Return Visit - Day 3

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

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

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

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

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

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

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

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

So there is a lot we don't know.

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