in, watching some TV (yes, you guessed it) and taking some naps. Unfortunately she still has nausea and vomiting and it is really becoming a concern. In the middle of the night last night, and again this evening, she vomited. This will be the primary discussion tomorrow as we are both very frustrated that we cannot figure this out. She had a good breakfast today, but a small lunch and no dinner as she had not appetite and was fearful of getting sick. She can only eat extremely small portions and if I serve anything bigger than bite-size she can;t even look at it. Obviously this is not a good situation, so thus our focus tomorrow with the medical team.

The hospital did call and state that we did not need to come in today for tests. The white blood cells were good and the platelets "not bad". They may give her more platelets tomorrow. Getting the platelets up is key as only when she is producing enough platelets through her bone marrow will they remove the catheter from her chest. These two tubes have been hanging out of her for over a month now, so you can imagine Chris is getting pretty tired of them.

Chris' sister came in tonight with Abby and Stephen. I took the kids out for dinner (a real meal for me) and Debbie did some yoga with Chris. Overall, Chris has lost so much weight and has so little exercise that even yoga is tough, but she knows how important it is. The Doctor told us last week that Chris will feel very weak for a few months and that she will need to devote herself to getting strong again. This will take 6-12 months. Chris' objective is to be back on the tennis court playing a tough game by April 4th of next year - the date she was first called by her primary doctor.

It was great to see two of the kids for dinner. They all have been very supportive and call frequently for updates and to talk to Chris. I know all five of us cannot wait to get back together again. Being away from the kids has been very tough and we miss them greatly. Hopefully we will be out of here in the next 7-10 days and things will get a little more normal. Chris will need to take it very slow for a good month or so, but I know how tough she has been and I know she will dedicate herself to her recovery. But, I realize I am getting ahead of myself. First thing is to get this nausea stopped.

So overall a restful weekend for Chris, but one that has been frustrating as we try to understand why her body is still suffering from the chemo so badly. Hopefully we will get some answers tomorrow.

July 5th - Bald is Beautiful

2008-07-05T21:28:00.002-04:00

So far so good today, but I seem to post these updates a few minutes too soon. Last night after stating that Chris had a good day and posting the update, Chris ended up vomiting for the next 2 hours. This is the second night in a row that she has gotten so ill prior to bed. We are trying to figure out what may be causing this and really are not sure. It could be a potassium pill she needs to take that she has had trouble with. It might possibly be exhaustion, and just running out of energy. Potentially it is the 10 hours of Friends (now on season 6) that we have been watching (she found out it is was unplugged). Or it could be my cooking. But whatever it is, it is frustrating as it seems to only happen very late at night.

We went to the hospital today and had the normal battery of tests, but did not get the results right away. She does continue to have some serious swelling in her legs, and that needs to get resolved.

Chris has now hit the bowling ball stage. There is just about no hair left, and my technique of using the lint brush has been a stroke of genius. I am sure I could patent this idea. She has been a real trooper about this, and she makes light of it a bit. Yet my jokes have not been well received and I will avoid putting them here as to avoid later repercussions later.

Last night we watched the Boston fireworks off the reflection of the building we can see near us. A little strange, but we were able to hear them very well. Chris was unfortunately vomiting at the same time, so I am now sure this will be one of those July 4'ths we will not forget.

We will not see the Doctor until Monday (although we will see nurses at the hospital for the tests tomorrow). The late night vomiting and the edema are still a concern, as this is now Day 15 and the normal last day of the bell curve of side effects. She has been sleeping relatively well, but she lacks any energy during the day (not totally unexpected). We did connect with another family who's wife/mother has been in the hospital for a week and had to be taken from her apartment to the hospital via ambulance. She is extremely weak and is generally not doing well with the toxicity of the chemo. When we compare Chris to her, Chris is doing well.

Chris remains incredibly positive. We have mentally prepared ourselves for being home in 10 days. After the hospital visit today, Chris was up to driving a bit around Boston. Of course we know Boston very well, but it was good to get her away from the normal route and routine. We agreed to come back in for dinner often in the future when she is up to it. We have not been able to go anywhere since we moved here, so all we can do is make plans to come back when she is at full health and really enjoy the city.

Tomorrow will be a third day of rest. That will be very important for her. Hopefully I can give her a good meal and she can keep it down.

Thanks again for all the prayers and support. It continues to be the most important element of her success.

Friday, July 4th - A Very Slow Day

2008-07-04T21:11:00.002-04:00

Without question, this was the slowest, easiest day of the last 30. Chris slept in until about 9:00 AM, had a good breakfast, and read the paper. No rush to the hospital, no need to get dressed, no pressure to move at all. As such, it was a very relaxing day for both of us. Chris never left the apartment, she simply read, watched TV, and napped. I had to get some new prescriptions so at least I got to walk around Boston a little bit today.

As we did not go to the hospital, we have no idea about blood counts, but we will be back there tomorrow (Saturday) morning. Chris had a little nausea at various times, but no vomiting. I would say that the timing of this holiday could not have been better. We both needed a break. The tough part is that we were away from the family, with one in Maine, one in New Hampshire, and one in Vermont. We also are just 3 blocks from the Boston Esplanade where the Boston Pops put on their annual show and some of the best fireworks in the US are displayed tonight. Unfortunately, we are facing the other side of the city. So close yet so far. Even though we are on the 21st floor, we will not be able to enjoy it other than on TV.

Hopefully Chris will have a good day tomorrow. She still is eating very little, but if we can get the nausea under control, her appetite may increase. Day 14 has been a good one.

Thursday, July 3 - A RollerCoaster Day

2008-07-03T23:54:00.002-04:00

The Up's and Down's are frustrating as we continue to move forward. Chris had a roller coaster of a day today. She had a good appetite this morning and ate a good breakfast. Then while brushing her teeth she got lightheaded and actually fell in the bathroom. She fell against the shower curtain and into the bathtub. That was very scary and she doesn't remember how it happened, other than that she thought it was all in slow motion. All I heard was a scream and a crash, and I ran to find her almost passed out. As she has a low level of platelets, her skin bruises very easily and her back looks like it was beaten up. We were lucky, but from now on she has me as a personal escort when she is standing at the sink in the bathroom.

At the hospital today they did the normal lab work with these results:

Measurement / Chris / Normal Range / Result
WBC (white) /3.0/ 4.0 - 11.0 / LOW
RBC (red) / 2.9 / 4.2 - 5.4 / LOW
HGB (Hemoglobin) / 9.5 / 12 - 16 / LOW
Platelet / 67.0 / 150 - 400 / LOW

The white blood cells went from 1.7 to 3.0 and are recovering nicely. The hemoglobin went from 14 to 67, but two bags were added yesterday (Wednesday) so it is not known if the bone marrow is producing any or not. Yet, this is alot better than 14.

