Friedreich’s Ataxia Statistics

Frequency:

In the US: FA occurs in 1-2 per 100,000 of the US population.

Internationally: Prevalence of FA is 1-2 per 100,000 of the international population; prevalence appears to be slightly higher in Quebec.

Progression: The rate of progression of FA is variable, but more than 95% of individuals with FA cannot ambulate by the time they are aged 45 years, and, on average, patients lose the ability to walk 15 years following onset of symptoms. Age at death is rather variable. Reported mean ages have been in the mid fourth decade of life, although survival into the sixth and seventh decades of life has been documented. Death tends to be earlier if heart disease and diabetes are associated.

Race: All races are affected.

Sex: Men and women are affected equally.

Age: Symptoms may begin in infancy or in the third decade; however, symptoms usually begin when an individual is aged 8-15 years.

Prognosis: The rate of progression of FA is variable. The mean age of loss of ambulation is 25 years. Death usually occurs in the mid fourth decade of life. However, survival into the sixth and seventh decades of life has been documented. Death often is related to cardiomyopathy and diabetes, but aspiration pneumonia due to dysphagia also may shorten the lifespan of patients with FA.

Patient Education: Because FA is inherited is autosomal recessive disease, parents of a child diagnosed with FA are both obligate carriers of an X25 gene mutation. Siblings of patients with FA have a 25% risk of being affected. Offspring of patients with FA all inherit one mutant allele from the affected parent. However, these children only have a risk of being affected if the unaffected parent is a carrier of a mutation in the X25 gene. The carrier status of the unaffected parent can be determined by DNA testing.

Source: www.emedicine.com/orthoped/topic109.htm