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Transcript of FOP
•Bone fusion in toe
•Swellings in fibrous tissue
•Swellings in aponeuroses , fasciae, tendons
•Progressive fusion of neck vertebrae
•Fibrous tissue ossification
•Restricted arm, joint, knew, wrist, shoulder, spine, neck mobility
•Mutations in the body’s repair system
•Genes mutate for no apparent reason
•A small amount of cases are inherited from a parent who has FOP
What Causes FOP? This is the skeleton of a person with FOP. Extra bones replace muscles. This is how you pronounce fibrodysplasia ossificans progressiva. fibro-dis-play-sha os-sih-fih-cans pro-gress-ev-a What does it do to the body? •Bone replaces fasciae, tendons, ligaments
•Affects the neck, spine, chest, shoulders, elbows, wrists, hips, knees, ankles, jaw, and many areas in between.
•Tends to occur in the neck, shoulders and the upper back early in life and in the hips and knees during adolescence or early adulthood.
•Bumps or injuries cause flare-ups and speeds up bone growth
•Surgeries to remove bone make condition worsen. Bones “repair” themselves with even more bone
•A flare-up occurs when the body starts to generate new bone. Leads to tissue swelling and discomfort.
•Sometimes people can go months or years without a flare-up
•Some people have very severe movement restriction. Others may have some range of motion.
•Creates “second skeleton” that traps the body
The malformed big toe is a very common symptom of FOP. This is the skeleton of Harry Eastlack. He lived with FOP until he died before his fortieth birthday. He gave his body to his physician, who danated his skeleton to the Mutter Museum. What research is being done? •University of Pennsylvania School of Medicine, is the only laboratory in the US dedicated to FOP research
•Funds spent on research - Approx. $1.5 million/year-
75% from FOP family fundraising and donations
25% from institutional support (NIH/NIAMS, Orthopaedic Research and Education Foundation)
University of Pennsylvania School of Medicine logo.