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Huntington's
Disease(HD)
is a devastating, degenerative brain disorder for which
there is, at present, no effective treatment or cure. HD slowly diminishes
the affected individual's ability to walk, think, talk and reason. Eventually,
the person with HD becomes totally dependent upon others for his or her care.
Huntington's Disease profoundly affects the lives of entire families: emotionally,
socially and economically.
Named for Dr. George Huntington,
who first described this hereditary disorder in 1872, HD is now recognized
as one of the more common genetic disorders. More than a quarter of a million
Americans have HD or are "at risk" of inheriting the disease from an affected
parent. HD affects as many people as Hemophilia, Cystic Fibrosis or muscular
dystrophy.
Early symptoms of Huntington's Disease may affect
cognitive ability or mobility and include depression, mood swings,
forgetfulness, clumsiness, involuntary twitching and lack of coördination.
As the disease progresses, concentration and short-term memory diminish and
involuntary movements of the head, trunk and limbs increase. Walking, speaking
and swallowing abilities deteriorate. Eventually the person is unable to
care for him or herself. Death follows from complications such as choking,
infection or heart failure.
HD typically begins in mid-life, between the ages
of 30 and 45, though onset may occur as early as the age of 2. Children who
develop the juvenile form of the disease rarely live to adulthood. HD affects
males and females equally and crosses all ethnic and racial boundaries. Each
child of a person with HD has a 50/50 chance of inheriting the fatal gene.
Everyone who carries the gene will develop the disease. In 1993, the HD gene
was isolated and a direct genetic test developed which can accurately determine
whether a person carries the HD gene. The test cannot predict when symptoms
will begin. However, in the absence of a cure, some individuals "at risk"
elect not to take the test.
Since the discovery of the gene that causes HD,
scientific research has accelerated and much has been added to our understanding
of Huntington's Disease and its effects upon different individuals. By continuing
to increase our investment in both clinical and basic HD research each year,
breakthroughs in treatment - and a cure - will be forthcoming.
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