Hello, fellow histori-medico-philes, Kevin here for another installment of CEPI Curiosities. This time around, we have another by Paul, our diligent Karabots Junior Fellows intern. You may recall in our last issue, Paul wrote about the famous “Siamese Twins,” Chang and Eng Bunker. Let’s see what he has in store for us this time.
Take it away Paul!
Fibrodysplasia Ossificans Progressiva (FOP) is a disorder where muscle tissue and connective tissue such as tendons and ligaments are replaced by bone (ossified), forming bone outside the skeleton (extra-skeleton) that constrains movement. The process of FOP is usually noticed in early childhood, with unusually big toes and and short thumbs. FOP usually starts from the neck on down and is a slow and painful disease.
The extra skeleton formed by FOP causes a loss of mobility in the limbs as they become affected. FOP may cause problems eating and speaking due to the extra skeleton immobilizing the jaw bone. Over time, people with this disorder may suffer from malnutrition (lack of proper nutrition) due to the eating problem. This disorder also causes difficulty breathing with another rib cage forming around the rib cage, compacting the heart and lungs. It is very common for someone with FOP to become paralyzed.
Approximately 1 in 2 million people is diagnosed with FOP. Only 800 cases have been confirmed around the globe and 285 in the United States. FOP is often mistaken for cancer. Unlike most diseases, surgery only makes the condition worse.
One notable person with FOP was Harry Eastlack who was born in November 1933 in Philadelphia, PA. At age 5, he broke his left leg while playing with his sister. There were complications with the fracture, which did not set properly. A couple years later, his hip and knee stiffened and bone growths began to develop on the muscles of his thigh. The condition spread to other parts of his body, ossifying his tendons and muscle and fusing his joints. When Harry turned 20 he became paralyzed and his body had completely fused together from his vertebrae. Harry died in November 1973,
FOP is one of the rarest, most disabling genetic conditions known to medicine. No medical therapy is known for FOP. The University of Oxford and the University of Pennsylvania are currently the two main FOP research institutes; however, others around the world are also looking for a cure. It is said that gene therapy such as bisphosphonates and corticosteroids are the hope for FOP.
For more information on Harry Eastlack and his disease visit his site in the Mütter Museum located on 19 S 22nd St, Philadelphia, PA 19103.