I have found a way to get Internet access in my room. I feel like I can step outside now, at least into the virtual world. I am feeling well. The medication, dopamine, has really improved my heart function. The loss of of that extra fluid has greatly improved my lungs and my abdominal organs. I actually feel quite normal. Many of the symptoms that concerned me as possibly secondary to amyloidosis were actually due to fluid retention.
Amyloidosis is a strange disease. It begins in plasma cells. They originate in the bone marrow and circulate through the body. They sense foreign proteins, (such as viruses and bacteria) and produce specific antibodies to fight off infection. Amyloidosis begins when a renegade plasma cell begins producing a faulty antibody protein that does not break down. I guess you could call these cells 'crazy' as it is referred to as a plasma-cell dyscrasia. It is not a cancer because the abnormality is not in the 'immortality" of the cell but rather the cell product. These proteins don't form or fold correctly and pile up in the body. They then get stuck in tissues in various locations; in my case the heart muscle.
This makes my heart muscle stiff and the heart then cannot relax well in between beats. The end result is that there is little time to fill up the heart ventricles so less blood is pumped per beat. What I feel is that any attempt to do any activity requiring greater blood flow is met with an immediate sensation of no energy. It feels like the battery died. Running, walking up stairs, bending over to pick something up all result in immediate fatigue and dizziness. The technical term for this is restrictive cardiomyopathy. My heart pumps about half of the volume that it should in one minute.
Amyloid can go all over the body and effect the gut, liver, kidneys, salivary glands, lungs etc... I had an extensive work-up at the Mayo Clinic and it appears that while other organs are involved, the most significant is my heart. Normally amyloid patients are not treated with heart transplants because they are either too sick with multiple organ involvement or there is a concern that the new heart will fail if the amyloid continues unchecked. The Mayo Clinic is one of the few places willing to combine heart transplant with amyloidosis treatment such as a bone marrow transplant.
However, because I am relatively healthy, and my other organs are not significantly involved, Stanford has agreed to accept me to their transplant list. I will be only the 2nd transplant at Stanford with amyloidodsis and the 1st with AL Amyloidosis.
Once I have a new heart, then I can receive treatment for the amyloidosis. Since this is a rare presentation of a rare disease there is no absolute right protocol for me. As it stands now, I will either get chemotherapy with Melphalan, Steroids and Revlamid or a stem cell tranplant. They will follow a relatively new blood marker (Kappa light chain levels) to ascertain my clinical response.
I thought this brief summary of my condition might be helpful to some of those following my progress.
Thanks for the explanation. Amyloidosis is one of those things you learn about in medical school that you never quite understand. I am glad that you are feeling better. Eschelle and I wanted to come visit you in the next couple weeks, if you are accepting visitors and feel up to it. Hang in there. . . you and Barbie are doing an amazing job at dealing with all this.
glad to see you virturally step out
nice summmary of the disease
I have a question about amyloidosis. How does the rate of antibody production by the "renegade" plasma cells compare to the rate of antibody production of normal plasma cells?
Wow, you're making history. Pretty and yet pretty scary to be the 1st. I'm sure you'll be fine, you're doing great so far! We love you!
kevin:thanks for the update & the explanation about your condition.everyone is behind you & wishing You the best.
kevin: thanks for the clear explanation of your condition.it is the best description i ever had.
you are in great hands & we are all behind you .continue to keep us up to date.
So happy to hear about your process and that you are doing much better. My husband has Familial Amyloidosis and we are leaving for Vanderbelt next week for a consultation regarding liver transplants as you know that is where the gene is since it is heredity. Then to Mayo for whatevery else tests they think he needs. Initally misdiagnosed for 10 months but also was 2 years before we even found someone we thought knew about his neuropothy.
We were since rediagnosed at Cleveland Clinic. We are from Ohio but now live in Tennessee. We do not know the best thing to do or where. I know Mayo is good but it is very far from us so we do not know if he has to have a transplant of liver and maybe even heart where to get it if it need to be close. No matter what it looks like we may have to leave our home. Maybe Mayo can help with this question.
I am so happy we founed your blog and that your transplant has worked for you. Keep the faith and God bless you. Joan
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