So the medical team was pleased with the blood counts. However, Chris has an unusual increase in edema (swelling) in her abdomen and ankles. Nobody was sure what is causing this, but they treat it with a diuretic (Lasik) which seems to work. Overall the numbers looked good enough that they gave us Friday (July 4th) off. No visit to the hospital at all, but back on both Saturday and Sunday. This was great to hear and everyone felt good about the progress. Chris ate a good lunch and we left at about 3;00 for the day, knowing we were heading for a long and very slow weekend. It had been a hell of a week with 4 nights in the hospital and we were glad to finally get a break. Unfortunately our confidence was fleeting. Within an hour of being back at the apartment, Chris started vomiting. Unfortunately these were not small little nausea episodes but some very bad vomiting, where I know she lost both of her previous meals. I felt so bad for Chris as she really had a good appetite, the doctors gave her a thumbs up heading into the weekend, and bang, a very tough night. She has been vomiting all night and is just falling asleep at midnight tonight. Thus a real roller coaster of a day, and very frustrating. But as always, she did not complain once, she just lets it happen and moves on.

Last night I cut her hair as close to her scalp as I could without nicking her skin. Good thing, as what was left has been flying all over the place today. Think of a pillow fight where the pillows break open, and you can imagine what is flying around the apartment. I bought one of those sticky lint rollers, and one or two passes requires a change in the sheet. She is wearing a hat or a scarf, but I keep telling her to go au naturale and let people think she is trying to make some kind of a fashion statement. I never thought my wife would allow me to play "barber" on her head, but it looked pretty good considering I had no idea what I was doing.

This will be a long weekend for Chris if we don't get the vomiting resolved. We are going to lay very low, catch up on some sleep, and try to eat some good meals. It will be a very strange July 4th, no parades and no fireworks, just a day devoted to getting Chris' body back in balance. It will be a July 4th that we will always remember and will reflect back on many years from now.

Our best wishes for a great July 4th to our US friends, and to all our friends around the world, thanks for checking in on Chris. She knows how many people are rooting for her.

It's a Marathon, not a Sprint

2008-07-02T20:23:00.003-04:00

Today was more forward progress, but Chris' blood counts are taking their sweet time in getting started. The stem cells that have been finding their way to her bone marrow need to kick in and start producing the white blood cells, the red blood cells, hemoglobin, and platelettes. Here is how she compares to that range as of today:

Measurement / Chris / Normal Range / Result

WBC (white) /1.7 / 4.0 - 11.0 / LOW
RBC (red) / 3.2 / 4.2 - 5.4 / LOW
HGB (Hemoglobin) / 10.4 / 12 - 16 / LOW
Platelet / 14.0 / 150 - 400 / VERY LOW

This was "day 12" as they refer to it (12 days after replant) and most counts get back to normal between 9 and 14 days. So it seems like Chris is running behind but in reality she is still within the normal timeframe. We just want to sprint to the finish (get to normal) when in the long run, one or two days won't matter. In the scope of many years, whether her counts get up today, tomorrow, or Friday will not really matter. Of course the first question I asked was "is it possible that the stem cells don't get to the bone marrow and start producing as they are supposed to?" The answer is no, just be patient, they will come.

Chris had no breakfast today at all, but she had a fair lunch and a good dinner. For the first time in weeks I actually felt that she ate a decent meal.

It is interesting to see the shifting attitude today in the center. You can kind of tell that they are preparing for the long weekend and are trying to position themselves to ensure the patients are fully prepared. Chris did get two bags of platelets via an IV today, and they may very well add more tomorrow, along with some red blood cells. They are effectively stocking the pantry, with the hope that nobody has to run to the store over the three day holiday. This is true of both the medical team and the patients. Certainly the doctors deserve a long weekend and it would be nice for Chris to not have to go to the hospital for at leat one day. She and I have been there every single day since June 4th in one capacity or another. She could really use a day where she sleeps in, lays around the apartment, takes naps on her schedule, reads the many books she has been trying to read, etc. It's too bad the DVD isn't working (oops).

I did have a discussion today with the team on the rationale as to the stem cell transplant as an out-patient vs in-patient. They were very blunt, stating "our mortality rate with our out-patients is significantly better than others doing inpatient". It made we wonder about the focus on the "mortality statistic" in the medical field. In theory, a hospital could ensure it has a great mortality rate (i.e. low) by only accepting extraordinarily low risk patients. Yet this would obviously cause a bias against medium risk patients who could very well benefit, albeit with the higher risk. It must be a very difficult decision sometimes whether to accept a candidate for the risky stem cell transplant/high-dose chemo program if the candidate has a higher than average risk level. These doctors have an incredibly difficult decision to make that I did not fully appreciate. Do they accept a higher risk patient into a high risk program that has a greater success rate (high dose vs oral) along with a higher morbidity rate, or do they do the low risk, lesser success rate route. Not sure that all made sense but the point is these doctors make decisions everyday that we (or at least I) do not appreciate the magnitude. One could almost say that they have to play God everyday. This reinforces my belief that medicine is an art, not a science. There is no script to follow when it comes to final decision making like this.

Again, I meander off the true subject here - Chris. My wanderings are sometimes theraputic, as they help me appreciate the enormity of this. Most important, Chris is taking small steps forward. Tonight I will give her a back massage as a way to coax those shy stem cells to get to work. Enough vacationing, we need to step it up. But again, in the long run this is not a sprint, but a journey to long and healthy life.

Progress!!

2008-07-01T21:23:00.003-04:00

Quick update. Chris got the go ahead to come back to the apartment and we made it back around 8 pm tonight. She still had a rough day with some nausea and no appetite at all. She tried to eat a dinner they served late this afternoon and she ate about half of it only to lose it 30 minutes later. Luckily this was after the discharge instructions had been given and we got out of there as quickly as possible before they knew what happened. The head of the medical team met with us today (as she does daily) but this morning she had 6 medical students in tow. As this is a teaching hospital, you see a slew of medical students all over the place. It actually is exciting as there is a ton of energy. These medical students just listen and learn from the experienced medical team and you can see that they are soaking it all up. That said, it must have been a bit disconcerting to Chris to have seven people examine her. I do not exaggerate when I see she had 4 stethoscopes on her back at the same time. It is very important that as many medical students as possible are exposed to this disease. It is so badly diagnosed with so many doctors missing it that we want as many medical students as possible to learn the symptoms.

