Mixed News - as predicted

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

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

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

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

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

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

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

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

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

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

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

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