What is Huntington's Disease?

 

Huntington's Disease (HD) is a devastating, hereditary, degenerative brain disorder that results in a loss of cognitive, behavioral and physical control, and for which, presently, there is no treatment or cure. HD slowly diminishes the affected individual's ability to walk, think, talk and reason. Symptoms usually appear in an individual between 30 and 50 years of age and progress over a 10 to 25 year period. Eventually, a person with HD becomes totally dependent upon others for his or her care. More than 30,000 people in the United States are currently diagnosed with HD. Each of their siblings and children has a 50 percent risk of developing the disease. Although medications can relieve some symptoms in certain individuals, research has yet to find a means of conquering or even slowing the deadly progression of HD.

HD was once thought to affect about one person in 20,000 but current estimates are closer to one in 10,000 people. As with other diseases of this sort, HD afflicts not only the affected person, but everyone in his or her family. HD is hereditary and each child of an affected parent has a 50-50 chance of inheriting the HD gene. This disease strikes individuals in the prime of their lives. The average onset of the disease is between the ages of 30-50 and the duration of the illness is approximately 10-25 years. Children as young as 2 and adults as old as 80 have developed HD.

In the early stages, perhaps even prior to diagnosis, there are some intellectual, motor, and emotional impairment. With the progression of the disease, the weakened individual succumbs to pneumonia, heart failure or other complications ultimately resulting in death.

The Huntington's Disease gene was discovered in the spring of 1993. This dramatic discovery has simplified the testing process, made it much more financially realistic and increased the accuracy of the diagnostic process. The cause of dying brain cells was discovered in the fall of 1997. This exciting breakthrough has enabled drug trials to begin and given renewed hope of finding a way to possibly slow the progression or prevent the onset of HD.

Presently, there is no effective treatment or known cure!!!

Symptoms
Early symptoms appear slowly. These can include involuntary movements of the body and limbs, marked personality changes, depression, withdrawal, euphoria, and increasing forgetfulness. As the disease progresses, speech becomes slurred, swallowing difficult, and gait unsteady. Reasoning and judgment become impaired.

Population Affected
Huntington's Disease is a hereditary disorder. Each child of a parent who has the Huntington's gene has a 50-50 chance of inheriting the disorder and is considered to be "at-risk". HD affects individuals of both sexes equally as well as all races and ethnic groups throughout the world.

Prevalence
Huntington's Disease, once thought of as rare, is now recognized as one of the more common hereditary diseases. In the past, families often hid HD as a shameful secret. This fact, coupled with frequent medical misdiagnosis of HD, has led to an underestimation of it's prevalence.

Current estimates are that up to 1 in 10,000 people in the United States have the disorder. HD creates a ripple effect because relatives are likely to experience the disease and it's varied consequences. It is therefore estimated that 1 in 1,000 Americans are directly affected by HD.
    




HD is named after Dr. George Huntington, an American physician, who was first to publish a precise description of the symptoms and a course of the disease in an article dated in 1872.

 

Presently, there is no effective treatment or known cure for Huntington's Disease!

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