I also met with the head of the nursing department today. As Chris has now spent a total of 10 nights in the hospital over the last 2 weeks, it was important to me that I tell the department head what I thought of the care. Within our family (and I hate to admit this), we have had 6 significant surgeries in the last 2 years. In all of those, I have never seen a nursing staff as good as this one. These people were so passionate about their patients, they had so much empathy for the misery Chris was going through, and they were very very patient with me (that alone is worth acknowledging). We had three nurses over the various shifts (they work 12 hours shifts over a 3 or 4 day period) and they are all good friends. They would call each other when they were home to ask their colleagues how Chris was doing. I wish everyone could experience the care these nurses provided -just incredible.
Of course I also took the opportunity to propose new processes that I thought could be reengineered within the hospital, but I'll leave that to another day.

Chris is very happy to be back in the apartment. I can't describe how strong she has been. Imagine days and days of nausea, vomiting, diarrhea, complete exhaustion, and not being home. Most of us would complain at some point, but Chris is so internally committed to beat this thing that she refuses to let it get her down. She is my hero.

So tonight we have no bells and buzzers, no vital signs every 4 hours, and no injections. Of course we are back there early tomorrow morning. But that is fine as she is making progress.

By the way, the haircut she got two weeks ago was not short enough. Hair is flying everywhere, so tomorrow she gets the buzz cut (another new talent I will learn).

Monday - Another tough day

2008-06-30T20:57:00.002-04:00

Unfortunately Chris had a tough day today and did not see the progress we had been hoping for. As such, they are going to keep her in the hospital yet another night tonight -Monday. She remains extremely tired and has some occasional nausea and still some digestive problems. Additionally, she has eaten very little today. So to call it what it is, it was not a good day. Her red blood count looks good and her platelets are good, but her white blood cells (WBC) are not back to an acceptable level. A normal count for WBC is between 4.0 and 11.00 K/UL. Today she was at .7 and thus she still has a long way to go. Night #4 is not what we expected, and while Chris is keeping up a good front, it is frustrating to us.
We have been told over and over again that the hospital is the worse place to be, yet here we are for another night. I questioned the doctor about the rationale for keeping her, and the answer was that this way they can monitor her more closely and avoid us from having to bring her back to the emergency room in the middle of the night. I am starting to believe that there is a lack of trust by the medical team in the emergency room and that by keeping her here, the Amyloid team maintains the decision making control of the patient vs entrusting it to the triage team in the emergency room. To me it looks like departmental politics vs patient prioritization.
Yet, that said, I have to contain my skepticism. Despite my natural inclination to suspect politics, I realize that in fact, this medical team knows more about this than anyone in the world. I am very comfortable asking questions and challenging, but in the end I realize that they know more than I do. (Those who know me well realize that is a very difficult admission for me to make).

So this has been a tough day for Chris. Again, not a single complaint and just an amazing patience for the rebound to happen.

I know we all hope for a much better day tomorrow and authorization to get home. She continues with her amazing internal strength.

Sunday Evening - Late

2008-06-29T23:13:00.002-04:00

This is night three in the hospital for Chris. They decided this afternoon to keep her as a precaution as both her white blood cells and her red blood cells were low. She has been very tired and is moving very slowly today, and dozing a fair amount. This afternoon they did a blood transfusion as a way to improve her hemoglobin (carrying her oxygen). Tonight, just before bed, she actually looked relatively good. She sat in the chair (my bed) for awhile and has been doing some of her yoga moves to stay flexible.

She doesn't know it yet, but she is losing her hair quickly. I am pretty sure it will be a mess tomorrow, so she may just decide to take it all off. I think it would be a great deal easier on her to not have to worry about the inevitable.

Chris' sister came in today and relieved me for a few hours. I actually re-entered the real world for about 3 hours. It was strange that people were not wearing lab coats, blue scrubs, and no stethoscope around their necks. People were dressed very strangely, with shorts, sandals, printed shirts and baseball hats. I had no idea that there is an entire world beyond the Boston Medical Center.

Hopefully we will get an early start on the blood tests tomorrow and will get our release from here. To be honest, today's No Go was very frustrating for both Chris and I, but we know in the end it is the right thing - better safe than sorry. Chris continues to be a rock, I am amazed that she can find so much strength.

Sunday Morning - Early

2008-06-29T07:08:00.004-04:00

Chris is resting comfortably after sleeping well through the night (she still is as of 7:30 am). She has been knocked out through a variety of drugs but I am glad she was able to sleep without much interruption. It appears that all systems are stable and I am hoping we are a GO for launch back to the apartment some time today (can you tell I watched a 2 hour documentary on NASA from 1-3 this morning ?).
Her blood pressure is now a bit low and again she has not eaten very much at all for the last two days, but if we have stopped the nausea she may start getting hungry. She has told me that everything tastes bad, or at least different, with a metallic flavor in most things. This is a frequent side effect of the chemo I understand. I would like her to get up out of bed this morning and walk around, as she has not been anywhere but her bed and the bathroom since Friday afternoon. It is very important that she stay limber and not get "deconditioned", where the heart and lungs start losing some of their strength due to lack of need.

Abby goes off to her college orientation today and Chris and I are both very disappointed that we can't join her. But she understands completely of course. One of my sisters will take her and they will spend the night tonight in NY. She took Melissa to Burlington as well earlier and has been a great help in offloading duties, along with Chris' sisters. Neither of us have been home for 2 weeks and it seems like an eternity. In the best case we'll be another 3 weeks as Chris rebuilds her hematological system.

I may quit this chair sleeping adventure I am on. I have learned that there are 72 ceiling tiles in this room, there are at least 11 different beeps, bells, or buzzers that seem to go off on average about every 4 minutes, and I can state unequivocally that the old woman in the room next to Chris knows more swear words than all of us combined. One of thr most incredible and long lasting tirades I have ever heard. Fortuntaly the Discovery Channel has great programming all night.

So bottom line, Chris appears to be doing better, but we'll know more when she wakes up. I really want her to be able to get back to the apartment where she is much more comfortable.
Thanks to all for keeping your fingers crossed and your hands clasped.

Saturday Afternoon

2008-06-28T14:04:00.002-04:00

I think Chris has summarized the day so far pretty well. To quote her directly, "this has been the suckiest day of my life". I think that just about nails it. I am not sure "suckiest" is much of a medical term, but it sure describes things clearly.

Chris has had things leaving violently from all ends of her body (again, my apologies for my lack of better elucidation). They have spent much of the day trying to balance nausea, diarrhea, blood pressure, potassium, fatigue, and anxiety. She did relatively well last night with only occasional bouts, but today has been rough on her. Hopefully the platelet's and some red blood cell increase will help her tonight.

