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.