I’ve been thinking about a quote from an FOP mom I heard a while ago. It goes something like this:
There’s just so much we don’t know about FOP. Almost every question you can ask about life with FOP, the answer is, “I don’t know. I don’t know. I DON’T KNOW!!!”
For me, this quote really captures the uncertainty about so much with Fibrodysplasia Ossificans Progressiva. Consider these kinds of questions, for example…
- If you become symptomatic with FOP at a young age, does that mean you’ll have extensive disability earlier in life than someone who becomes symptomatic older? Maybe, but not for sure. I know someone who became symptomatic at age 18, and within just a couple of years needed to use a wheelchair. On the other hand, I know someone else who became symptomatic at age 1, and he’s still walking and living independently at almost age 40.
- If someone with FOP takes Prednisone in the recommended way at the beginning of a flare-up, will it stop the flare-up? Well, it might, but… There are plenty of stories of people using Prednisone and having a flare-up END completely, and plenty of other stories about it seeming to have no effect at all (Miranda has had both of these results).
- Having FOP means you’ll always get a swelling from a hard impact, right? Not necessarily. A number of people with FOP have been in car accidents and had no effect to their FOP. Ditto for breaking bones in falls or other accidents. And yet, often a swelling can result from just knocking into a table or a door.
…and so on, and so on. I’ve indicated the “long answers” to these questions, but the “short answer” for every single one boils down to I DON’T KNOW.
This makes life with FOP very hard to predict. Beyond a few general broad principles, it’ difficult to say with any kind of confidence how FOP will progress in any given person. Almost the only certainty is that it will progress – we just don’t know the timetable.
When Miranda was first diagnosed with FOP, I used to hope that maybe she’d be affected with it more slowly because some of the signs of her FOP aren’t as pronounced as in some people. For instance, her toe deformity isn’t as extreme as in many cases. Her great toes bend inward, true, but they’re a normal size – lots of folks with FOP have shrunken, clearly abnormal great toes. As well, Miranda doesn’t seem to have the sparse, thin eyebrows seen in many people with FOP. Miranda also didn’t have some of the pre-natal changes sometimes seen in FOP. For instance, many people with FOP are born with fused or partially fused vertebrae in the neck, and this pre-dates any flare-ups. In Miranda’s case, though, she had almost full neck movement in her infancy, and x-rays have confirmed that she had much less pre-natal vertebral fusion than is often seen. As a baby she could turn her head both ways, and crawled normally – most kids with FOP don’t learn to crawl, because they haven’t got the neck mobility for it. Here’s a picture of Miranda at about age 1…
Sometimes I just stare at this photo. It’s hard for me to believe Miranda could ever stretch her arms above her head like this, or move her neck so well… But I digress. The point is, I thought maybe Miranda’s FOP would progress slowly because of the features I described above. You know what? Wrong! Miranda at age 4.5 has almost fully fused shoulders, her right elbow can move very little and her left arm can’t fully straighten - much of this extreme shoulder stuff and elbow limitations doesn’t happen in many kids with FOP until much later. So, it appears maybe Miranda’s FOP has progressed more at a younger age… But, does that mean it will continue to progress quickly, and that she’ll need to use a wheelchair in a few years?
Nope. Not necessarily. In fact, the answer is: I don’t know. I don’t know. I DON’T KNOW!!! Nobody knows, yet. But… scientific research is starting to give us the answers, and I suspect that one day in the not too distant future, we will know.
