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Huntington’s Disease (HD) is a devastating, hereditary, degenerative brain disorder that 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, which in turn profoundly disrupts the lives of the entire family; emotionally, socially, and economically.
Named for Dr. George Huntington, who first described this hereditary disorder in 1872, HD is currently recognized as one of the more common genetic disorders in the U.S. More that 30,000 Americans are actively afflicted with HD, and more than 250,000 are “at risk” of inheriting the disease from an affected parent. HD strikes as many people as hemophilia, cystic fibrosis or muscular dystrophy.
HD affects males and females equally across all ethnic and racial populations. Each child of a person with HD has a 50/50 chance of inheriting the fatal gene. Anyone who carries the mutated gene will develop the disease. While HD typically begins in mid-life between the ages of 30 and 45, physicians have identified cases of Juvenile HD, where onset may be as early as the age of 2. Children who develop the juvenile form of the disease rarely live to adulthood.
HD symptoms fall into three categories: motor problems; personality changes; and cognitive difficulties. Motor problems can include deterioration of coordination, walking and balance, slurred speech, difficulty in swallowing and the presence of involuntary movements such as twitching, spasms and the appearance of being intoxicated. Personality changes many include irritability, temper tantrums, depression, paranoia, and violent outbursts. Cognitive difficulties evolve later in the disease, which hinders memory, mental flexibility, fluency of thought and communication. Eventually complications such as choking, infection or heart failure result in fatality.
At present, there is no effective treatment or cure. |