She has been amazing through this. The Doctors all said this was coming, so she was mentally prepared for it, but I know she just wants to scream out "Enough already, no more pills, no more IV's, no more anything"!!! but she has an internal strength that is amazing. She knows this is the price to pay for her beating this and her fortitude is beyond what even I though she had.

The notes and prayers and positive thoughts from all of you have given her this strength. As one of her best friends said today in a note; Every second of every day God is hearing your name from people who love you. So listen while you're meditating...you'll hear millions of prayers, thoughts and wishes filled with love. What a fantastic thought.

More tonight or tomorrow morning.

Friday - a very tough day

2008-06-27T20:24:00.002-04:00

Well, the nice ride through the country side has turned into a dirt road in the thick woods with many potholes. We were warned it was coming and sure enough - WHACK!! Chris is back in the hospital as of Friday evening. She has had very serious digestive issues (ok, I'll call it what it is -diarrhea) with significant blood loss in the stool (sorry, I don't know how to say it nicer). As we understand it, this is an occasional side effect due to the toxicity of the chemo in the GI tract. She did great Thursday night and this morning, was eating well, was able to walk around the apartment, had no cough or swelling, but then as she started her tests this morning, things went down hill fast. She has been hooked up to IV's most of the day, and they added two bags of platelet's (which is what causes blood to clot). You can imagine that the combination of chemo impacting the GI tract, with no immunity system, and no ability to clot is a difficult combination to control. As such we are now going to spend the weekend in the hospital for monitoring. This is the third time Chris will be admitted in the last 3 weeks and she is getting very frustrated and a little depressed. Things were going relatively well all week, and they warned us a tough road was coming, and sure enough here we are.

Chris' white blood cells were still down to nothing, but hopefully they will start reappearing by Sunday or Monday. Today was "day 7" as they keep referring to it around here - seven days since her stem cell replant. Days 5-9 are the tough ones. The risk of infection is the biggest concern and can be a very serious issue if it occurs. We have heard stories about people spending 8 weeks in the hospital due to infections - so that is concern #1.

I'll sleep in a chair again (the back is a goner) as Chris likes someone here to make sure they clean and use the cather correctly. Because it is an unusual catheter that goes into the heart, many of the nurses are not familair with it and if not done properly it can cause a big problem. I am hoping I can get a hall pass for Sunday afternoon and grab a quick sandwich with a friend who will be in town.

So some big bumps in the road, but Chris is ok with the increased monitoring, and the staff here is the best. Hopefully tonight they can get the right balance of fluids in her body. And, for now she still has the full head of hair.

Overall she is hanging very tough, but this has been a miserable day to be honest. I'm sure tomorrow will be much better.

Still at Rock Bottom

2008-06-26T19:30:00.002-04:00

Another "unremarkable" day today. All three blood count levels are at or close to rock bottom as expected, and Chris is in the middle of one her highest risk periods right now. Just as yesterday, she has no immunity system at all and as such, we are being very careful. For about a week now, Chris has been in one of three places: the hospital, the apartment, or my car. She is unfortunately getting to "dislike" this routine (I am sure more explicit words will be used soon) as she is ready to go home. However, that is not going to happen.

the side effects remain surprisingly non-existent. Even the Doctors are surprised and keep warning us that they are coming. I had to ask today - "Did she really get the Chemo?". I was assured that they gave it to her, the high dose in fact. So we still wait. In the meantime she still has a full head of hair!

Over the last few days, I have come to two conclusions about the Medical world:
1) medicine is an art, not a science. Of course there is chemistry, biology, anatomy, etc. but I am struck by the amount of difference in opinion on courses of action, medications, and dosages from doctors in the same practice. There is an amazing amount of opinion, gut feeling, and unique perspectives. I have seen one doctor give a prescription and instructions on frequency only to see another doctor on the same medical team shake their head and say "no, don't do that, do this". That kind of shakes ones confidence that their is a single right answer that gets to the end objective. When two colleagues say two different, contradictory things, that is pretty scary.
2) the other observation I have had is that like any community, there is an amazing amount of politics. In my never ending research about this disease, I have found papers from researchers all over the world. I have seen lectures on the internet from researchers in the UK who claim that they "have the single best center for this in the world". They also have the best diagnosis capability, the lowest morbidity rates, etc. They explain in their lectures their findings and approaches. Yet when I bring these topics up to other doctors or ask questions about theses approaches, I see posturing, competitiveness, and even dismissal. So what is the point I am trying to make here: very simple - question everything you are told (why, how, who, etc), find out as much as you can about an illness , and for anything serious , always get a second of third opinion. Medicine to me as an art not a science.

OK, sorry for the off topic rambling, I just needed to vent. Most important is that Chris is in fact getting what I believe is the best possible treatment in the entire world for this disease, she is doing very well considering the incredible stress her body is undergoing, and the single thing we are most grateful for is the love being sent from family and friends. Tomorrow will be a critical day as we hope to avoid any infections with no immune system. That is the single biggest risk she faces.

Hit Bottom - as expected

2008-06-25T21:21:00.003-04:00

This medical team knows what they are doing. They told us that Chris would have no white blood cells today and sure enough, she has none. Her red blood cells and her platelet's are also down significantly but they have not needed to give her any as of yet. So right now, the replanted stem cells are quickly finding their way to the bone marrow (primarily in the pelvis and the "long bones") and will kick into gear this weekend. This is why they called last Friday her new birthday. She will have an entirely new system sometime next week. Of course without any immunity system at all right now, she is highly susceptible to infection. She is wearing a mask everywhere and touching nothing. On the way to the hospital, she keeps her hands in her pockets, and I open all doors for her (which I have been doing for years by the way). I have been wiping every surface with a disinfectant and I swear you could perform open heart surgery in this apartment it is so clean.

Chris has had none of the possible side effects. She is sleeping about 20 hours a day, but that is to be expected. It gives me more time to cook, clean, do laundry, pay the bills, etc. More importantly, she is too tired to watch Friends, so we are stuck on Season 4 (hooray)!!

We have received well wishes from around the world and it is greatly appreciated. Friends in France, Australia, Singapore, and even NY have sent their encouragement. Chris feels the love from everyone, and it has been a huge help when she is down.

One question I asked today was: When will we know if all of this had any impact? In other words, when will we know that the new plasma cells, created by the stem cells are not building these amyloid proteins? The answer I was given was 6-9 months. Only then will we know the three possible outcomes: complete response, partial response, or no response. The second question I have asked is: Will the body try to rid itself of the amyloid proteins deposited in the organs? the answer here is that it is possible, as long as there is not too much damage already. So yes, there can be an improvement and the organs can slowly repair themselves. This may take years, but we'll take that.

