I thought I’d blog the latest on Noah, our four year old with more than his share of issues! For the recaps, I blogged about the buildup to Noah’s biopsy here and here, and then I blogged here about the results. Since then we’ve spent a bunch of time talking to a bunch of specialists, and Noah has managed to turn all his good behavior, and blood work torture sessions into milkshakes and Bakugan toys. Poor kid – he spends more time in the hospital and with doctors than the rest of the family combined.
He definitely has a form of localized scleroderma. We’re seeing a rheumatologist now that really seems to know his stuff; he’s helped other kids with the condition and he’s great with Noah. Normally, Noah clams up when he’s with a doctor – he does everything he’s told, but he won’t say a word and won’t answer questions. But with this doctor he was laughing, talking, answering, cooperating, playing – it was really good to see. Unfortunately, the news isn’t all good. It looks like it’s going to be a 6-8 year ordeal and we’ll have to use some pretty powerful medication to combat the spread of the scleroderma, and try to minimize the inflammation (the redness) and scarring. Because there is no cure, and it’s so rare a condition that even the treatments are all still kind of experimenting and there is so much unknown, the doctor told us the goal is that eight years from now there is very little, if any scarring and redness left. In addition, there is some atrophy going on – places where it’s like the scleroderma is eating underneath the skin and it’s thinning, or dipping in. It’s very minimal at the moment, but it can get worse and that is permanent, so part of the aggressive treatment is to prevent any more of that from happening. What stinks for Noah, though, is that the medication has a small chance of serious side affects, so he’ll have to get blood work every six weeks to make sure the medication isn’t impacting him negatively. We had to pin him down for the first round of blood work on Friday, and while he was screaming and crying I kept thinking, ‘every six weeks for the next eight years?’
Another encouraging thing for us, though, was that this doctor was the first to take all of our concerns about systemic scleroderma (the fatal form that killed my grandmother a few years ago) seriously – hence all the blood work. He seems as interested as we are in ruling out any forms of internal scleroderma, which we’re encouraged by. So at the end of the day, as depressing as some of the news was (I got a lump in my throat when I hear eight years), it’s the first time in over a year that Heather and I have left an appointment feeling like there was a plan, the doctor knew what he was doing, and knew what to do for Noah.
One other bummer of an update for Noah, though, is his eye! He also has ptosis, a muscle disorder that causes a droopy eyelid – it’s in the eye not surrounded by scleroderma. He actually had to have surgery on his droopy lid when he was a year old to pull the eyelid back up – it was blocking his vision and would have impaired his development. We’ve always known it was a surgery that will have to be repeated from time to time the rest of his life … and it looks like he’s due again. At his appointment with the eye doctor yesterday she said it’s drooping too low again, causing some damage to his eye development, so he’ll be having that surgery again in about three to four months.
The poor kid just can’t win.