Discovering FOP

Until a few weeks ago, I had never heard of FOP.

FOP stands for fibrodysplasia ossificans progressiva, which means "soft connective tissue that progressively turns to bone." Simply put, FOP causes tissues like muscles, tendons and ligaments to ossify, or turn into bone. Painful, fibrous nodules develop over the neck, back and shoulders of children within the first or second decade of life, and then gradually move into the lower body and the extremities. These lesions harden into ribbons, sheets, and plates of bone that can fuse vertebrae, weld shut joints, even grow in between the ribs and around the jawbones.

Flare-ups (where the body starts generating new bone) occur with varying frequency, and generally lead to tissue swelling and discomfort. They are usually painful; the person may not feel well, and may have a low grade fever. Sometimes stiffness in joints can occur overnight, from the pressure and swelling of the early stages of new bone formation in the muscles. Attempts to remove the bone surgically only cause more explosive growths. Bone growth can also be triggered by bumps and bruises, and even vaccinations.

These bone growths eventually immobilize the person, turning them into a living statue; those who have frozen in a standing position have to sleep leaning in a corner. In the very advanced stages, the person has to decide if they want to live out the rest of their days sitting or standing. The person eventually dies of complications caused by FOP, usually suffocation from the rib cage being immobilized, or starvation when their jaws freeze shut, even heart failure, the muscle exhausting itself trying to pump blood through the increased pressure.

It is a genetic disease, meaning that it is the result of a mutation in the gene sequencing in one part of the afflicted persons DNA. That is part of what makes FOP such a devastating disease: there is no medication that can fix the error in the gene. There is hope, however: scientists have recently discovered the mutated gene ACVR1 to be the cause of FOP. Researchers are trying to spread awareness of FOP, and families of FOP sufferers and their supporters have been helping to raise money for the study through fund raisers.

It is hoped that learning more about FOP (which makes extra bone) can help with diseases such as osteoporosis (in which the bones are breaking down).

If you wish to learn more: http://www.ifopa.org/index.html]http://www.ifopa.org/index.html

If you wish to donate to the IFOPA (International FOP Association) check out their options and ideas at: http://www.ifopa.org/donate.html]http://www.ifopa.org/donate.html

(c) Neko-chan