[Chirley]

I'm in daycare this week. My copper level had dropped last time so my doctor increased the dose of copper sulfate from 4mgs/day to 5mgs. I asked if I could have this run in over an extra hour because I already get tummy pains and diarrhea from the 4 mgs. The nurse agreed to do this but then the pharmacist (new) came in and insisted the infusion was run faster. Needless to say I haven't had very good nights this week.

I saw my doctor this morning and I'm booked to have my liver biopsy next Tuesday. Apparently it's done with a skin local anesthetic under ultrasound in radiology. My liver enzymes are still slowly climbing despite the venesections and the Prof thinks that I have protein transport problem associated with the Hephaestrin and COMMD1 genes which causes an intestinal absorption problem and a renal tubule wasting syndrome. He thinks that the copper that I'm receiving is building up in my liver and causing damage.

The problem is that if the copper is accumulating in the liver it will cause liver failure and I'll die but if I don't get IV copper I'll get progressive neurological damage and bone marrow failure and I'll die. I've thought about what I want to do given the possible outcomes. This decision is really very easy, I've already decided what to do if the Prof is right about the copper accumulating in the liver. I've decided that I'll stop having the copper infusions and just have supportive care. I definitely don't want to die from liver failure.

Then again, there is so little known about my disorder that no one knows what's going to happen and I'll ignore this other stuff until the findings come back.

I was just interrupted by a phone call from radiology to tell me I have to be
admitted to hospital for the biopsy......has anyone else had to be admitted, does this sound routine? I HATE hospitals.

Regards

Chirley