What is Fibrodysplasia Ossificans Progressiva (FOP)?
One of the rarest, most disabling genetic conditions known to medicine, it 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.
An example of the typical progression of FOP:
Spontaneous flare-ups of the disease arise in defined temporal and spatial patterns, resulting in ribbons and sheets of bone that fuse the joints of the axial and appendicular skeleton, entombing a patient in a skeleton of heterotopic bone.
“What would happen if some soft tissue cells in your body randomly got the message to transform into stiff bone cells? Patients born with a disease called fibrodysplasia ossificans progressiva (FOP) are locked into this fate, often becoming severely disabled before adulthood.
The disease first manifests itself at birth, when a baby appears normal but has bent big toes. By early childhood, however, some of the body’s connective tissues—including muscles, ligaments and tendons—have begun ossifying into skeletal bone, locking the joints and distorting posture and movement. Some bone formation appears to be spontaneous, while some can be brought on by trauma from surgery or even a mild impact.”
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