Have you ever thought that domestic falls could lead to a deadly condition often misdiagnosed as cancer? Fibrodysplasia ossificans progressiva is a disorder in which muscle and connective tissues such as tendons and ligaments are gradually replaced by bone (ossified).
The condition leads to bones forming outside the skeleton and constraining movement. The effects of the disease, which causes damaged soft tissue to regrow as bone is that sufferers are slowly imprisoned by their own skeletons. This disease is sometimes referred to as Stone Man Syndrome.
According to an anatomic pathologist and histopathologist at the University of Lagos Teaching Hospital, Dr. Kikelomo Adeleke, “it is a mutation of the body’s repair mechanism, which causes fibrous tissue (including muscle, tendon and ligament) to be ossified spontaneously or when damaged.”
“Some Nigerian doctors have done some research, but I doubt if the cases were confirmed because the tools to do genetic confirmation are not available in Nigeria,” she said.
SYMPTOMS
Medical practitioners have, however, found that the diagnosis of the disease is often delayed due to its rarity. “The clinical manifestations may also be confused with the various forms of childhood rheumatic disease among others,” doctors at the University of Capetown Medical School said in a report.
The main features are often present at birth with shortening and deviation of the big toes. In mid-childhood, tender subcutaneous lumps may appear, frequently on the upper region of the back, that becomes ossified, and widespread ectopic ossification resulting in immobility. Finally, movements are limited to the external muscle of the eye and the diaphragm. The median lifespan of a sufferer is approximately 40 years,
Most patients are wheelchairbound by the end of the second decade of life. The disease is incurable, and eventually results in death when the cartilage holding the ribs together also solidifies, making it impossible to breathe. Patients eventually suffocate or suffer a cardiac arrest as their bodies attempt to get enough oxygen.
DIAGNOSIS
The diagnosis of FOP is made by clinical evaluation, experts say, adding that plain
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