This used to just be an ideal temperature for me – warm enough to play outside but not so warm that you end up sweating. But this week 65 degrees has a new meaning.
Sienna used to be able to lift her left arm out to the side about 90 degrees, but she can now lift it only about 25 degrees. 65 degrees of motion lost since the flare-up in her left shoulder began on February 11th.
I can think of few things worse than watching F.O.P. take over your child’s body. Knowing exactly where the flare-up is and what is happening, but being powerless to stop it. And there’s no playbook that tells us how long a flare up will last, where it will go next, or what degree of destruction it will leave behind. Moments of hope can quickly turn to despair as it turns out that it’s not gone, only shifted locations. Yesterday afternoon we declared the swelling resolved, finally! And then last night, just a few hours later, a new swelling emerged on the front side of her shoulder. Today we found yet another new swelling on the left side of her neck.
I’ve heard from adults with F.O.P. is that the hardest thing about having F.O.P. is losing independence as you get older rather than gaining it. And now I’m witnessing it first hand.
65 degrees is the difference between being able to change shirts alone.
65 degrees is the difference between being able to do your own hair.
65 degrees is the difference between reaching the cabinet in our kitchen.
65 degrees is the difference between being able to pour the milk.
65 degrees is the difference between being able to wear a shirt that fits rather than one two sizes too big.
65 degrees is the difference between being able to build a fort and then get down on the floor to crawl in.
65 degrees is the difference between being able to give your mom a full hug.
65 degrees lost is unacceptable.
Over the past few weeks we’ve cleared out shirts that Sienna can no longer comfortably get her arms into, and bought new ones in larger sizes. Last weekend we lowered the bars in her closet so that she can better reach her clothes. Through it all, Sienna has been resourceful and resilient. She is learning how to do more and reach more with her right arm to compensate for the new limitations in her left shoulder.
Sienna doesn’t like to discuss her flare-up. But she does like to discuss her upcoming 9th birthday, which is a welcome distraction and her excitement has reached near fever-pitch. I’m glad that she has that to focus on. But the birthday is not enough distraction for me.
We are turning over every stone to try to find something that can help her, as soon as possible, and are setting up an appointment with a new specialist next week.
And we still have so much hope that with a treatment, we can stop Sienna’s FOP progression where it is, and even consider surgical removal of FOP bone in the future. We are fundraising for the Penn Medicine Orphan Disease Center 501(c)(3) Million Dollar Bike Ride Fibrodysplasia Ossificans Progressiva fund to fund new FOP research grants – all donations up to $30k are matched, so your dollars would count double in the fight against FOP!