Posted by Karen
There are many, many frustrating things about Fibrodysplasia Ossificans Progressiva. Given that our Miranda is only 6 years old, and that her FOP has been symptomatic for only 4 years, we have yet to even experience many of the “joys” (ha ha) of FOP. One thing we’ve had all too much experience with, however, is the FOP waiting game…
Let’s say a person with FOP has a trauma to the body. It doesn’t have to even be a severe trauma. As a result of said event, the person may, or may not, develop an FOP flare-up. A flare-up is characterized by swelling to the affected area, heat, pain and stiffness (all in varying degrees), and progresses its way over several days to new bone formation. But when there’s an impact to the body, a flare-up isn’t inevitable. Nor is it necessarily connected to the severity of the injury – there have been people with FOP in serious car accidents, and who’ve broken bones from falls, and yet not had a resulting flare-up, while others take a minor, seemingly insignificant scuff and then FOP rears its ugly head.
To make matters worse, an FOP flare-up doesn’t necessarily occur right after a trauma. In Miranda’s case, I’ve noted that if she suffers an impact of some sort, it’s usually 6 to 8 days before any swelling even appears (the exception to this is with impacts to the head, which cause swelling right away). And so, when Miranda has a trauma to her body, we get to play the FOP waiting game.
Take, for example, our recent experience. Just under 2 weeks ago, I took Miranda for her swimming lesson. I was getting her clothes off in the change room when she somehow slipped off the bench, sliding down onto the floor and scraping her back on the wooden seat in the process. It didn’t seem like TOO big of a deal though, and life goes on, so she went ahead and did her lesson. After the lesson, however, I noted that she had 3 distinct red patches on her back, with the skin even rubbed off a couple of scrapes. I judge an injury such as that as being worthy of dosing with Prednisone (the only medication known to sometimes prevent a flare-up), and so I gave Miranda the drug 4 days in a row as recommended, and then hunkered down to see what would happen. Every day I checked it out – anything? Anything? ANYTHING? When we finally made it to day 9 with no sign of swelling, I was greatly relieved. Miranda has never had a flare-up develop more than 9 days after an impact. Phew, bullet dodged. Maybe the Prednisone prevented it, or maybe it just wasn’t going to happen that time. Either way, it’s all good.
But naturally, that evening, she hurt herself again. As Miranda was walking upstairs to her bedroom, she bumped her right shoulder into the edge of the handrail. This, of course, caused her a new red mark and some scraped off skin. This one was really borderline in terms of whether or not to give Prednisone. Given that she had just finished a dose a few days earlier, and since it was Miranda’s “bad” arm, I decided to cross my fingers and not give it – Prednisone is powerful stuff, with side effects from using too much.
At present, we’re now on our second round of the waiting game in as many weeks. I’ve been watching the shoulder mark gradually fade away, but we haven’t yet passed through the danger zone. Sigh…
If you have FOP and ever had a flare-up from a really minor incident, or else NOT had a flare-up from something major, tell me about it… I’m interested to hear your stories.