Six Years and Living Very Well

I've tried to keep a running history of Chris' Journey to Health since she was first diagnosed with Amyloidosis in the spring of 2008.  This post will hopefully provide a good update as to her current health and future outlook. 

Using the theme of the annual State of the Union address that the US President gives annually, I'll state here that the status of Chris' health is "strong and the future continues to look positive".  Her story is documented in all of the previous posts entered over the last six years, but of course it is difficult to truly capture all of the dynamics; the minor set backs, the continued tracking of all of the clinical trials, the emotions (both good and bad), "the wait" for the latest numbers from her regular tests at Boston Medical, the loss of good friends, etc.  So here is a brief glimpse of her life (from my vantage point) in 2014:

-we have moved to Bratislava, Slovakia (where on earth is that?) on an International Assignment for IBM.  Chris and I have been here since January and will spend a few years here.  After many previous attempts to get her to move to China on an assignment, she finally agreed to another tour of Europe (her third -Belgium as a teen, Paris with all the kids ten years ago).  Bratislava is just 30 miles form Vienna and Vienna is just 2 relatively easy flights into Boston.  Chris feels that should she need to get home quickly, she could.  Additionally, communication technology (e.g. MagicJack, SlingBox, FaceTime) has really shortened the perception of being far from home.  We can talk to anyone in the US as if we are next door, which really reduces the perception of being far away. 

-after one particularly relieving annual visit to Boston Medical, I remember Chris getting into the car and saying "OK, I am ready for a big change".  Well she got what she asked for as Slovakia is a big change for sure (despite the technology).  Yet, she and I are having a great time here.  We live in an amazing apartment right on the Danube, we can walk to great restaurants, and most weekends we get in the car and travel:  Vienna, Budapest, Prague, Slovenia, etc.  I post this not to brag about it (we know how fortunate/lucky we are), but to show how Chris has a zest for life.  She wants to see the world and enjoys her healthy status as much as she can.  Earlier this year we took a trip to China, Vietnam, Cambodia, Thailand, and Singapore.  Why? because she was healthy enough to do it.  We do not take a single day for granted and Chris truly believes in "seizing the day".  For me, I never want to look back someday and say "I wish we had done more when she healthy".  The famous phrase "hope for the best, plan for the worst" comes to mind often.  We are both realistic that Chris has not had a Complete Hematological Response from her stem cell transplant, so again, we "seize the day".

-Chris overall feels very well.  She is not limited in any daily activity.  She continues to walk daily (now around Bratislava) and goes to the gym regularly.  She has reduced heart function (due to increased heart wall thickness  and reduced ejection fraction), but she knows how to self-regulate if she feels fatigued or heart stressed.  What we have learned is that she continues to suffer from a reduced immunological system that really hits her hard when she gets exposed.  This spring she spent over a week in the hospital with Respiratory Syncytial Virus (RSV) which usually hits children under the age of 3.  This resulted in severe pneumonia which really knocked her for a loop.  Yet she has recovered well.  There is very little upside to  these events, but one that continues to be amazing is how quickly a support system forms (remember my previous posts about "it takes a village").  The minute Chris started getting sick, her volunteer army deployed into action.  Her best friends, her sisters from PA and NJ, my sister, our children, the community, etc. were all fantastic.  Chris happened to be home in Boston for tests at BMC and got sick after that appointment.  While I was back in Slovakia, our son Stephen took on the role as Chris' "personal aggressive medical advocate" and did an outstanding job.  His knowledge of amlyoidosis, his biology degree, his current job as a medical assistant, and more importantly his maturity in patient advocacy all aligned to provide the support she needed.  I flew back as soon as I could get a flight home but knowing the amazing support system and advocacy were in place made all the difference.  Chris and I thank everyone who helped so much.

