Fighting FOP

Vincent Whelan is a 22-year-old UC Irvine medical student who lives the life of any normal medical student. He goes to class, partakes in extracurricular activities and studies. However, Vincent goes through daily routines slightly different than most and goes through his day trying not to fall or bump into anything too hard. If he does, a flare-up will occur and he will have to worry about yet another part of his body turning into bone and limiting his range of motion. This is because Vincent is one of the estimated 2,500 people worldwide diagnosed with a rare condition known as Fibrodysplasia Ossifican Progressiva (FOP).
It is a disease that replaces muscle and connective tissue with bone whenever the body experiences any type of trauma: surgery, hard bumps, falling down, etc. There is no known cure for FOP and that is why Vincent hopes to eventually discover this cure after medical school. But that’s not until after the year 2012.
Vincent was only nine years old when he was first diagnosed with this condition. He was swimming in his grandfather’s pool when he felt a sharp pain in his left leg. A combination of this pain and fear paralyzed him. He thought he was going to drown because he couldn’t move. Eventually, Vincent got himself out of the water safely, but immediately afterward he began limping.
“We all thought it was just a pulled muscle or something,” Vincent said. “But after a couple of weeks, it didn’t go away.”
This was a major indication that something was definitely wrong. After the cramp in the swimming pool, Vincent and his parents spent half a year visiting doctors across the country, and he was diagnosed incorrectly with many different conditions. At one point Vincent was even told that he had cancer. The doctor proposed that a biopsy was in order, but luckily before it was performed, a different doctor, a rheumatologist (a doctor who specializes in extremity and back disorders), diagnosed Vincent correctly with FOP. Had the biopsy been performed, it would have further restricted his movement because instead of healing the muscle and connective tissue cut during the surgery, Vincent’s body would have turned those portions of his body into bone.
After the emotionally exhausting search to find out what was wrong with Vincent, it was now time to deal with the reality of his condition. At age nine, Vincent had to stop doing all the things he had grown accustomed to doing throughout his life.
“My parents sat me down and told me that I had to be really careful,” Vincent remembered, “and make sure that I didn’t fall down, nobody hit me and that I didn’t play sports or I would get new bones that would form in my body and lessen my ability to move.”
But Vincent was so young that he did not fully understand what was being told to him.
“It just meant that I couldn’t play sports anymore, so that’s when I started playing the piano,” Vincent said.
Vincent has progressively lost some body movement due to the growth of new bone formation throughout the course of his life: parts of his back around his shoulders and neck have ossified and his right hip is partially locked. However, his restrictions have been relatively stable since the age of 13 and no major flare-ups have occurred since.
An FOP flare-up is when the body begins to generate new bone and leads to tissue swelling, pain and discomfort. It is usually initiated by trauma (falling, major bumps, etc.) but can randomly happen at any time on any part of the body, particularly the neck, spine, shoulders, chest, ankles, elbows, wrists, hips, knees and jaw, but will never affect the hands or feet.
At age 22, Vincent knows his body and his condition so well that he begins taking his medications, Prednisone and Thalidomide, before he even has the usual flare-up symptoms: pain, redness, swelling and warm sensations in the area that is trying to turn itself into bone.
“I don’t really know how to explain it,” Vincent said. “I guess I’ve just gotten so used to what it feels like, as soon as I feel it coming I know I need to take my medication.”
The medication helps to eliminate the flare-ups or make them completely go away, but cannot prevent bone growth from occurring and also has side effects. Prednisone increases appetite, and can cause depression and swelling, while Thalidomide causes drowsiness, and if not taken with caution Vincent risks losing nerve sensations in his hands and feet.
Although Vincent’s condition has partially hampered his range of motion, he is able to go through his day as normally as anyone else. He has devices that help him put shoes and socks on, and claw-like reachers that aid him when he needs to pick something up from the floor or grab something over his head. Some things may take him longer to do, like getting dressed in the morning, but he gets them done without complaint, and goes through the rest of the day normally.
FOP effects one out of every two million people in the world today and Vincent knows that when he is done with school he wants to aid in the research for finding a cure. An FOP patient’s lifespan on average is 45 years; however, Vincent is optimistic that he will live to a ripe old age and is hopeful that he will find a cure for his disease within his lifetime.
Today, Vincent is in his first year as a medical student. He graduated from Santa Clara University in June of 2008 as a biology major and moved to Irvine shortly after to attend UCI medical school. His days are a constant cycle of class and study time while paying close attention to any signs that he is experiencing a flare-up.
Vincent is also part of MEDleys a group of UCI medical students who get together on Tuesday nights for singing rehearsal on campus. They practice the melodies that they will sing to children in hospitals and senior citizens in nursing homes.
When Vincent sings, he stands still, so one could never notice that he walks with a limp. He also holds the music in front of him so that no one could ever tell that his upper back is almost completely ossified and prevents him from raising his arms over his head. There is no need for him to bend over while singing, so no one could ever tell that this simple movement is a very slow process, requiring him to take his time, balance and gently guide his knee to the floor to pick up whatever has fallen.
When you scan the room filled with singing medical students, your eye does not rest on Vincent, inspecting his awkward movements; instead it passes right by him without hesitation. Here, he is no different than the other medical students who share a love for music.

Update: The Internation Fibrodysplasia Ossificans Progressiva Association’s Web site, located at, lists the confirmed cases at 700 across the world with 268 in the United States as of December 2009.