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.