Wednesday, April 30, 2014

ECD Update

I recently spent some time chatting with three new people who were recently diagnosed with ECD (Erdheim Chester Disease). They were speaking with me as if I was an expert, but the fact is it hasn't even been a year since I was first diagnosed.

It is amazing how fast one becomes an "expert" when one needs to be.


Since I was informed that I had this disease (that seems to be a cross between cancer and a autoimmune disorder) it has hampered the proper functioning of my pancreas, blocked my spleen, invaded my testes, caused bone hardening, and spread throughout my retro peritoneal cavity.

The medication I am taking to stop the growth of the ECD is called Kineret (it is injected daily) and it seems to be working. There is no hope for a cure at this point, a standoff is the best that can be hoped for. Unfortunately the side effect of this drug is that it has destroyed my immune system. This along with a whole bunch of random genetic immunological deficiencies I found out about while at the NIH does leave me a little concerned about my ability to stay infection free over the long run.

The doctors have also diagnosed me as having pancreatic enzyme deficiency and I have started to take medication that should help me eat a more varied diet. The idea is the enzymes will help my body process the food I am eating and help me maintain/gain weight. The problem is I don't think its working (my stomach hurts right now as I type this). Fact is the more normal food I eat the worse I seem to be feeling.

I have been experiencing a lot of muscle weakness and fatigue recently but haven't brought it up with the doctor yet. I just don't want to deal with them for a while. I would guess that this is related to my diagnosis at the NIH of having low testosterone, but since I am not a candidate for testosterone replacement therapy I'm not sure there is anything that can be done about it.

So I would say my current status is stable with problem areas that need to be addressed in the next 3-6 months. I need to see a immunologist, gastroenterologist, hematologist, urologist, and stop in at JHU for my 6 month exam. Don't feel like doing any of it at this point. I think that with some medical issues being proactive is a waste of time because none of these schlubs really want to get involved until things are bad.

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