Huntington disease is an inherited disorder. Many people with Huntington's have already had children by the time they are diagnosed. These children have a 50-50 chance of inheriting the gene for the disorder.
How the disease progresses
The degeneration of brain cells in Huntington disease starts out slowly. Early symptoms may include mood swings, depression, irritability, trouble driving, learning new things, remembering a fact, or making a decision.
As the disorder continues, and more brain cells are destroyed, it becomes increasingly difficult for the person with Huntington's to concentrate on tasks, and he may have difficulty feeding himself and swallowing. Purposeless movements of the face, arms, and legs, called chorea, may develop. How fast the disorder progresses varies from person to person, but the average survival time from diagnosis is 16 years. In other words, this disorder has a long, slow course in which the person afflicted gradually loses control of his/her body movements and mental abilities.
Treatment
There is no cure, despite the fact that scientists discovered the gene for Huntington's in 1993. There are medications that can help control the chorea and behavioral disturbances, but these medications have side effects that cause other kinds of problems such as fatigue or restlessness. Therefore, drug therapy is usually limited to people who have severe symptoms.
The two main ways used to help reduce the effects of the disorder are for the person with Huntington's to keep active and physically fit through exercise, and the use of supportive care. Supportive care means using aids and services such as psychotherapy, physiotherapy, speech therapy, communicative and memory aids, social support services, and dietary advice.
Research and the future
Although cracking the genetic code for the disorder has not yet led to a cure, discovery of the Huntington's gene has allowed scientists to create strains of mice that carry the gene. Although the mice don't develop exactly the same symptoms as human beings do, the result is close enough that scientists can try out treatments and gene therapies. Scientists can also test possible drugs for ones that slow the damage the disorder causes.
Sources:
1. Adis International Limited. (1999). Drug therapy limited in patients with Huntington's disease. Drugs & Therapy Perspectives, no. 14, vol 12, 5-7.
2. National Institute for Neurological Disorders and Stroke. Huntington's Disease.
3. Saltus, Richard. After bad gene found, no quick leap to
a cure. The Boston Globe, August 7, 2000.
