Friends With FOP

By Karen

Halloween is a time for all things strange and scary…  It was fitting then (in a twisted, black humour sort of a way) that this past week, leading to the end of October, we had the bizarre and disturbing experience of watching Miranda’s bicep swell up to twice it’s normal size.  And, believe it or not, the swelling DOESN’T seem to have been connected to Fibrodysplasia Ossificans Progressiva.

Let me now back up several steps in telling this story.  As everyone knows, unless you have been living under a rock, there has been much, much concern of late over the H1N1 flu.  The flu is of particular concern to people who have FOP, for a couple of reasons.  One reason is that people with FOP have variable degrees of restricted lung capacity, due to both congenital slight abnormalities in the rib joints, and to bone from flare-ups in chest mucles.  The second concern is that the flu virus itself sometimes causes FOP flare-ups in people with FOP – the experts don’t know why, but it may be something to do with the immune system, which plays a role in FOP.

Anyway, on Monday of this week (6 days before Halloween), Miranda had an appointment with her pediatrician.  Pete took her, and one of the subjects they discussed was flu shots.  Miranda had had a mild bout of flu a couple of weeks earlier (no FOP consequences though, thank goodness), and we think it was probably H1N1, but as she was never tested and it wasn’t confirmed, the doctor thought Miss M should have the newly available vaccine.  She falls into the “at risk” category, and so is approved at this point to receive the shot – right now only people with health concerns are getting the shot in Canada.  Since Miranda had no active flare-ups at the time of the appointment, Pete agreed Miranda could have the shot.  Actually, the doctor recommended both the H1N1 shot and seasonal flu shot, and he proceeded to give them to her – one in each arm.

Our doctor proceeded in the approved way.  That is to say, he administered the shots both subcutaneously, which is understood to be safe for people with FOP (intra-muscularly is out, as it can cause flare-ups).   In the 24 hours following the shots, the right arm reacted exactly as one would expect – Miranda had a little bit of mild swelling there, and a titch of redness, but nothing significant, and these symptoms were gone after a couple of day.  The left arm, however, was a different story…

When Miranda came home from the doctor’s office, I noted that her left arm was quite a bit more red and swollen than the other.  I iced it a few times during the rest of that day, but the next day it was worse.  By the day after that, it was even more swollen, and red like a third-degree sunburn.  However, though her arm was very swollen, she had no trouble moving it, so it didn’t seem to be an FOP flare-up.  Besides, they don’t generally get that red, and aren’t exquisitely tender to the slightest touch as this swelling was.

By this time I was getting pretty alarmed.  I don’t think flu shots typically react like this one did.  Also, I was pretty worried that the amount of swelling present could compress Miranda’s arm muscles and lead to an FOP flare-up.  So, day 2 after the shot, I contacted Miranda’s doctor.  He said those kinds of reactions to vaccines are rare, but occasionally they can occur.  Both he and Dr. Kaplan (FOP expert whom I contacted as well) agreed that I should give Miranda 4 days of Prednisone as a precautionary measure.  So, I did.

I’m relieved to say that Miranda’s arm was a bit less red and swollen by the next day, and got progressively better over the next 4 days.  As of today, 5 days after the shots, there’s only a slight amount of swelling left and the redness has pretty much disappeared.  So far, no appearance of any FOP flare-ups (in fairness that could still occur, but I’m optimistic it won’t).

I remain a believer in vaccination, but this was definitely scary…  Who would have expected such a reaction??  Very strange indeed.  I’m relieved that we seem to be through this bizarre episode.  Will discuss further with our pediatrician. 

On to more fun things…  Miranda, despite her poor little arm, was in a very good mood this week.  Every day it was, “How many more sleeps til Halloween?  I can’t wait!”  The merriment started on Friday, when I dressed Miranda up in her mermaid costume and took her to her preschool Halloween party.  She had a great time singing, collaborating on the jack-o-lantern, playing and showing off her costume.  Friday night, we went to a rockin’ good dance at Owen’s school, and both kids had a blast running around in the gym (though in Miranda’s case, with me dancing along close behind her to guard against falls).  Then, yesterday was the main event!  In the afternoon, Pete and I took our kids to a neighbour’s for a Halloween party, and they enjoyed cupcakes with mile-high icing and making Halloween necklaces.  Later, Pete took Miranda and Owen out trick-or-treating while I stayed at home to give out candy.  They were so lucky; the torrential rains of the previous day were no-where in sight.  When they came back home, bags bulging with candy, Miranda enjoyed standing outside with me to dole out goodies for latecomers.

Miranda after trick-or-treating, clutching her loot.

I’m so glad the end of this week was lots of fun for Miranda, because I’m sure that swollen arm business earlier on was NOT fun at all…