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Huntington's Disease: A Caregiver's Guide

To the uninitiated, a first read-through of the symptoms of Huntington’s disease is reminiscent of the behaviors of very early childhood. Those afflicted with what is sometimes also called Huntington’s chorea eventually can’t walk or communicate the way they once did. They need help eating and drinking and even toileting and become dependent on others for almost everything. But a deeper read promises heartbreak that only the caregivers of those with this hereditary disorder and the patients themselves can truly understand; for in spite of the fact that those living with Huntington’s disease are as needy as our own young, they are also completely coherent all the way to the end of life. They know what’s happening but there’s nothing they can do about it. Whole inside but prisoners of their own bodies, they yearn to communicate but can’t. They understand what’s being said but can’t always reply. They try to walk and carry on but instead stumble or fall or even become confined to wheelchairs. The indignity of the disease can cancel out pride, self-esteem and joy, and it is the wise caregiver who learns early-on the importance of compassion, of reaching out to another who is intellectually an equal but physically, verbally and emotionally has once again been relegated to infancy.

First described by Dr. George Huntington in 1872, Huntington’s disease is now considered a fairly common genetic disorder. Passed from parent to child in a genetic mutation of a normal gene discovered in 1993 called the Huntington gene, the disease is egalitarian, affecting as many men as women and no more prevalent in one race or ethnicity than another. While onset is most common between the ages of 30 and 45, some are afflicted as early as age two. (Children living with Huntington’s disease rarely live to adulthood.) The sobering facts are these – children of parents who carry the gene have a 50/50 chance of inheriting it, and those who have the gene will develop the disease. There is no question or possibility otherwise, and the disease is fatal. Over time, Huntington’s disease affects the afflicted person’s ability to walk, talk, think and reason. Its impact is felt in three major areas – emotional, physical and cognitive.

Early symptoms affect cognition and mobility. Other indicators include depression, mood swings, forgetfulness, clumsiness, involuntary twitching and lack of coordination. As the disease advances, concentration and short-term memory are affected and involuntary movements of the head, trunk and limbs increase. Walking, speaking and swallowing skills decrease. Eventually the patient becomes totally dependent. Death is often caused by choking, infection or heart failure. While there is currently no cure, those living with with the disease must learn to live with it, for life after diagnosis can last as long as 20 or 30 years. Physical fitness is very important – those who are fit handle the disease’s progression better than those who are not.

Caregivers of those living with Huntington’s disease know as perhaps not many others do the complexities of lending support to someone who is involuntarily traveling backwards emotionally while continuing to relate to them on a level intellectual playing field for as long as possible. Faced with behavioral problems, particularly those of dementia, an altered perception of the world and changes in family dynamics, the caregiver must learn to speak a new language, one whose vocabulary is not verbal but behavioral. Learning to look beyond unwanted behaviors to see what the patient is trying to say or demonstrate is a pre-requisite to successful caregiving, for it is up to the helper to see through, interpret, analyze and deliver while still preserving the patient’s dignity and self worth. Imagine if you will the difficulty of knowing what you want to say or do but being unable to follow through. It is this difficult truth that becomes the new reality of those living with Huntington’s disease. Caregivers function as life preservers, helping love ones hold onto a remnant of the life they once knew.

 There is absolutely no doubt that it takes a special person with a heart of gold who can look beyond, understand, translate and accomplish what is needed in the face of the disease and debilitation of a loved one. It is an ability to translate the language of disability into productivity that will enable a caregiver to observe and see what he or she is capable of and still interested in doing and adjust expectations accordingly. It is in helping to find things he or she can still do that self worth is saved and confidence restored. Aggression, depression, obsessions and compulsions, hallucinations and paranoia are all behaviors the caregiver may have to confront. Add to that irrevocably changed family dynamics, and it becomes clear that families must work together to learn new, unfamiliar terrain. The imbalance is that the lion’s share of the work will fall to the caregiver. Following are some tips that should be helpful:

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