Is that a flare-up, or…?

Posted by Karen - August 29th, 2012

Posted by Karen

OK, I normally blog on Sunday mornings, but was travelling this past weekend.  Better late than never, hey?

Ahem…  So anyway, my theme today is, “What is that lump on Miranda’s body?”  It so happens that I found myself asking that THREE times this summer.  Yes, three.

You see, Fibrodysplasia Ossificans Progressiva is a tricky bugger that way.  FOP, as you may know (or maybe not?), causes random soft tissue swellings in which soft tissue is converted to bone, progressively fusing joints.  The most terrifying thing for a parent of a child with FOP is to find one of those tell-tale lumps.  But the thing is, just because you see a swollen bit of flesh, it doesn’t necessarily mean there’s a flare-up going on.

Early this summer, my daughter Miranda developed a big, red, warm and firm swelling on her leg.  This would normally be cause for major alarm, since Miranda’s never had a flare-up below the waist before, and we’d really like to preserve her ability to walk for some time to come.  But – in this particular instance, I concluded quickly that this bump wasn’t a flare-up.  This one was, in fact, just a bad reaction to a mosquito bite.  I could see the bite on it, and my gal said it itched like crazy.  So, no flare-up here.

But a couple of weeks later, I looked at Miranda’s left hand and saw that the area around the thumb was swollen.  And red.  And warm.  I asked Miranda if this one itched?  She said yes, but wasn’t making any moves to scratch it, and wasn’t actually complaining about it.  Also, I couldn’t see any obvious bug bite.   Growing more suspicious, and dismayed, I asked Miranda if she had hurt her hand recently.  She told me that yes, she had banged her thumb a couple of days prior.  Oy…  OK, this time I was pretty sure we were dealing with a flare-up (the first ever on either of her hands).  Feeling kind of sick at heart, I made the decision to dose M with Prednisone, which needs to be given for 4 days in hopes of “short-circuiting” the flare-up.

A day into the Pred, however, I asked Miranda if her hand hurt.  Nope.  Not at all.  So then I started to second guess myself, because that’s not typical of a flare-up (they tend to hurt, even with steroid medication).  Was this or wasn’t it?  Again M declared that it itched.  Still, I gave Pred for a second day.  But during that next day, I noticed that the swelling was pretty much all gone.  And, lo and behold, I did find a mosquito bite on the base of her thumb.  OK, so NOT a flare-up.  I kicked myself a few times.  Prednisone shouldn’t be just administered willy-nilly, and here I went and needlessly gave it for TWO days.

The next time I found a suspicious bump was about 10 days ago.  Miranda has a bony bump in a spot on her back where FOP has done damage before, and I noted that the skin over it had become a bit puffy.  This time I was SURE we were dealing with a flare-up – that spot on my poor girl’s back has taken flare-ups a number of times, almost like a bull’s eye.  You shouldn’t give Prednisone for a trunk flare-up, so I didn’t give it this time, but I did start her on Celebrex – which is a powerful anti-inflammatory and anti-angiogenic drug (ie, prevents blood vessel formation) which is thought to have possible beneficial effect on FOP.  With sadness, I watched Miranda’s slightly swollen back for a couple of days, expecting it to get worse and worse.

But…  Again, M said it didn’t hurt.  Which is wierd, but I figured maybe the Celebrex was killing the pain?  Anway, by about the third or fourth day, I noticed that not only was the swelling not getting bigger, it seemed to be not as noticeable.  And it never had gotten actually warm, either.  By the next day, I didn’t think we were dealing with a flare-up at all.  Which is good.  But geez!  Was there even any swelling there at all, or had I been imagining things?

Dealing with FOP is frustrating, and nerve-wracking.  In the end, none of my girl’s three lumps this summer turned out to be flare-ups – yay, but definitely stressful while they happened.

If you are an FOP parent, or someone with FOP, have you ever made this kind of mistake before?  Please tell me it’s not just me…

2 Responses to “Is that a flare-up, or…?”

  1. Annalena says:

    Yes, we had almost the same experience this summer.
    First time on the leg, just like Miranda and a few weeks ago on her shoulders, on both sides. We talked about giving her prednisone but decided finally not to since it looked like a mosquito bite, but that one was the scary one since it was on her shoulders.
    I work with kids so I have seen alot of kids before with those reactions from insectsbites but I really understand, it is so difficult to know when it comes to FOP. A couple of months ago she also got a reaction on her forehead and we emailed Dr Pignolo and Dr Kaplan a photo just to make sure it wasnt a flare-up.
    I beleive It was just a reaction from high doses of pollen in the air. We didnt give any prednisone but we where really thinking alot about it, so yes I know what you are taking about:)

  2. Wendy Henke says:

    Hi, Karen – Justin, too, at times, seems to have significant reactions to bug (mosquito, I assume) bites, creating alarmingly large welts. Some he scratches til they bleed, usually in his sleep, while others don’t seem to bother him.

    I’m glad Miranda has had a ‘swell’ summer in other happy respects, with no FOP flare-ups:-)

    Abundant Blessings,

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