FOP in 2017, and hopes for 2018

Posted by Karen - December 31st, 2017

Today is the last day of 2017. Phew – 2017, don’t let the door hit you on the way out.

OK, maybe I exaggerate a bit. In fact, in some ways this was a good year for fibrodysplasia ossificans progressiva (FOP). Looking on a broad scale, 2017 definitely had some positive developments. It’s just that speaking more personally, this year was abysmally bad for my daughter. She isn’t the only one either; a number of our friends with FOP were hit hard by the disease.

Starting with the bad… We weren’t far into January last year when Miranda told me, hesitatingly, that her good left knee was hurting a bit. A quick examination revealed that this was indeed, the beginning of a flare-up. Unfortunately, that flare proved to be the worst one Miranda’s had in years. Over the next 8 months, the FOP monster got its grips ferociously into my poor girl’s leg, and travelled up, down, and all around her knee, calf, ankle, thigh, and hip. No sooner would one part begin to feel better when a new region would become sore and swollen. By the time all of this finally drew to a close in early September, Miranda’s left knee and hip were locked, and she had new bony lumps in her hip and thigh muscles (and probably elsewhere too; those are just the ones we can detect). These new restrictions caused Miranda’s leg to be thrust forward so that she now can’t straighten up on her left side, and she has to walk bent over. She also lost a great deal of stamina for walking, and now can only go short distances. As if the leg stuff wasn’t enough, my daughter also suffered an unrelated flare-up in her left cheek, which caused her to have a partial jaw fusion, AND a flare in her left bicep further reduced her already limited movement in her left elbow. (NOTE – upon typing this, I’m struck by the fact that everything happened on the left side this year. That’s probably just coincidental, but it’s interesting.)

Miranda wanted me to mention our cat, Gally, in today's blog post. For that reason, here is Miranda with Gally.

Miranda wanted me to mention our cat, Gally, in today’s blog post. For that reason, here is Miranda with Gally.

FOP didn’t only cause new and substantial physical restrictions. This was also the year in which Miranda began experiencing (apparently) FOP related stomach problems, and completely unsurprisingly, anxiety about her situation. Finally, to top it all off, the end of the year saw the development of a small pressure sore on her left hip, which almost certainly was caused by the fact that Miranda can only get into bed and lay on her left side. Gah, enough already, we surrender.

And as bad as this year was for Miranda, it was ten times worse for our friend Erin Kate (of this website). Erin had struggled for at least a year with nausea and maintaining her body weight, and then suddenly in the spring, she developed breathing problems. Dear Erin ended up in hospital for the remainder of the year and had to be intubated and eventually undergo extremely risky surgery to insert a tracheostomy. She couldn’t speak for a while, and became reliant on a ventilator to breathe. On at least one occasion, Erin’s health became so precarious that she was at risk of mortality – thankfully, that episode passed. While in hospital, Erin also experienced FOP flare-ups and other complications, and had a feeding tube implanted and lost the ability to eat food with her mouth. Erin remains in hospital and is currently struggling with a serious cheek/neck/chest flare-up. Honestly, this year was just unreal for Erin.

The girls, August 2017.

The girls, August 2017.

Here are these two girls, both 12 years old and on the verge of becoming teenagers, and this is the stuff they have to deal with. Other friends with FOP had a hard year as well. One young woman in the USA has had a very challenging year with FOP, as did a little girl in the UK, a little boy in Australia, and several others. You see why we so desperately want an effective treatment or cure for FOP?

And on that note… 2017 was at least a good year in terms of getting us toward our goal. Two pharmaceutical companies progressed with their FOP drug candidates – one potential medication started phase 3 clinical testing and the other initiated phase 2. Additionally, a group in Japan started clinical testing of a re-purposed drug (i.e., one which is already approved and on the market for other health conditions). I understand there is also a handful of other drugs waiting in the wings to start testing within the next few years.

There were other positive things as well. I had the good fortune to travel 3 times this year to FOP related events – in February of last year I went to the annual “in person” board meeting of the International FOP Association in Florida, in June Miranda and I attended a family conference held in London, Ontario by the Canadian FOP Network, and finally, in early December I went on my own to the IFOPA’s family conference in San Francisco. All of these were excellent opportunities to become immersed in the world of FOP and learn the latest about research progress, not to mention being extremely valuable for meeting and talking at length with other FOP families (really, that was my favourite part).

Me with Debbie and Carrie,  board members of the Canadian FOP Network, with the IFOPA's Adam Sherman.

Me with Debbie and Carrie, board members of the Canadian FOP Network, with the IFOPA’s Adam Sherman.

To make a long story short – FOP was bad on the micro level for us and a number of families, but good on a macro level for research progress.

What do we want for 2018? One word says it all: CURE. That said, I’ll be happy to at least see a merciful calming of FOP for Miranda, Erin, and our friends in the community, along with ongoing positive progress toward treatments.

Happy new year, and welcome 2018!

2 Responses to “FOP in 2017, and hopes for 2018”

  1. Corinne says:

    I always enjoy reading your blog Karen. I am so very sorry to hear of all the troubles FOP has caused Miranda and your family this year. It is never an easy road with this dreadful disease. I am praying for 2018 to be a much better year for all those suffering. Sending my love.

  2. Connie says:

    Hello, I am a student giving a presentation on FOP at school. You mention there are a couple of drug companies with drugs in clinical trials. Since the disease is so rare, how do they go about choosing patients for the trial? Have you been asked to join a trial or tried to get into a trial?
    Thank you

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