“P” is for “progressiva”… sigh

Posted by Karen - February 19th, 2017

One of the many frustrating things about fibrodysplasia ossificans progressiva is that a person with FOP can go months, even years, with no change in their ability to function, and then SUDDENLY lose movement overnight. That’s what happened to my daughter Miranda recently. She realised about a month ago that her knee was hurting, and by the next day, she could bend it only a third of her former range. That movement is now gone for good.

You probably know that FOP is all about building bone where there wasn’t any before. However, it takes time for bone to grow, and it certainly doesn’t happen in less than 24 hours. What takes place actually is that the body initiates a “flare-up” in a certain region of the body, and in that area, soft tissue swelling and pain occurs. The swelling is what makes movement difficult, and becomes the site where, a number of days into the flare-up, cartilage starts to grow, and then is ultimately replaced by bone. This whole process takes place over a number of weeks – but nevertheless, the mobility in the affected area is often gone very, very quickly because of the initial swelling.

Sometimes flare-ups can spontaneously disappear without leaving damage, and other times they leave bone, but not much or in an area which is already limited by FOP – but neither of these is what happened to my daughter this time around. Instead, Miranda lost vital left knee movement, and with it, the ability to easily handle stairs and other situations where knee bending is involved. She can still climb stairs, but with two partially locked knees, she is more unstable and has to move about 3x more slowly than she did before. Additionally, she needs help getting out of most chairs after sitting.

This newly limited knee has had all sorts of consequences… For instance, we no longer walk the 400 metres from our front door to Miranda’s school, because there are too many stairs in between – and as such I drive her this extremely short distance. Also, at home, Miranda now tends to stay on the main floor until bedtime, and only go to her own bedroom (which is up a flight of stairs) when she plans to stay up there for the rest of the evening. When she sits in the living room, she first piles cushions on the chair to give her extra height and help her stand up.

I have to give Miranda credit; she has handled all of this with surprisingly good grace. She has rarely complained about it, and has maintained her usual happy mood. In fact, I’d have to say she’s adapted to this better than I have. I’ve spent a lot of time thinking about how dismaying all of this is (I appreciate that it’s a “watcha gonna do?” situation, but still).

On a related note, we had a meeting a few days ago with the BC Children’s Hospital Sunny Hill Centre to talk about wheelchair options. In light of this recent flare-up, we actually started talking about getting Miranda set up with a motorized wheelchair. Again, I’m very impressed at how maturely Miranda took in all of this; she was calm, rational, and forward thinking. Me? Well, let’s just say I left with my head spinning…

On another related note, we’re starting to ramp up with plans to buy a new home which will not require Miranda to use stairs. We’re getting all of the financial stuff sorted out, and will soon be able to begin seriously looking for a new place. We had plans to do this in 2017 regardless of Miranda’s recent flare-up, but this sudden mobility change has added a certain sense of urgency to the process.

As has been said by many of my friends recently in the FOP community – FOP SUCKS. Bigtime.

Miranda on our recent trip to San Francisco for Dr. Hsiao to examine her flare-up for the FOP Natural History Study. See that smile? Good grace, like I said.

Miranda on our recent trip to San Francisco for Dr. Hsiao to examine her flare-up for the FOP Natural History Study. See that smile? Good grace, like I said.


One Response to ““P” is for “progressiva”… sigh”

  1. RoJeanne Doege says:

    FOP mother to FOP mother, my heart is with yours, Karen. Everything you wrote is so descriptive of the ongoing unpredictability of FOP progression. Adapting to these changes is very difficult. Through the years, as Jasmin’s mom, I have tried, (sometimes successfully-sometimes not) to come to some sort of acceptance of that which I cannot change. I usually feel quite angry, powerless, frustrated, and sad. Jasmin, like your daughter Miranda, seems to handle these life altering changes with grace and a smile; she is also calm, rational and forward thinking.
    I just wanted to validate the feelings you expressed in your post and let you know I understand.

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