Fibrodysplasia Ossificans Progressiva
The human body is a remarkable structure! Physicians and scientists around the world have been fascinated by its intricate systems for centuries. Fibrodysplasia ossificans progressiva, also know as FOP, has been of peculiar interest to those who have happened to stumble upon it. FOP is a condition in which soft tissue permanently transforms into bone over time. This abnormal growth is not limited to a particular area of the body but forms in muscles, tendons ligaments, and other connective tissue. This fatal disease, in essence, traps “the body in a prison of bone” (Massey). FOP is a very rare and unusual genetic disease and is “caused by the mutation of a gene in the bone morphogenetic protein” (WebMD). …show more content…
This along with the later rapid formation of other nodules on the neck, back and shoulders are used to make a proper diagnosis. FOP is also characterised by prolonged joint stiffness accompanied by excessive pain and discomfort. The disease affects a majority of the body excluding the diaphragm, tongue and also the cardiac and smooth muscles. Rather than crawling, most infants will “scoot on their buttocks; then get up and walk. Most cannot crawl because joints in the back of the neck have not formed properly or have fused, thus limiting movement” (IFOPA). Many children suffering from FOP have an “80 percent chance” (USCF) of being misdiagnosed due to the lack of knowledge concerning the disease amongst medical professionals. FOP is often misdiagnosed as “cancer, aggressive juvenile fibromatosis, also called desmoid tumors, or progressive osseous heteroplasia, another rare disease characterized by the abnormal growth of bone” (UCSF). Signs and symptoms vary from person to person but all lead to fatal …show more content…
Bone growth can begin to constrict the lungs and slowly suffocate the individual. The gradual or rapid stiffening of joints causes immobility and patients are commonly placed in wheelchairs or medical chairs. Acute thrombophlebitis, a blood clot in a vein resulting in inflammation, is yet “another a complication that can occur in patients with FOP due to generalized immobility and associated venous stasis” (NCBI). Other stiffness in the jaw can make it difficult to eat or speak. “Hearing impairment is a common feature of FOP and occurs in approximately 50% of patients due to middle ear ossification or neurological damage” (NCBI). Individuals dealing with FOP are very sensitive to illness or trauma. Even medical procedures, a fall, flu or even dental work can trigger a “flare-up,” accompanied by an increase in bone on the affected
The human body is a remarkable structure! Physicians and scientists around the world have been fascinated by its intricate systems for centuries. Fibrodysplasia ossificans progressiva, also know as FOP, has been of peculiar interest to those who have happened to stumble upon it. FOP is a condition in which soft tissue permanently transforms into bone over time. This abnormal growth is not limited to a particular area of the body but forms in muscles, tendons ligaments, and other connective tissue. This fatal disease, in essence, traps “the body in a prison of bone” (Massey). FOP is a very rare and unusual genetic disease and is “caused by the mutation of a gene in the bone morphogenetic protein” (WebMD). …show more content…
This along with the later rapid formation of other nodules on the neck, back and shoulders are used to make a proper diagnosis. FOP is also characterised by prolonged joint stiffness accompanied by excessive pain and discomfort. The disease affects a majority of the body excluding the diaphragm, tongue and also the cardiac and smooth muscles. Rather than crawling, most infants will “scoot on their buttocks; then get up and walk. Most cannot crawl because joints in the back of the neck have not formed properly or have fused, thus limiting movement” (IFOPA). Many children suffering from FOP have an “80 percent chance” (USCF) of being misdiagnosed due to the lack of knowledge concerning the disease amongst medical professionals. FOP is often misdiagnosed as “cancer, aggressive juvenile fibromatosis, also called desmoid tumors, or progressive osseous heteroplasia, another rare disease characterized by the abnormal growth of bone” (UCSF). Signs and symptoms vary from person to person but all lead to fatal …show more content…
Bone growth can begin to constrict the lungs and slowly suffocate the individual. The gradual or rapid stiffening of joints causes immobility and patients are commonly placed in wheelchairs or medical chairs. Acute thrombophlebitis, a blood clot in a vein resulting in inflammation, is yet “another a complication that can occur in patients with FOP due to generalized immobility and associated venous stasis” (NCBI). Other stiffness in the jaw can make it difficult to eat or speak. “Hearing impairment is a common feature of FOP and occurs in approximately 50% of patients due to middle ear ossification or neurological damage” (NCBI). Individuals dealing with FOP are very sensitive to illness or trauma. Even medical procedures, a fall, flu or even dental work can trigger a “flare-up,” accompanied by an increase in bone on the affected