Part 3: Huntington's Disease
In this series we will take an in-depth look at the various types of dementia and how to improve care in a long-term care setting. There are several lesser known dementias affecting our seniors. As part of their care team we need to educate ourselves on how to better understand and meet the needs of those living with these dementias.
Huntington's Disease (HD) is a genetic disease that is associated with or may lead to dementia. HD affects the whole brain, but mostly the basal ganglia. It generally begins earlier in life, between ages 30 and 45, and is characterized by mood swings, lack of coordination and involuntary movement of the head, trunk and limbs. Its first diagnosis was in 1872 when George Huntington, MD, presented a disease featuring "hereditary nature, adult onset, chorea, mind impairment…with a tendency to insanity and suicide.” Compared to Alzheimer's disease, people with dementia from HD have fewer memory issues but more problems with language/communication and decision-making.
An estimated 30,000 Americans are living with HD, which is categorized by three stages. The early stages involve subtle changes in coordination, trouble with problem solving and a depressed or irritable mood. During this stage people often start treatment with antidepressants. During the middle stage involuntary movements become more pronounced. Physical and occupational therapy can help with maintaining some control, but there is a significant impact on the activities of daily life. Patients also experience diminished speech and swallowing abilities. The late stage requires total dependence on care from others. The person with HD can no longer walk or speak, but they will maintain the ability to comprehend. Choking risks are high while weight loss and malnutrition are common.
1.Good nutrition is a key factor in managing the disease. A low fat, high vegetable/fruit diet is key.
“Research has shown that people with Huntington’s Disease often have a lower than average body weight for height, and may have higher than average calorie needs. This may be due to chorea, metabolic changes or some other factor yet undiscovered. There is also some evidence that maintaining a body weight slightly above ‘desirable’ weight will facilitate control of the disease. Therefore, people with HD should be encouraged to eat and every effort should be made to make eating enjoyable.”
Caregivers should use adaptive equipment to encourage the person to feed themselves as long as possible. They may consider tube feeding in some cases.
2.Speech and language therapy (SLP) is important.
“The SLP can be helpful at all stages of the disease. In the early stages, the SLP can assist in developing strategies to help an individual with HD compensate for the communication, swallowing and cognitive problems he or she might be experiencing. As the disease progresses, the role of the SLP additionally evolves into helping preserve and maintain the person's highest level of independence in communication and swallowing. The SLP can also evaluate a person's ability to use augmentative or alternative communication (AAC) devices and techniques that can supplement the individual's verbal communication.
“The SLP can evaluate a person's swallowing function and make recommendations that involve positioning issues, feeding techniques, diet consistency changes and education of the person with HD and family members or caregivers. Special testing known as videofluoroscopy (or a modified barium swallow) can be done by an SLP and a radiologist to determine if a person is actually aspirating a particular consistency. This test provides an inside view of a person in the act of swallowing food or liquid and can be a useful tool in developing strategies for safe swallowing.”
Find communication tips geared toward HD for both speaker and listener from the American Speech-Language-Hearing Association.
3.Medications from chorea are often used to help alleviate involuntary movements. There are several drugs currently in testing stage two or three for HD, including:
Tetrabenazine is used to treat the involuntary movements (chorea) of Huntington’s Disease. It is a vesicular monoamine transporter 2 (VMAT2) inhibitor, and acts by decreasing the levels of the neurotransmitter dopamine in the synapse (space between neurons). On August 15, 2008 the US Food and Drug Administration (FDA) approved the use of tetrabenazine to treat chorea associated with HD.
This drug, in stage three of testing, is an investigational drug with the same mechanism of action as tetrabenazine to treat the involuntary movements (chorea) of Huntington’s Disease. SD-809 ER tablets release more slowly and are broken down by the body more slowly than tetrabenazine. These differences may mean1) the drug may be taken fewer times per day than tetrabenazine; 2) a lower dose may be as effective at controlling chorea and 3) this may improve how well the body reacts to the drug compared to tetrabenazine, with potentially fewer side effects plus good chorea control.
There are currently 8 other drugs currently in stage two of testing.
There is a great deal we can do to help our residents and their families as they face the realities of Huntington’s Disease. In our next newsletter, we will continue our series on dementia. Until then, you can find more information on Huntington’s Disease from the following resources:
 Huntington’s Disease Society of America
 American Speech-Language-Hearing Association