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.