Thursday, September 1, 2011
My Vanderbilt doctor has worked really hard at figuring out what is happening and how to treat me. My doctor is a professor of medicine who specializes in Myasthenia Gravis and ALS.
My MG is not responding to treatment, probably because I test negative for MG except for the Single Fiber EMG--which is the gold standard for diagnosing MG. It is the same way with the Small Fiber Neuropathy. I test negative on the skin biopsy, but clinically my small fiber nerves feel nothing at all. Lots of my testing has been evaluated by Mayo and Johns Hopkins.
These are all autoimmune diseases. They know my body is producing an antibody that has and is damaging my nervous system. They do not know what antibody is causing the problem and it would take years of research to figure it out. I am negative for all known antibodies. That figures!
The standard treatments for any autoimmune disease begins with prednisone and progresses from there. I have been on an organ-rejection drug for over a year to reduce my immune system. I have had all the "big gun" treatments: IVIG to overload my immune system with good antibodies and Plasma Exchange (22 treatments) to pull ALL antibodies from my body. Nothing has stopped the progression yet, but has slowed it somewhat.
The next step is IV Cytoxan chemotherapy. This is reserved for severe cases. The goal is to reduce my immune system even more and hopefully stop the progression. If the progression is stopped the damage to my nerves may improve over a long, long time.
We weighed the serious side-effects against the continued decline and opted for Cytoxan. If my MG is not stopped, I will eventually be on a portable ventilator to keep me breathing. My breathing muscles keep getting weaker. I start chemo as soon as insurance approves the treatments.
The absolute last step is called HiCy. It is high dose Cytoxan that will destroy my immune system (except for my own stem cells). In theory, it will reboot my immune system. But it would require my to spend a month in Hospital isolation and weeks of isolation near the hospital. This is similar to a bone marrow transplant. But that is the last option and not on the table at this point.
What I like about Vanderbilt and my Neurologist is that I can email him directly at any time. He has even called to talk with me on occasions. He listens and takes a lot of time with me! That is hard to find in a doctor now. I have read a lot about my condition in medical texts so I can ask the tough questions about my condition and treatments. He thinks outside the box as well, which i really like.
So far, I am the only one who has this combination of MG and Small Fiber Neuropathy to this extent. Even my insurance company has assigned me a case manager and the talk about my case once a month. I am a "lab rat." This is one reason I have been doing the blog and videos.