Huntington's Disease, sometimes known as Huntington's Chorea due to
the characteristic involuntary movement of head and limbs, is a
progressive neurological disease. It was made famous in several
ways. Woody Guthrie, the legendary folk singer, died of HD. HD has
dominant inheritance, and is ultimately fatal.
HD sufferers experience uncontrolled movements as the neurons involved
with various body functions die. Typically, physical decay happens
first, followed by mental decay and ultimately a vegetative state.
HD was among the first human disease that was located by positional
cloning on the human genome using modern marker technology (see references below). It was sequenced in 1993,
confirming that the malady involved a trinucleotide repeat. Despite
all the research on this tragic disease, there is no cure and very few
medications to alleviate symptoms.
There are now many web resources out there, but be careful. Some will
be disturbing. Some are merely outlets for people who are confused
or suffering. However, these and more constructive resources can
provide comfort and information for people with HD themselves, and
as importantly for caregivers. Below are some that I have found helpful.
In the near future, I intend to post menus and daily schedules that
have been helpful for my friend with HD. My friend has obsessive
compulsive disorder (OCD), which seems to be common with this
disease. If he was a creature of habit before, he is many times more
now. Here a few pieces of advice:
- Gusella et al. (1983) "A polymorphic DNA marker genetically
linked to Huntington's disease", Nature 306, 234-238.
- The Huntington's Disease Collaborative Research Group (1993) "A
novel gene containing a trinucleotide repeat that is expanded
and unstable on Huntington's disease chromosomes",
Cell 72, 971-983.
- See Renate
- Get a memory phone with big digits, and post all relevant phone
numbers on the wall near the phone.
- Have another large font sheet
plainly posted with the HD person's name, address, and phone number
clearly listed. This will be important for caregivers in emergencies,
and for daily activities such as deliveries.
- Keep a log of what the HD person likes to eat and drink.
- Keep a book of menus. Bland likes smoothies, which are easy to
swallow and can contain many useful foods. Have a rich but manageable
variety of choices, and let the HD person choose.
Return to Ewing page.