Friends With FOP

Posted by Karen

This was a really busy week for Miranda.  It started out last Sunday with a birthday bash for a friend, who invited both Miranda and Owen to her party at a local gymnastics club.  At first I thought this might not be such a good place for a 5 year old with Fibrodysplasia Ossificans Progressiva, but it worked out very well.

It turned out that the place we went to had wall-to-wall gym mats on the floor, and lots and lots of bigger cushions for throwing one’s self upon after jumping, tumbling, cartwheeling, etc.  This was, actually, a really good choice of party venue for Miranda, and I made a mental note to remember it as a possibility for her own birthday when she turns 6.

Here's Miranda bouncing on a looooooong trampoline at the gym.

Next, on Tuesday, Miranda had her second last dance class.  This was the program she started 5 weeks ago which is run by the BC Centre for Ability (agency which provides services to special needs and disabled kids).   As always, Miranda had a lot of fun “putting the icing on the cake” (foot stretched out to tap the floor), “making a window” (the traditional ballet plie) and twirling ribbons through the air.  The class ends this week, and we’ll be sorry for it to be over…

One of the moves the kids do in the dance class is called a monkey roll, where the kids lay down and roll a bit across the floor.  In the past, Miranda has always been willing to do this move, but this week, she said it “hurt her back” and she didn’t want to do it (which was fine; I had told the teacher never to push her to do something she didn’t want to).  I didn’t think much of it at the time, but that evening, as I was putting on Miranda’s PJs, I learned why rolling would hurt…  Unfortunately, Miss M had a new FOP flare-up, her first in 7 months.     It wasn’t on her back, as she had asserted, but rather on the left side of her rib cage. 

As always happens when I discover a new flare-up, my heart jumped into my throat.  I took a few deep breaths to calm down, and then had a closer look.  It wasn’t a very big swelling, but was unmistakeable and hadn’t been there that morning (at least not noticeably).  I gave Miranda a hug and kiss, which confused her, as she couldn’t figure out why I did this right in the middle of changing her clothes. 

Anyway, the next thing I did was get the bottle of Celecoxib (Celebrex) pills which we had prescribed by the pediatrician to try out at the next flare-up.  The instructions are to take one pill twice per day for the duration of the flare.  The idea with Celecoxib isn’t that it will eliminate a flare-up, but it’s a powerful painkiller, anti-inflammatory and anti-angiogenic drug – ie, it inhibits blood vessel formation, which is helpful because blood vessels grow in flare-ups and supply the cells which turn into bone.  The hope is that taking Celecoxib will help minimize the size of the flare-up and eventual bone formation, and maybe inhibit the flare-up from spreading (which those buggers are wont to do). 

Aside – use of Celecoxib for FOP hasn’t been tested with scientific double-blind studies, etc, but has been suggested as an option by FOP experts because of the properties of the drug.  I’ve talked to some adults with FOP who say their sense is that Celecoxib does have beneficial effects.  That’s enough for me…  For more information on Celecoxib (brand-name Celebrex), see the FOP Treatment Guidelines which can be downloaded at the IFOPA website (www.ifopa.org – my apologies if this link doesn’t work properly).

That night I tried Miranda with her first Celecoxib pill.  I wasn’t sure how well this would go, as Miranda’s never taken pills before.  To my great surprise, she swallowed it like an old pro!  No worries at all.  It was a smooth capsule rather than a powdery pill, but still…  It was much, much easier for her to do than I had thought it would be.

The next 2.5 days were spent with Miranda asking at least once an hour, “When are we going to the airport to pick up Auntie Susie and Ella?”    She was, as you can see, REALLY looking forward to my sister and niece visiting us.  That silly old FOP flare-up was completely forgotten (by M, anyway, if not by me).

The moment Miranda has been waiting for finally came on Friday, when M and I drove out to the airport to pick up Susie and Ella.  Ella, as it happens, is very close in age to M, just a half year younger, and the girls get along very well together.  After we picked up Sus and Ella, M and E were chatting away on the drive home just as though they had seen each other yesterday.

Since Friday, Miranda and Ella have spent almost every waking moment playing Barbies, Littlest Petshop and all the other things beloved of 4 and 5 year old girls.  They were happy to take a break from playing on Saturday, though, when we took a trip to the Vancouver Aquarium to see the “baby belugas” (Ella’s particular request).

Of course, the other main event was Easter.  We started the fun with egg painting on Saturday night.  Next, the two little girls (and Owen, too) rain downstairs yesterday morning and immediately dove into the fun of the Easter egg hunt.  Each kid found a whole bunch of plastic eggs containing mini-chocolate foil wrapped eggs, and each kid also got a basket with a large-size chocolate character and some smaller Easter eggs.  The hunt was so much fun that the kids wanted to do it again right way, so Susie and I re-hid the eggs and let them have at it.

The kids and their goodies.

Yesterday was also the day Miranda and Ella wore their matching “guitar dresses”.  They thought this was very cool.

The girls in their matching dresses.

Except for the flare-up, this has been a fun week.  We’ll be sad to drive Susie and Ella to the airport tomorrow for their flight back to Calgary.

(PS – I don’t know if this is the effects of the Celecoxib, but the flare-up hasn’t grown any bigger or spread yet, and Miranda hasn’t complained of any pain.  I’m hopeful this will turn out to be a good treatment regime for flare-ups, especially the ones on the torso for which the steroid Prednisone isn’t recommended.)