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Friedreichs
Ataxia is a life shortening, neurodegenerative and currently incurable
disease, similar to Muscular Dystrophy, attacking the cerebellum causing
severe scoliosis and eventually leading to swelling of the heart,
known as cardiomyopathy. About one in 50,000 people in the United
States have Friedreich's Ataxia. Onset of symptoms is usually between
the ages of 5 and 15, but can also occur even earlier or significantly
later. Symptoms include: muscle weakness and loss of coordination
(ataxia) in the arms and legs; impairment of vision, hearing loss
and slurred speech; aggressive scoliosis (curvature of the spine);
carbohydrate intolerance or diabetes mellitus; and serious heart condition
(enlarged heart - hypertrophic cardiomyopathy).
These symptoms reflect the death of cells in certain parts of the
nervous system. The mental capabilities of people coping with Friedreich's
Ataxia, however, remain completely intact. For most, progressive loss
of muscle strength and control leads to motor incapacitation and the
full-time use of a wheelchair by the late teens or early twenties.
Many require surgery for their scoliosis. There are currently no treatments
or cures. Because the course of the disorder is progressive, most
young people diagnosed with Friedreich's Ataxia require mobility aids
such as a cane, walker, or wheelchair by their teens or early 20s.
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