The other day was World Cancer Day. Cancer is a dreaded word, but a known one (unfortunately). I’ve had family members battle various forms of cancer. It runs rampant in my family. I know it. I know what it does. I know what the treatment options are. Dementia/Alzheimer, I know about those too. I know what those do, what their treatments (or lack thereof) are. But Fibrodysplasia Ossificans Progressive (FOP) is so rare that no one really knows what I’m talking about. However, as one person just mentioned to me… it is used in horror shows. Well, that’s comforting. The only knowledge of this is through horror or the label “stone-man”. But this is my daily life with my husband because just a few months before our wedding, this was the label placed upon him by the VA. What followed were constant phone calls from doctors wanting to study him, to run tests on him, to poke and prod. He on the other hand, said not just “no”, but “h— no!”. He didn’t want to be a case study.
We went through our wedding and the next two months in that newlywed bliss and denial of this disease. We’ve made our house a home for not just us, but the three kiddos as well. We are a family. And then reality hit this weekend. We could no longer be in denial, we had to face the cold-hard truth of this disease. And it’s ugly.
To give you a better understanding, here are some facts pulled from the glorious internet:
- Fribrodysplasia Ossificans Progressiva (FOP) is one of the rarest, most disabling genetic conditions known to medicine, FOP causes bone to form in muscles, tendons, ligaments and other connective tissues. Bridges of extra bone develop across joints, progressively restricting movement and forming a second skeleton that imprisons the body in bone. There are no other known examples in medicine of one normal organ system turning into another.”
- Any trauma to the muscles of an individual with fibrodysplasia ossificans progressiva, such as a fall or invasive medical procedures, may trigger episodes of muscle swelling and inflammation (myositis) followed by more rapid ossification in the injured area. Flare-ups may also be caused by viral illnesses such as influenza.
- Specifically, this disorder causes the body’s skeletal muscles and soft connective tissues to undergo a metamorphosis, essentially a transformation into bone, progressively locking joints in place and making movement difficult or impossible.
- In the affected areas, bone slowly replaces connective tissue; in addition to skeletal muscle, bony growths also occur in various tendons, ligaments, and bands of fibrous tissue that support muscles (fascia). The neck, back, chest, arms, and legs are usually the first areas affected. The disease may eventually affect the hips, ankles, wrists, elbows, shoulders, and/or jaw as well as the abdominal wall. In some affected individuals, the progression of bone development may be rapid; in others, the process may be gradual.
- FOP may eventually result in complete immobilization. Affected individuals may experience progressive pain and stiffness in affected areas, complete fusion of the spine, and/or pain in certain areas of the body caused by abnormal bony growths that compress the nerves in these areas (entrapment neuropathies). As mobility begins to deteriorate, affected individuals may exhibit an increased susceptibility to respiratory infection or right sided congestive heart failure.
- There are no known effective treatments for FOP. Certain types of drugs have been used to relieve pain and swelling associated with FOP during acute flare-ups (most notably corticosteroids) and non-steroidal anti-inflammatory medication between flare-ups.
- No treatment exists, but a clinical trial is currently underway
Comforting, no? So now do you see what we are up against? An extremely rare disease that is usually genetically present when you are first born. No, Daniel does not have the genetic signs of FOP. I do not believe he was born with this, and have my theories of how this has occurred. But I’ll save that for another day.
Here’s what we do know right now: it has already begun in his neck. The x-rays show it, the doctors have confirmed it. His neck is ossifying and it is painful. The new growth is pressing upon his nerves and I have to watch as he goes through full-body muscle spasms and see the pain in his eyes. I’ve had to endure the “I want you to be happy after all of this” talk from him. I’ve had to talk wills, life insurance, wheelchairs, and such. I’m not even three months into my marriage and have had to hear this.
We’ve had our glorious few months but with the sudden increase in the pain and nerve damage we have to face a new reality. He is ready to call the VA and start getting more answers. Daniel is looking ahead and trying to plan for the kids and for me. He’s hurting not just physically but emotionally as well. To have to face your own mortality, there are no words. And here I sit, trying to process it all, trying to stay strong. But I am not. I’m hurting. My faith is digging deep to try to find understanding. But I’m not ready. I’m barely holding on. Daniel says I’m strong. But I am weak. I can no longer be in denial as this has slapped me in the face.
And since neither of us really know what the future holds, what we are asking from you is prayer. Prayer for us as we navigate the unknown, the doctor appointments, planning for the future…for the kids. And please pray for our faith….that has taken the hardest hit of them all. The tough questions we’ve been asking of God, the trudging through to the deep recesses of what we believe and to find hope at the end of ourselves….be praying for us.
And if you would like more information, you can check out The International Fibrodysplasia Ossificans Progressiva website here.