Every time Sam goes to California for the drug trial, they take a blood sample to measure how much cystine has accumulated in his white blood cells. That's how they track how well the drug is working. We are always stressed out about his levels because Sam's tummy doesn't empty very well, and the drug will get activated too soon if it comes in contact with dairy or fatty foods. This is a problem since the drug is extended release (every 12 hours instead of every 6), so if too much gets activated too soon, then he won't have enough left in his system to hold him over till the next dose.
His last cystine level came back at a 2.4, which is REALLY high. He is supposed to be below 1. Our doctor at Stanford thought it was most likely a lab error, and didn't recommend boosting Sam's dose. We took him to Primary Children's Hospital to get the same test immediately after we got the Stanford results. It takes about 4 weeks (at least) to get the results of the test back, so we've been pretty nervous for the last month.
Our nurse practitioner called us two days ago to let us know Sam's cystine levels were 0.55. That's low! We wish they were down that low all the time. So we're relieved to know that Sam's actual levels are probably somewhere in between, but it's still unnerving to know that they fluctuate that much.
The good news is Sam is a hyperactive, always-on-the-go little boy, and from the outside, he seems to be doing great. We hope his kidneys are doing just as well.
Wahoo! That's great. Sam's the man.
ReplyDeleteyou guys, that's so stressful!
ReplyDeletehe is a hyperactive little boy, but so fun. you guys are amazing, and so strong. i look up to you in so many ways.
go kidneys!