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.