Huntington's
Disease (HD)
By Doretha Ennels
First Appearance: October, 1998 issue of Positive Reflections
What is HD?
Huntington’s Chorea, also called Huntington’s Disease, was first identified
in 1872 by Dr. George Huntington, a New York physician. "Chorea", a Greek word
for dance, refers to involuntary movements of the head, trunk, and limbs that affect
people with Huntington’s, according to the Huntington’s Disease Society of
America.
Huntington’s Chorea is a rare hereditary disease of the nervous system and the
brain said Dr. Clifford Reed, a Reading Hospital neurologist. It affects all racial and
ethnic groups. "It is a terrible disease that does not usually express itself until
the fourth or fifth decade", Reed said. Reed also said that the genetic abnormality
that cause Huntington’s Chorea appears on the fourth chromosome. Humans have 23 pairs
of chromosomes. People who carry the gene will eventually develop Huntington’s
Chorea, which is terminal. Doctors prescribe some medications that treat specific symptoms
of Huntington’s, but there is no cure for the disease. People with
Huntington’s lose their memory and need to be institutionalized as the disease
progresses. Most people who suffer from Huntington’s disease die within 15 years of
the onset of the disease according to the Huntington’s Society of American.
Each child of a HD affected parent has a 50% chance of inheriting the disorder and is
said to be "at risk". HD usually progresses over a 10 to 25 year period.
Symptoms generally appear between 30 and 50 years of age, but have appeared as young as
2 and as old as 70.
Characteristics/Symptoms:
HD does not skip generations, if you do not inherit the gene you cannot pass it on. If
you carry the gene, you will develop the disease if you live long enough, and you can pass
it on. It is a challenge to the whole family whether they have the disease or not.
They are challenged emotionally, socially, and economically.
Research breakthroughs:
After a 10 year search, scientists announced in March of 1993 that they have found the
gene which causes HD. Researchers are now trying to determine what the gene’s
normal function is and how the flawed gene causes HD. . Many avenues of
research are being investigated in the search for a treatment or cure for HD.
Genetic Testing:
The gene discovery has made possible a new predictive test for HD allowing those at
risk to find out whether or not they will develop the disease. The new test requires
a blood sample from the person being tested. Some testing centers request a sample
from a parent for confirmation purposes. Pre– and post test counseling is
necessary and important part of the testing procedure. People wishing to be tested
should seek out a specialized HD testing center.
For a list of testing centers, call or write to HDSA (Editor’s note: Address is
given at the end of the article)
To Find Out More:
For information about Huntington’s Chorea contact the Huntington’s Disease
Society of America Delaware Valley Chapter at 1427 Vine St., MS 950, Philadelphia, PA
19102 or reach them by phone call 215-762-6667.
The National Headquarters of the Huntington’s Disease Society is located at 158 W.
29th St., 7th floor, New York, New York 10001 or call 1-800-345-HDSA
The forerunner of the Huntington’s Society, the Committee to Combat
Huntington’s Disease, was founded in 1967 by Marjorie Guthrie, after her husband,
folk singer Woody Guthrie, died of the disease.
