Friedreich’s ataxia (FA) is a rare, inherited multisystem disease with prominent CNS and cardiac features and high unmet medical need. FA affects the nerves and spinal cord, causing loss of control of body movements (ataxia).
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A person with FA
Will usually need a wheelchair within 10 to 20 years of symptom onset
May be completely incapacitated in later stages of the disease
May have a shortened life span
MORTALITY IN FA IS MOST COMMONLY DUE TO CARDIAC COMPLICATIONS
of FA deaths are from cardiac dysfunction
0%
Only symptomatic treatment options are available, with none addressing the underlying cause of the disease—the defective frataxin gene
FA IS THE MOST COMMON HEREDITARY ATAXIA
Males and females are equally affected
One in every 40,000 to 50,000 people have FA
Approximately 9,000 patients in the United States and ~26,000 patients in the European Union are affected
A person with FA
The most common manifestations of FA are progressive neurological symptoms
LOSS of balance and coordination
LOSS of sensation in the arms and legs
LOSS of vision and hearing
FA is a multisystem disease
Cardiac
Hypertrophic and dilated cardiomyopathy, arrhythmia, and mortality
Skeletal abnormalities
Scoliosis and pes cavus
Hearing loss
Auditory neuropathy/dyssychrony and vestibular dysfunction
Endocrine
Diabetes, impaired fasting glucose, and impaired glucose tolerance
Vision
Nystagmus and oculomotor disturbances
Fatigue
Nearly all patients experience significant fatigue that impacts quality of life
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