So, Chris has hit the ground floor, she is at great risk of infection, and we are being very careful. Thanks to all for the ongoing support and prayers.

No News is Good News

2008-06-24T19:54:00.003-04:00

As they say in the medical world, today was an "unremarkable day". That always seems like a very strange comment when it is said. To them, unremarkable means that everything is going as planned and there were no major incidents. To me, the entire process is incredibly remarkable, in fact it is completely amazing. So Chris' "unremarkable" day consisted of her daily trip to the hospital and her evaluation and blood work. The real indicator of activity is her white blood cell (WBC) count. The normal range for all of us is 4,000 - 11,000 WBC/Milliliter. Yesterday, her count was 9,000 (this was 6 days after the chemo). Today it was 1,200. They predict that tomorrow (Wednesday) it will be down to zero. She will then see the start of the new white blood cells (generated by the replanted stem cells) start to be produced by Sunday. Within two days, she will be back to an acceptable level (much quicker than I had understood).
She is extremely tired, sleeping often from exhaustion, but she is doing great. They ask her a laundry list of questions every day (does she have heartburn, dizziness, nausea, mouth sores, etc.) and she really has not had any in the last 2 days. Very unremarkable!!!

One thing I did today was to sit in the "Amyloid Support Group" that is held weekly at the Amyloid Center. This is really a one hour sharing session with anyone who is there as a patient or a caregiver. Chris and I sat in on this the first week of May when we were there for the initial evaluation. Back then, just about a month after we had heard of Amyloidosis for the first time, we were in a state of complete shock. We sat in this support group like "deer in the headlights", completely dazed at what was happening. There were four patients today who were being diagnosed with their three days of tests, and I knew exactly what they were feeling like. They have a blank stare, they have lost all color, and the are on the verge of a complete breakdown. Chris and I know this feeling painfully well. However, as I listened to these stories today, I was amazed to here the similarities. One woman said " I kept telling my Doctor I just did not feel right" as she was losing weight and energy. Another man said that he had been diagnosed with four different diseases, none of which were Amyloidosis and none that had any treatment. Yet, he got to Boston after doing his own research, they did the one required biopsy, and it was confirmed he has Amyloidosis. Finally a woman told her story, that she had felt bad for about 2 years and kept losing weight and getting weaker. She told herself that she was getting older and this was normal, but she had lost over 40 pounds. Her delay has caused her to get so ill that they cannot treat it with either the stem cell transplant or even oral chemo. Other stories I heard are a testament to how lucky Chris and I are. Many people are using their life savings to go through this. They have a single room in an inexpensive motel where the father( patient), the mother, and there two kids are living for the summer. They take a bus to the center everyday and the kids sit in the lobby all day long. Another man said his son left for Iraq last week for 14 months and he did not even tell him of his disease. The reason I explain these stories is that Chris and I both feel so incredibly blessed. We have gone from symptoms to treatment in about 6 months (others are years and too late), we have the financial resources to make this comfortable for Chris, we are very close to our family and friends, we have the most amazing support network, we have people praying for us daily, and I have enough experience in my job to know how to handle crisis situations. We certainly do not know what the end result of all of this will be, but one thing I am certain: we have everything going for us and we truly know how lucky we are. We know somebody is looking out for us.

Good Day Today - Monday

2008-06-23T18:49:00.002-04:00

Overall a great day compared to some others this past week. Chris has held down all three meals (albeit all small) today. Her weight has stabilized, and she generally feels pretty good, although very tired and weak. Very little nausea, no bone pain (which we know is coming) and she slept well. She is getting bored and told me today she is ready to go home, but that is not happening for many weeks even in the best of circumstances. However, two patients that we have gotten to know both went home today (to Ohio and Tennessee) after many months of living away from their families. We have it pretty easy as Chris' sisters have been able to come in and swap laundry, drop off mail, etc. while we have been at the hospital.

The daily routine is a trip to the hospital (about an 8 minute drive) for blood work and lots of tests. If things look good they send us home (apartment) for a fistful of pills that she takes. I am still amazed at how many different drugs she takes on a daily basis. She also has a device to exercise her lungs ans she is very careful about oral hygiene as mouth sores can be a huge problem.

One issue that still exists is the number of seasons of Friends remaining. I had no idea when I agreed to watch these with her that over 25 million episodes had been recorded (I may be off by a million or two but that's all). We are still on season 3 and I am very tempted to accidentally spill something on or in the DVD player.

So overall as of Monday evening, things are generally good. We know the storm is coming mid-week and things will get very tough. She has been a rock in this storm so far.

Sunday Morning

2008-06-22T11:01:00.003-04:00

Chris was released from the hospital Saturday evening and we got back to the apartment uneventfully. She did get sick a bit last night and again this morning, but she slept well. Amazing how much better you can sleep without bells and alarms and buzzers going off all night long. We went back to the hospital for her daily checkup and some injections this morning and all went well. However, they did warn her that unless she increased her fluid and food intake she would be "right back in the hospital again". Many of the drugs she is taking are making her very groggy, so she moves slowly and sleeps a great deal. She also has weird cravings and sends me out to the grocery store (basically at the foot of our building) for certain things. Today was Welch's Frozen Fruit Pop's, yesterday was Crytal Light. If I get the wrong brand name, it's back to the store again. She's Madison Avenue's best friend as she will accept only that name.

She has been trying to watch each of the season DVD's of "Friends". We are on season 3, and I think if I watch one more season I will be fighting her for the nausea medicine.

There has been no major hair loss yet, but we know it's coming this week. She has bought some nice scarves, so she is ready. I keep suggesting a Harley-Davidson scarf or a Confederate Flag - something that gives her a whole new persona. Those kinds of suggestions are not going over too well, so I guess I'll stop with those.

We've made friends with other amyloid patients who are at various stages of their own treatment. Some of the stories are very sad. Many of the patients are from around the world and have been away from their families for months. They all "just want to go home", but they know that is not an option. For now, we are doing OK with that. Abby and Chris' Mom and Sister came to visit for about 45 minutes this morning. They need to stay on the other side of the room and Chris wears a mask around, but it is nice to be able to see the family face to face - we are very fortunate that we have that.

So all is going well on this beautiful Sunday in Boston.

Saturday Noon

2008-06-21T11:52:00.003-04:00

Chris had a relatively good night last night and this morning (Saturday). She has been infused with her regiment of ant-infection, anti-nausea, anti-anxiety drugs, which has made her comfortable but sleepy. Generally she slept well, (but I however will be scheduling another back surgery from sleeping in this dang chair all night) and she actually ate half of a bagel and had some juice this morning. The amyloid team will be in this afternoon and may allow her to go back to the apartment tonight if she can keep her food down. She continues to lose weight as she is not eating, but we are all hopeful that will improve when her nausea stops.

