Zip started showing outward symptoms of Fibrodysplasia Ossificans Progressiva, (FOP) on June 2nd, 2011. He came in that morning with his shoulders swollen up like the Incredible Hulk. The swelling moved throughout his torso for about two months, up and down his trunk, always leaving a little bit more mobility behind.
He was correctly diagnosed on July 28th, 2011, after 42 doctors and a five-day hospital stay. Thankfully, they did not perform any procedures that would worsen the condition.
For the next 18 months, Zip continued to have flare-ups, or bone-building sessions within his little body. Most of the mobility that was lost, was lost rather quickly in the two months before diagnosis. His back has taken the brunt of bone growth, and to this point, it hasn’t restricted his mobility any further. Just a very bumpy back.
His shoulders are “pinned” and his neck and back are fused. His left elbow has also been affected.
Dr. Kaplan explains it as living with a bear in your living room. When the bear is asleep, you can dance and play and he will continue to rest. When he is awake, you can’t tiptoe across the room without him coming after you. Our bear has been sleeping for about six months.
All bone built by the condition is healthy bone. There’s a positive! It would be possible for Zip to have surgery to remove the FOP bone, but the trauma to the muscle would cause new flare-ups and bone growth would explode in that area.
At this time, Zip takes three medicines daily to help his condition. Singulair (allergy drug, Gastrochrom (asthma drug) and Celebrex (anti-inflammatory drug). If he has a large fall or trauma to an unaffected joint, we would administer Prednisone to stop the inflammation. I am not a doctor, but this is my very layman’s terms understanding. The bone builds when the area is swollen. If they can reduce the inflammation, they can reduce the time the bone has to grow, hopefully causing less bone growth in his little body.
In 2006, the gene that causes FOP was isolated. This is probably the largest leap towards a cure we could ask for as an FOP community. Basic research on FOP is beginning to provide the scientific basis for the rational design of meaningful trials.
Palovarotene is the drug with the most promising outcome at this time. However, many different paths are being explored.
We are holding our 4th annual fundraiser called Zipper Q, which is a BBQ “Throwdown” for the whole family and benefits iFOPa and F.O.P. Disease Research. It will be held on October 3, 2015, at the Claremore Expo-Gates open 3pm. BBQ tasting 4-6.
To learn more about ZipperQ visit ZipperQ.com