Posted by Karen - January 11th, 2015

Sometimes people tell me, “You’re so strong; I could never be as strong as you”, or similar words. This is, of course, in reference to dealing with my daughter Miranda’s fibrodysplasia ossificans progressiva. My response to this well-meaning (but ultimately frustrating) comment is to repeat something I once saw on the internet, which was that you don’t know how strong you can be until being strong is the only option.¬†

So am I strong? I don’t know if I am inherently, but I’ve definitely had to become that way due to good old FOP. It’s either be strong, or else thoroughly fail to cope. Those are the only choices.

But… No matter how strong I try to be, I can’t banish fear. It’s always there, sitting under the surface, waiting to pop out. Mostly I manage to keep it quiet, but I can’t always force it down. And in fact, I’ve got quite the grab bag of things to worry about, if I feel like worrying…

In no particular order, here are the fear-demons which stick their pointy little pitchforks into me:

– That my daughter will trip over something one day, and pitch forehead first into a concrete sidewalk, giving herself a concussion (or worse). This is both a less acute fear as Miranda has grown older and more conscious of her own safety, and a more acute fear as she wears her protective helmet on fewer occasions than she did as a young child.

– That despite all the FOP research going on right now, much of it very promising, it will all come to nought and there will be no effective treatment for FOP during Miranda’s lifetime. The result is that Miranda will become more and more progressively physically restricted.

Skeleton of Harry Eastlack, a man who had FOP.

Skeleton of Harry Eastlack, a man who had FOP. This is what FOP is doing to Miranda’s body.

– That the current FOP drug trial will fail because the researchers can’t get enough subjects over age 15 who are in a flare-up state and are physically able to travel to where the drug testing is going on.

– That there WILL be an effective treatment approved for FOP, but because there are so few people in the world ¬†with FOP, and the disease progresses at such different rates in different people, the cost of the medication will be astronomically unaffordable. On a related point, we won’t be saved from this particular nightmare by British Columbia’s provincial Pharmacare program or our family’s extended health medical plan, both of which will decide not to cover the drug (for whatever reason).

– That although I am currently in good health, something will change and I will die young, and thus be unavailable to take care of Miranda and support her.

– That though my husband is currently in good health, something will change and he will die young, thus leaving us without his help and support.

– That the skin in Miranda’s left armpit, which gets no air circulation because her left shoulder is 98% locked in place, will develop an infection which rages out of control and causes skin ulceration (despite our daily efforts to keep her armpits clean and dry).

– That Miranda will have further FOP flare-ups in her legs in the near future, leaving her unable to walk or climb stairs. That these particular flare-ups will happen before we can move out of our current 3 level townhouse, which has stairs from the front door to the main living area and then stairs again up to the bedrooms and full bathrooms (with no bathroom at all on the main floor).

– That because of insane real estate prices in the Vancouver area, where we live, we will have to pay an additional $450,000 to move to a single level bungalow or rancher home. This is even after selling our townhouse. This will occur *just* after we’ve paid off the mortgage on our townhouse, and this doesn’t include the costs of adapting the new home for a wheelchair user.

– That FOP will cause Miranda’s jaw to fuse, making it difficult for her to eat and possibly resulting in her having to get teeth pulled just to make access for food.

– That Miranda will become depressed about the FOP changes in her body, causing her to have to deal with depression along with FOP.

– That my son Owen, who does not have FOP, will feel stressed and worry about his sister as he grows older and learns more about her disease.

– That Miranda will have a hard time adapting to high school when she goes there at the beginning of grade 8 because there are hundreds of kids there who aren’t used to her, and so will stare and not want to be friends with her. (We are extremely lucky right now, because Miranda is in a school program which keeps her with exactly the same kids from grade 2 through 7 – all those kids know her, are used to her and like her. This is one important reason why we don’t want to move out of our crazy-expensive real estate area.)

– That because Miranda can’t get certain vaccinations which can only be administered intramuscularly, which is a no-no because it can cause FOP flare-ups, she will contract a debilitating virus by coming in contact with a sick, unvaccinated child. Especially pertussis – whooping cough – which has been making the rounds recently and which is all the more worrisome for people with FOP, who have restricted ribcages. Oh god, this one makes me shiver just thinking about it.

See what I mean? I have no shortage of things to stress about; some of them more realistic or probable than others, but all sitting there, waiting to burst forth like the contents of a packed up jack-in-the-box.


Miranda, who is worth every one of my fears and more.

Miranda, who is worth every one of my fears and more.











2 Responses to “Fear”

  1. Marilyn Hair says:

    Oh my gosh, Karen. You could reboot and take a different path than fear. I have been in your shoes for 30 years. I have been terrified of most of the fears you list. I have learned (1) not to waste my energy worrying about things that might never happen, like the armpit infection and dying young, (2) I discovered that some things I was terrified about, when they happened they were not as bad as I feared. Ask me about when Sarah’s jaw became locked. And when Sarah could no longer walk I was surprised by my relief, first that what I had feared had happened and I didn’t need to fear it anymore, and second that she was now seated and would no longer trip and fall (3) You are about the join the IFOPA Board of Directors which takes your involvement in the FOP community to the next level, makes you a leader in the effort to find treatments and support FOP families, and will let you know more about the research and clinical trials. That last bit will happen at the Board meeting. I will see you there!

  2. Blair says:

    Karen – not to get all Dr. Phil, but I think it’s important to talk about your fears. Articulating them helps to overcome them, or at least to deal with them. So good for you. In addition to being a negative force that holds us back, fear can also motivate us to address and possibly mitigate its causes. As humans we all have fears. I certainly do, including some of the ones you mention. But I do want you to know that I respect your ability to openly admit your fears and that we are here to help however we can.

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