Abby may come in to visit her today, but will need to wear a mask as we are now tightening down on exposure to infections. Melissa and Stephen are in Iowa as Stephen is competing in the National Junior Elite Triathlon Championships this weekend. They have been calling regularly to check in on her.

Overall, it has been a hell of a week. But now we need the melphalan to have done it's work and the replanted stem cells to wake up and start producing her new blood system. Throughout this arduous period of vomiting, nonstop nausea, aches, 100's of injections and pill taking, I have not heard her complain once. Literally, she has taken this with an amazingly stoic attitude and has not shed a single tear or even expressed a hint of frustration. She knew this was going to be a hard-fought battle and she has gone in with guns blazing. It would not be possible to state how proud I am of her. It would also not be possible to tell everyone how thankful we are for the incredible support we have been given from our extended support system. Chris' sisters have been fantastic, all of our families, friends, and colleagues have been incredibly helpful. This support has allowed me to focus on job #1.

Just as an aside, a quick comment as to how the world and God work in mysterious ways. June 20 was declared by a scientist in the UK ( http://www.ajc.com/health/content/health/stories/2008/06/20/happiest_day_0620.html)
as the Happiest Day of the Year. I couldn't agree more.

Happy Birthday Chris!!

2008-06-20T14:52:00.002-04:00

Just a quick update, with more later, but to let interested parties know, Chris was infused with 6.2 Million of her own stem cells today. Everything went successfully!!! At 1PM this afternoon, they drained the last of 4 bags of thawed stem cells. I think there was a tube or a line for each single stem cell as it was amazing how many different things she was connnected to. This was the last of three major steps in this process 1) the stem cell collection 2) the malphalan high dose chemo and 3) the stem cell replant. When you consider the fact that we are all born with a finite number of stem cells and that those stem cells have never been outside of her body before and they took a 1 week journey to the deep freeze, it is pretty amazing. I doubt any of us truly understand the power of stem cells. I'll avoid politics here, but I am confident saying that these particualr stem cells will give Chris her new lease on life. It's her new birthday today. Happy Birthday Chris.

Thursday Evening - Re-admitted to the Hospital

2008-06-19T19:30:00.002-04:00

Because tomorrow is a critical day and potentially one of the riskiest, the medical team has decided to admit Chris back into the hospital tonight. Her nausea has been stabilized with a list of medications that even the Doctors can't pronounce. She has been sleeping most of the day, but this is better then the non-stop vomiting. She still has no appetite at all, and to get her to eat a couple of spoons of soup requires incredible persuasion skills.

The stem cell collection will occur mid morning tomorrow (Friday). This is hopefully the start of her new life of wellness. I know a number of people who have told me already that this is their birthday (June 20) and it is a great day to have one. Her first birth date was December 27 and she always claims she gets "jipped' because she gets fewer or combined presents with Christmas. Now she will have two birthdays. I know for me, I can't wait to celebrate two birthdays with her for many many many years. I would even say that June 20 will become the most important day of our families life.

The process is incredible. Here is a link that describes the process in very simple terms:
http://www.medterms.com/script/main/art.asp?articlekey=4846

Although Chris does not have cancer, the approach is the same, but in her case the intent is to kill the defective plasma cells creating this protein.

So as of 8:00 pm Thursday night, Chris is resting comfortably, all vitals are stable, and the chemo is currently killing her blood cells. The stem cells she was born with will be replanted tomorrow and our prayer is that we have killed the bad plasma cells that are causing such damage to her heart. Thanks again to all for the countless offers of support and the incredible number of prayers.

Thurs Noon - Very Tough Night

2008-06-19T11:39:00.002-04:00

Chris had a miserable night, basically up the entire time with non-stop vomiting and nausea. We got in early to the center today and they put her on an IV for the vomiting. She is having a very difficult stretch, but she is being very strong as well. My guess is that she will sleep most of the afternoon. They also came in and did acupuncture for the nausea and she felt that this really worked.

She really has not eaten in 2 days, and her weight this morning was 108. The good news is that her lungs have really cleared up significantly, so this is a positive.

Chemo - Day 2

2008-06-18T19:16:00.002-04:00

Done!! Two rounds of an incredibly toxic drug designed to kill blood cells that in reality will hopefully stop this disease. Last night, while getting ready for bed, we both agreed that things had gone very well (yesterday). However, this morning, Chris did not feel well at all. She was very nauseous, a bit lightheaded, and vomited the little breakfast that she had nibbled on earlier. She is retaining fluids (3 lb's worth) and this has caused swelling in her legs and abdomen. The medical team debated this morning whether to only give her the smaller dose or continue with the stronger dose. In the end, they wanted her to make the decision. The stronger dose (200Mg) is much more toxic and the side effects much worse. But as always, Chris went the tough route. The success rate of remission is greater with the stronger dose, but she will suffer a more difficult period for a month or so. Overall Chris had a tough day today, and it is only going to get worse. I think the reality of how bad she is going to feel is going to hit sooner rather than later. Tonight, she is not feeling well, can't eat anything, and has little energy.

Tomorrow we start a much more intensive potpourri of drugs. She will be taking drugs for nausea, infection, mouth sores, etc. The regiment is incredibly scripted. This will continue for a month. Friday is the Big Day as they say around here. This is when they replant the stem cells and start her from the beginning. They call this her new birthday, and knowing all things are going to go well, it will be June 20. As I have stated earlier, she will have zero immunity system, but the newly replanted stem cells will start waking up from their deep freeze outside of her body and slowly begin producing the cells we all take for granted - the red blood cells, white blood cells, and plasma. The actual stem cell replant is extremely hard on the body, so it will take every ounce of Chris' strength. But we all know she will do this.

Chris' siblings have been a huge help. Her brother was great last week and her two sisters have just been incredibly helpful. They both have left their own families and are living at the house. Whether it is running errands, doing laundry, helping the kids, running things into Boston, it has been amazing.

Chris has been very strong so far, and she will need to be even stronger going forward. This is going to get worse over the next 5-7 days, so everyones prayers and support will be greatly appreciated. They certainly have made a difference so far, and we are both very thankful.