- As stated, we do not take a single day for granted.  The reality of this disease hit us both very hard this spring and we carry a hole in our hearts for the loss of two great pioneers in the battle against Amyloidosis.  Judy Lessard passed away in March from Amyloidosis.  She was a fantastic fighter and advocate and Chris and I had the amazing opportunity to meet her at the annual Amyloidosis event sponsored by the Amyloidosis Foundation led by Mary O'Donnell.  We first got to know Judy when she reached out and asked our son Stephen if she could join him for part of his fund-raiser bike ride for Amyloidosis  Awareness from Florida to Boston.  Here was an Amyloidosis survivor riding her bike, basket in front, a very pleasant diminutive woman riding along with him on his 1200 mile ride.  Stephen in full cycling gear and Judy on her bike with the press in tow.  Judy was a great fighter.  The other great fighter we lost was Tim Hornbeck.  Tim and his amazing wife Connie founded Secure Harbor http://www.secureharbor.org/ to help those in need as they fought this disease.  We got to know Tim and Connie as Tim underwent Chemo and a Stem Cell Transplant at the same time as Chris.  We spent many a dinner later on comparing notes, hearing abuot the success of Secure Harbor, and the the details of their two boys.  Tim was one of the most pleasant, gentle, and truly genuine men I have met.  He suffered through many chemo regiments and treatment related illnesses, but never did I ever hear him complain and never did he stop advocating for those in need.  Tim leaves a huge legacy in the Amyloidosis community as well as the hole in our hearts.  I know Connie will continue to be one of the best advocates possible in this battle.  We will miss both Judy and Tim whom we had the great fortune to meet due to this disease.

- Clinical Trials and Research continue to be regular reading and investigation for me.  I have tried to keep current on as many of the ongoing trials as possible.  I strongly encourage anybody who want to understand the clinical trial activity for any disease to search on that disease on www.clinicaltrials.gov.  Whether amyloidosis or any disease a loved one is battling, you can arm yourself with knowledge as to the current state of the art in research and become aware of trials that are underway - many of which are open to patients.  For amyloidosis, the International Symposium on Amyloidosis was held in April in Indianapolis, Indiana.  This is a critical event where the medical community leaders worldwide who are working to beat this disease meet every two years to review their progress.  There is a great deal of research going on and I am personally amazed at the progress. I watch the trials on lenalidomide and bortezomib and pomalidomide carefully and truly feel there is significant progress in the last six years that I've been watching.  Of particular note is that our son Stephen has been published as a researcher on a trial to identify mis-folded amlyloidosis proteins in affected cells. 

So wrapping up, I'll summarize with the view of the world from this vantage point:

- Chris is doing very well, but we do not take a single day for granted.  It may be easy to forget that exactly six years ago Chris was in a fight for her life - literally. She was replanted with her own stem cells after a round of high dose chemo that came as close as possible to killing her without doing so.  We both remember often just laying bed, holding each other very very tight thinking about all of the documentation that showed average life expectancy, death rates from the high dose chemo, risks of stem cell transplants. etc. We live our lives to the fullest now because we have the opportunity to do so.  We know that her Amyloidosis will be back -painful to say but that is the hard truth.  In the meantime she prepares for the battle by exercising daily while enjoying her family and friends.  Our daughters Melissa and Abby have been fantastic at keeping her emotionally healthy. They have a zest for life that is infectious to Chris (this time a good infection) and she feeds off of their success and their regular (sometimes daily) calls.  Stephen has always been an advocate as well and remains connected to the amyloidosis research community. Of course her family and friends have been been incredibly helpful and unselfish.

- This battle against this lethal disease continues.  For amyloidosis readers who are new to Chris' story, I urge them to stay current on the trials and research.  I am very optimistic about medicine's ability to win this battle, but we all need to be armed with the knowledge.

- My final thought  for this post sums up what I truly believe is the single biggest lesson learned in this six year battle:  Be overly aggressive when it comes to medical care.  Don't let these diseases manage you, but instead fight as hard as you can to manage (and beat) it.  Find the best physicians in the world (not your local practice), know more about the disease then your primary physician, read every article you can find, know every trial under-way, question every single diagnosis (the first three of Chris' were wrong), and be aggressive, aggressive, aggressive. 

Thanks for reading and all the amazing support for Chris.  

Our prayers for Judy and Tim.

Chris doing very well after 4 years

We did our annual intensive evaluation at the BU Amyloidosis Research and Clinic this week and I can report things are going well.  As the doctors keep telling us, the most important factor is how Chris is feeling overall, from her own perspective.  When asked by all of the various doctors in each of the exams, she always responded with a strong "I'm feeling good!".  To me, she looks great, she continues to have lots of energy, she has a positive disposition, and things are going well in her life. 

