Like Alan and Suzanne, who blogged on this subject, I was also very upset to hear about an injury suffered by our young friend. For anyone who didn’t read those blogs, there’s a boy who is age 13 and has Fibrodysplasia Ossificans Progressiva, just like Miranda and Erin. A couple of days ago, a classmate intentionally struck our friend’s knee with a drum mallet. The boy had much swelling as a result of the hit, and now his family fears an FOP flare-up which will lock his knee.
Hearing of this reminded me again that when you’re dealing with FOP, it’s vital to live in the moment. Of course, that’s good advice for anyone, because at any time you could be hit by a car, or suffer a heart attack, or whatever. But… in the case of FOP, life-changing events aren’t just a remote possibility. For people with FOP, life-changing events are inevitable reality. If you have FOP, you might have full use of a limb for years, and then suddenly, without warning, permanently lose mobility over the course of 2 or 3 days.
Those losses have happened to Miranda. Very shortly after she became symptomatic with her first FOP flare-up in her neck, at just over age 2, the flare-up spread like wildfire across her back. Her shoulder blades became partially fixed in place, causing her to be unable to raise her arms above mid-chest level. Still, that was manageable. What was really hard to take came just a few short months later, when I realized one day that she wasn’t straightening her left arm. I gently felt all over the arm, and discovered a hard lump in her upper bicep. I tried dosing her with Prednisone, as recommended, but it was no good. Within a few short days, Miranda’s left shoulder became fused almost completely in place, and she was never again able to straighten her arm beyond about a 110 degree angle.
The losses of mobility in Miranda’s left arm were terribly distressing. For a long time, the left arm was her “bad” arm, and Miranda did most things with her right arm. However, eventually we got used to it, and that was simply reality, until the day when the “bad” arm became the “good” arm. That happened about a year later, when I realized that Miranda suddenly wasn’t straightening her right arm either. I discovered a flare-up in the right bicep, in almost the same place the left one had been. Again we tried Prednisone, and again it didn’t work – maybe we were too late? Anyway, Miranda was never again able to fully stretch out that arm either, though it straightens a little further than the left. More upsetting, however, was the fact that unlike the left arm, Miranda also couldn’t bend the right arm beyond about 90 degrees. Her right elbow is now almost fully locked, with maybe a 10 degree range of movement. Now, Miranda feeds herself and does most things with the left arm, which can still bend normally upward…
With FOP, you have no opportunity to gradually ease into these losses. They simply happen, and the person with FOP has no choice but to accept it. You can’t do surgery to unlock the joint, because surgery is a form of tissue trauma and just results in more bone growth. If there’s anything “good” about any of this, in a twisted sort of way, it’s that you have no way to predict when these losses will happen. A joint might lock at age 3, or not til age 30 – FOP is unpredictable that way.
So… Where does that leave us? It leaves us needing to enjoy life as it is IN THE PRESENT. Maybe in the future, Miranda won’t be able to run, or chew, or clap her hands, but she can do those things right now. We want to take advantage of all that to the best extent possible, and let Miranda have lots of great experiences. We could sit and worry about things (and I won’t pretend we don’t still worry), but we’re determined not to let fear incapacitate us.