Chemo (Melphalan) - Day 1

2008-06-17T20:42:00.002-04:00

One down, one to go. Today Chris did an outstanding job of taking the high dose (200mg) of the chemo. We have been told that the two day dose is the equivalent of many months of normal chemo. She was a champ today. It is a very interesting process. As the dose is so strong/toxic, it can actually create lesions on blood capillaries. The most troublesome area is the mouth, so prior to receiving the chemo (through the catheter in her chest), she has to pack her mouth with crushed ice for 30 minutes. This constricts the blood vessles and reduces/prevents the lesions which might form. The actual chemo drip takes only about 20 minutes, very quick, with all the shaved ice she can stand during this period. It is amazing to think that this small amount of drug will effectively kill every cell in her blood system over a period of a few days, including the plasma cells that are creating the amyloid protein. It will also kill all of her immune system, which will be wiped out quickly. As such, she is required to wear a surgical mask over her nose and mouth at all times outside of the apartment or the clinic itself. She can't touch a door knob, an elevator button, or breath any open air. She also has a very restricted diet. No foods that are fresh (that might have exposure to an airborne disease). This means no fruits or vegetables, no restaraunt food, nothing but packaged meals.
They apparantly had a long debate about the dosage among the doctors and decided the best approach was the stronger amount. With the heart damage, it is critical that they stop any further amyloid deposits and they thought she was tough enough and young enough to tolerate the side effects which may be very severe. As Machiavelli said, the end justifies the means. If she can tolerate the side affects for a month in order to get a higher success rate, it is worth it.

Tonights dinner was a three cheese tortolini with an alfredo sauce and some slices of cheddar cheese. All prepackaged, and of course it was overcooked by the new chief chef. But, the direction from the doctors is to overcook everything, so that is a simple instruction someone who overcooks all the time! We are generally doing well in the new apartment. The views are spectacular (21st floor) and we are close to everything. The parking is underneath the building and a straigh shot down to the car from our floor. It couldn't be easier. The concierge service keeps reminding us that they can get us reservations or tickets upon request, but unfortunately we aren't going anyhere.

Chris' spirits have been very high today as we feel like we have made great progress with one of two chemo's done. Unfortunatly this is the easy part. We are told that next week she will hit bottom. Losing all her hair, vomiting, 22 hours of restless and groggy sleep per day, etc. The trick will be to avoid all infections and to immedualty address any hint of a fever or lesion.

All the kids have been calling to check up on the day. All three of them have been great, and Chris' sisters are both at the house keeping things under control there. As of Monday evening, things are going very well considering the seriousness of what we are facing. Everybody has been willing to help, everyone has been offering their prayers, and we are incredibly fortunate to have the combination of the best medical care and expertise in the world, the support of our family, friends and colleauges, and the financial capabilty to provide a very nice living environment as we fight this disease. Day two will be another step in counquring this!

It's a GO!!!

2008-06-16T23:23:00.001-04:00

Great news today!! They did a lung x-ray and things have improved significantly since Friday. They increased the amount of diuretics significantly over the weekend and this has done the trick. The diuretics (Lasik) reduce the edema (swelling) and you can see this in her legs and she can feel it in her breathing. The doctors were very pleased to see this and have given the go ahead for the chemo. Now the big debate is whether to use a very high dose (200 mg) of Melphalan) or a reduced scope (140mg). The higher dose has a higher success rate but it also has much more toxicity and worse side effects. The doctors are debating whether she can tolerate the higher dose, with the lung specialist stating that he believes the lower dose is the right option. So we are a GO. We had dinner at home tonight with the family and then moved to the apartment in Boston. This will be our germ free cave for the next 4 weeks. We have Lysol wipes, Purell, hand soap, etc. everywhere. Chris also starts a regimen of drugs (13 different prescriptions) which requires an advanced degree in pharmaceuticals to administer. That is Steve's job, as the caregiver, to manage all items outside of the Amyloid center. He will do all meal preparations (not a strength), all cleaning (not bad at it), all transportation, shopping, phone updates (pretty good), psychologist (better than most) and be the medical advocate (excellent).
Tomorrow will be a major milestone in beating this thing.

Long Weekend

2008-06-15T22:01:00.000-04:00

It has been a long weekend. Stephen had a triathlon on Cape Cod (6th out of over 700) on Saturday and both he and Abby have been working as lifeguards. Chris' sister and brother (Sally and Tim) left to go back to PA on Saturday after being up here all week, and here other sister Debbie arrived. She will be taking charge of the household while we are in Boston. We went to a graduation party Saturday night, but Chris is clearly slowing down, not eating, and feeling very anxious and losing hope. The discussion on the lungs on Friday has thrown us for a loop. Things were going so positively, but now we have great concerns that things have progresses so bad that the entire course of treatment may be put on hold.
We had a private prayer session with our Pastor after todays service. He was very supportive and calming and reconfirmed that we are not alone in this very scary journey. In fact, that is obvious every day. The support from friends around town has been beyond amazing. The meals, the errands, the phone calls, all just incredible. As a former candidate wrote, "it takes a village to raise a child" but in this case it is more like " it takes a village to help those in need" and we have been on the receiving end of that.
Chris has also been trying Reiki, and that seems to have been especially successful for her. This is a Japanese approach for stress reduction and channelling "life force energy". Steve is always the ultimate skeptic, but Chris is very clear that she is getting benefit from it so we will continue for sure.
Tomorrow is going to be a difficult day. We don't know whether it will be a Go, or put on hold. We are both ready to start the chemo as soon as possible, but if her body can't tolerate it, it can be very very bad. We'll leave ourselves in the hands of the Doctors and God.

Not a great day - getting worried

2008-06-13T20:30:00.000-04:00

We were back in the hospital for evaluations today (Friday) and we met with the pulmonary (lung) specialist as well as the program Director. They are becoming increasingly worried about the fluid in her lungs and have decided to hold off on the chemo planned for Monday. Fluid in the lungs is a concern as it is a potential indicator of congestive heart failure (the heart working too hard to pump blood around the body). They will do an x-ray of her lungs on Monday and decide one of three options: 1) start on Tuesday of next week, 2) delay a full week and try to address the lung issue, or 3) hold off indefinitely and consider other options. Of course we want to be as aggressive ans urgent as possible, but we also do not want to add risk if by waiting we can get a more optimal result. This is our first set back. We have been moving at incredible speed and have contacted the program, done the evaluations, been accepted, and done the stem cell collection faster than any other in the program, our aggressiveness and advocacy have paid off. However, now we have this issue ahead of us. We both hate going into the weekend with this hanging over our heads.

Even More

2008-06-12T20:12:00.000-04:00

Day two of collection today. Chris has developed some side effects such as nausea, digestive issues, and fluid in her lungs, but they are treating those. Because they collected so much yesterday, they only did 3 hours on the machine today (yesterday was six hours). They collected another 3 million plus today, so about 10 million total - more than enough. They are watching a concern around fluid in her lungs, but she is able to go home. Overall good news on the collection, many many cells, some of which will be replanted next week.

