Posted by Karen
April 19, 2010 will mark the third anniversary of my daughter Miranda’s diagnosis with Fibrodysplasia Ossificans Progressiva.
A lot of FOP has happened in those three years. When Miranda was first diagnosed, we learned that many kids with FOP don’t have significant joint restrictions until their late teen years, or even twenties. I clung to that, telling myself it would surely be the case for Miranda, and she would have not much beyond flare-ups in her back and neck for several years.
Guess what? Nope, FOP had different plans in store. Miranda hadn’t had her diagnosis for even 3 months when a left arm flare-up caused her to lose almost all movement in the shoulder, and unable to straighten her arm at the elbow beyond 120 degrees. Then, less than a year later, she had a flare-up in her right arm which locked the right shoulder almost as much as the left, and which fused her right elbow in a permanently bent position. Throughout the time until the right arm fusion, she ALSO had numerous flare-ups on her back, neck and abdomen.
During this very difficult period, it felt like FOP was just kicking us in the teeth. Picture a Batman – “Joker-ish” character shouting, “THOUGHT YOU’D HAVE AN EASY TIME OF IT, EH?? HA HA, TAKE THAT!!!” But then, all of a sudden, FOP just sort of… eased off. For the last year and a half, it’s been just a little flare-up here, a little there, and none near any valuable joints. In fact, Miranda recently had a 7 month period with no flare-ups at all, and that period ended with a little one on her rib-cage which resolved quickly and just left a tiny nub of bone behind.
If our first 3 years with FOP have taught us anything, it’s that life with FOP is crazy and unpredictable. FOP progresses furiously for a while, and then stops and does nothing for a long time, and then goes in fits and starts, and then… Who knows?
Ironically, throughout Miranda’s experience with FOP flare-ups, she doesn’t seem to have been much bothered by it. If she’s got a flare-up, she only complains of pain if something directly presses on it. Otherwise, she generally seems fine (though a couple of times she had really BIG flares, and she did complain during those). In fact, she gets much, MUCH more freaked out by the idea of getting even a small, insignificant cut than by anything FOP dishes out (“Mommy, BLOOD!!!!!!! Get a band-aid!!!!).
Of course, the physical changes caused by FOP aren’t the only thing we’ve dealt with. FOP has also caused tremendous emotional and psychological anguish (that is, for my husband Pete and I – Miranda is blissfully oblivious), and necessitated a lot of coping with difficult and terrible things. The first 6 months after diagnosis, in particular, was an extremely challenging time. But, you know that old saying, “What doesn’t kill you only makes you stronger?” I feel like that’s really, really true, at least in our case. We didn’t fall apart. We were determined to carry on with life, and make it a good life. And you know what? We did. Now, 3 years after diagnosis, FOP is part of our lives, yes, but not an obsession. On our good days (and there are lots of those), FOP is just a mild inconvenience, like our older child Owen’s glasses.
In no special order, here’s a list of some things we’ve learned about our life with FOP (the good, the bad and the ugly):
1. Someone forgot to tell Miranda she’s disabled, or that she should be bothered by it. She shouts, argues, whines, runs around, belly laughs, does crafts, plays with Barbie dolls, goes to preschool, torments the cat and refuses to eat vegetables, just like any other 5 year old. For her, FOP is just “that silly old FOP”, the reason why her right arm is “stuck”.
2. We have tremendously generous friends and relatives. Truly, it takes my breath away to think of how our loved ones have donated their hearts out to help find a cure for FOP.
3. There are worse things than FOP. Much, much worse things.
4. One of the kindest, most compassionate, amazing and truly inspiring physicians in the world has devoted his life to curing FOP. I’m humbled by our Dr. Kaplan. The genetecist he works with, Dr. Shore, is equally incredible.
5. The FOP community is a tremendously supportive and wonderful group of people. I feel we’ve made dear and beloved friends among them.
6. If you’re crossing the Canadian/American border with kids but without husband, be documented up the ying yang – passports and birth certificates and a notarized letter from the kids’ father, because otherwise the border officials will give you significant grief (I learned this one on a trip to visit our friends the McCloskeys in Seattle).
7. Most people have never heard of FOP, and look slightly green when you explain to them what it does. I hate to say it, but there’s an extra “horror” element to this disorder that freaks people out more than hearing about other disabling disorders such as muscular dystrophy or cerebral palsy. The reaction people have is not pleasant, and even though people aren’t crass enough to say anything negative, I’ve learned to really dislike the moment I tell a new acquaintance about FOP.
8. To me, the scariest consequences of FOP are frozen jaws and hips locked so that the person is permanently standing. It gives me the absolute willies to think about these things. And yet, I know many people who experience one or both problems, and still go on and lead happy lives.
9. I enjoy blogging about FOP!
10. If your arms are fused, you can still learn to swim on your back.
So that’s it for the first 3 years. Here’s to many more, with a cure for FOP somewhere along the line…