Of course we watch her "numbers" maniacally, and I'll give the basics here as I do not yet have the final report.  The most important, her FLC (free light chain) was 45.  This continues to inch up ever so slowly in my mind, but they always say the increase in 'insignificant".  I look at the overall trend and I do see the random jumping of the number in a + or - 6 point range (39 -45) but that is off a baseline of 600+ back 4 years ago, so overall pretty stable.  Chris' other biomarkers also remained equivalent.  We watch the 24 hour proteinuria, the BNP, the left ventricle ejection fraction, and the overall heart wall thickness.  In general, all seem to be stable although jumping around each evaluation.  We will get the famous "call" on Friday, but I've become enough of an expert that I know exactly what they will say: "things look good, don't worry about the slight rise, you look and feel great, so lets do another blood and urine test in 3-4 months".  So bottom line is that Chris continues to have a "Very Good Partial Response".  My nature, as stated many times in previous blog posts is to keep going until we get a "very good complete response", but they keep telling us there is no need yet. So we live our lives as fully as we can, even thought we know the amyloidosis will come back at some point.

As a quick update as to Chris' ability to live a good life, 4 years after her stem cell transplant, she continues to walk 2-3 miles in the woods most days, goes to a personal trainer 3 days a week, and yoga two times per week.  She gardens, helps many of the senior citizens in her life, and travels.  This spring Chris and I went to Dubai and India for 2 weeks and she did great.  These were long days of trekking through cities like Mumbai and Muscat, but she never tired or felt any nausea or limitations. We even went skiing at an indoor ski slope inside the worlds largest mall in Dubai.  She watched our daughter Abby graduate from college in May, will help her move to an apartment in Philly this summer, and will get away most summer weekends to Maine or NH.  Finally, we have plans to go to Oktoberfest in Munich followed by a week in Tuscany this fall with our good friends and another trip back to London to see the Patriots play at Wembley.  I mention these trips only to let amyloidosis patients understand a few points:  1) it is possible to keep a very active life post diagnosis and post treatment and 2) our belief that we need to do the things we want to do when we can.  We never take Chris' health for granted and we never want to regret not taking advantage of her current strength to do the things we want to do.  Effectively we have a "carpe diem" approach and I would encourage that attitude amongst all amyloidosis patients.

The other important element of the week at BU is the "support group" meeting on Tuesday.  Here, all of the weeks visitors to the center get together and share their stories.  I am always fascinated by the stories: how were people diagnosed, what were their symptoms, where are they in the process, what is their disposition, etc.  This session included two first time visitors with the deer in the headlights look, a survivor of six years and another survivor of 15 years.  Very inspiring stories.  I think how scared Chris and I were at our first support group session and the fear we had after reading the stats of average life expectancy.  I know the first time visitors have that exact same fear, but I am hopeful that the stories they hear give them hope.  Additionally, I think everyone learns the critical importance of a full time caregiver.  Those of us who were caregivers shared some of our stories about lifting our patients out of bed, getting them dressed, fed, cleaning up all kinds of messes, etc.  Nobody can fight this alone and  the caregiver/advocate role is the unheralded, often frustrating, and always rewarding responsibility of someone who has to swallow their ego and their own fears and be the rock of support for the patent.

I continue to watch every clinical trial and every published paper as I have set up Google alerts on key words which I get daily.  The trials continue to make progress and the drug combinations or "cocktails" continue to seem to be promising.  Velcade (Bortezomib) is now often given orally as a treatment option or in low doses prior to stem cell transplant.  Additionally, the combination of velcade with cyclophosphamide and dexamethasone seems to be promising.

So overall, things continue to go well for Chris.  However, to be sure, we never take a single day for granted.   I know many of the stories such as Paul in NY and the doctor in CT, and those with less successful responses and we both think of all of them often.  We have also learned of acquaintances who have recently been diagnosed and will do all we can to reach out and help.

I'll post another update after we get the formal evaluation results.  As always a huge THANKS for all those who have helped and shown interest in Chris for the last four years.  It means everything to us.

Chris at almost three years

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

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

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

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

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

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

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

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

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

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...

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

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

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

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....

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...

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...

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

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

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

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.