Very Successful!

2008-06-11T22:45:00.001-04:00

In order to proceed with the chemo, they need to collect at least 2.5 million stem cells. The amount collected depends on general health, blood flow, etc. They collected a whopping 6.2 million today! That is a very good result. The kids said she should get an award for best stem cell collection.
The procedure is similar to dialysis, but for this, they pump the blood from the catheter tubes into a machine, which then uses a centrifuge to separate out the red, white, plasma, and stem cells due to different densities. They place the stem cell layer in its own bag, and treat it like gold. We are all born with a certain number of stem cells, and will never get new ones, so they are very precious. They come up with a special box on wheels, wrap the bag in layers of bubble wrap and blankets, and then transport it to the blood bank. The woman who had to do this was told that if she were to see a bus coming at her, she is to save the stem cells first. It was incredible how carefully they treat this bag. In the blood bank they will freeze it and then thaw a portion of it for the replant after the chemo. So a great day for collection.

Admitted to Hospital for Stem Cell Collection

2008-06-10T23:40:00.000-04:00

Today Chris was admitted to the hospital for her strem cell collection on Wednesday and Thursday. As she has heart damage, they want to monitor her very closely during the process. In some cases, Stem Cells are collected outpatient, but better safe than sorry. She is a bit nervous but knows this is a required step. We went out for a good dinner in Boston, and then she got admitted and into the bed. So far so good, we'll see how tomorrow goes.

Catheter Placed Today

2008-06-05T19:41:00.000-04:00

Chris had a catheter placed in her chest today via surgery. This is really two tubes, 14 inches long that will serve as the input and output "ports" for her collections, injections, etc. The surgery went well. She now has about 2 inches of tubing hanging from her right chest that enter her heart. She was very tired after this, but we went home with no issues. So this was a major step and all went well.

Chris' Background - How we got here

2008-06-04T17:05:00.000-04:00

This post will be a long overview of how we got to where we are today with Chris. I won't go into excruciating detail, but will try to give a brief history for those who may not be familiar with it.

December, 2007
Chris was very active and busy with many things, including tennis, a personal trainer, yoga, skiing, etc. While she felt very healthy overall, she occasionally felt tired at her training session at the health club. She felt that instead of getting stronger, that in fact she was almost "losing ground" a bit. She chalked this up to normal old-age related slowing.
January, 2008
Chris had a normally scheduled annual physical exam with her primary physician, with normal blood work, etc. Everything in the exam appeared normal, but one indicator from a urine specimen showed an unusually high level of protein.

February, 2008
Chris continued with her activities, the tennis, the long walks with the dog, her training, etc, but started to state that "things just are not feeling right". She felt tired, but had no signs of anemia.

March, 2008
Chris had a follow-up appointment with her primary physician. They discussed the need for more testing and decided to do a 24 hour urine collection along with many other tests.

Chris also read an article in the local paper about a woman in a town nearby who had been finally diagnosed with a "mystery disease". As she read the article, she kept thinking "these are the same symptoms I am starting to see". Some shortness of breath, some sores around her eyes, some swelling in her legs, etc. Here is the article: http://www.metrowestdailynews.com/homepage/x1853598606

Chris showed the article to her physician as something to possibly explore.

April, 2008
On April 4th, Chris got a call (while playing tennis) from her physician asking her if she could come in to meet with her. Her tests had shown that she had a highly elevated protein level in her urine (known as an m-spike) that was an indicator for a number of very serious diseases, including the one that she had read about in the paper - Amyloidosis. Steve was in Oneonta, NY with our daughter Abby trying to make a final college decision for Hartwick College. Abby spent the night with the Hartwick swim team and Chris and Steve each had a separate and extremely frightful night apart. One of many future nights of no sleep.

We immediately went into action, scheduling appointments with Oncologists/Hemotologists/Nephrologists (kidney), etc. Chris continued to show highly elevated proteins in her urine and a number of biopsy's were performed. One was a bone marrow biopsy which came back as negative. At this point the Hemotoligist believed Chris has either Multiple Myloma or Waldenstroms Disease. We contacted the Dana Farber Cancer Center for a second opinion and continued on with biopsies. She continued to feel tired, had significant weight loss, had edema (swelling) in her legs, and showed a few other unusual symptoms. The next biopsy was a "fat pad" biopsy, taken from the abdomen. This is the required test for amyloid. The collected specimen is stained with a "red congo" stain and looked at under a special green light. This is the only valid test for positive confirmation for amyloidosis and is hardly ever performed as the disease is so rare (about 1500 -3500 diagnosis' a year). This test was required for us to meet with the Boston Medical Amyloid Center. Steve had contacted them earlier and they looked at the results and invited us in for three days of extensive testing the first week of May.

May, 2008
We went to the Boston Medical Center for three days of tests on May 5-7. On the first day, we were still unclear whether Chris has Multiple Myloma, Waldenstrom's, or Amyloidosis. On Mayt 7th, we were told, with absolute certainty that the diagnosis was Amyloidosis. This was confirmed with the congo red fat biopsy. They explained to us the genesis of the amyloid protein, the role of the plasma cells, the bone marrow, the details of light-chain proteins, the kappa and lamda ratio's, etc. They were very blunt, this was not Myloma and not Waldenstroms. Hopefully by now you have read the sidebar in this blog that describes the basics of amyloidosis. They explained the treatment options, the success rate ( odds of complete remission, partial remission, no remission) as well as the risk factors. Treatments include a very aggressive treatment called High-dose melphalan/stem cell transplant ( HDM/SCT ), as well as oral chemotherapy(similar to cancer treatment, but this is not cancer).
They asked for additional tests (stress test, pulmonary strength, etc.) and they informed us on May 19th that Chris had been accepted into the HDM/SCT program. Very simply, HDM/SCT consists of a three step process: 1) removal of her stem cells from her body (specifically her bone marrow) after a "port" or catheter is placed in her chest with tubes to her heart, 2) doses of a very powerful chemo drug called melphalan normally given over months, that will be given in 20 minute session over two days), and 3) replant of her previously harvested stem cells. The basics of the approach is that the melphalan effectively kills the defective plasma cells that are creating the amyloid proteins that are causing the organ damage. The chemo is so strong that had the stem cells not been collected, they also would be killed by the chemo (thus the removal and the replant). The process will completely destroy her immunity system. She will have no white blood cells to fight disease for about a month or two until the replanted stem cells start producing white blood cells again. As such, the top priority for this period is to avoid all exposure to infection.

So we now have a diagnosis, and a treatment plan. Read above for the next set of activities.