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Huntington's Disease: A Caregiver's Guide

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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 become incapacitated.

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 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:

The caregiver must accept responsibility for but not control conversations. Patients should be encouraged to speak slowly. Messages should be repeated or rephrased and simplified as necessary. The caregiver should ask yes/no questions to gain clarity and should try to have an understanding of what the family member may be discussing so as to make comprehension easier. If the speaker gets distracted, he or she should be refocused. Presenting two choices instead of asking open-ended questions can help. It may become difficult not to cause frustration or anger, but the caregiver must exercise great patience to preserve equanimity. Addressing requests promptly helps prevent angry outbursts.

Non-verbal modeling and participating in activities helps encourage patients to participate and prevents apathy. In spite of the fact that the lurching, dance-like, abnormal movements of a patient with Huntington’s disease can be awkward, making both of you feel uncomfortable, encouraging involvement in activities is important and can fight depression. Sometimes activities can be found in which your loved one can participate that will give a caregiver much-needed respite.

Remember that in late stage Huntington’s disease, the a person may not be able to verbally communicate any longer, but he or she will not have lost the ability to understand. It is at this point that it becomes critical for the caregiver to maintain communication.. He or she still has much to share, and helping them do that as well as giving them something to think about will be invaluable.

When eating and swallowing become difficult, the caregiver should understand that eating in a peaceful environment will help. Your loved one should not be permitted to eat when he or she is upset or anxious. Good lighting will permit the food to be seen more easily. Caregivers should not talk when the family member is eating. Good posture is critical, and the patient should be encouraged to sit up straight, chin pointed to chest and head tilted down for easy swallowing. Try to remain at the table for 20-30 minutes after the meal, and keep head movements to a minimum.

Because people living with Huntington’s disease are moving constantly, they are often very hungry. An increased calorie load means that eating several smaller meals more frequently through the day may be better than three large meals. Encourage your love one to take smaller bites and eat smaller pieces of food. Drinking from a straw is helpful, as is eating blended, softened and pureed foods. Dry, tough and stringy foods should be avoided, and thicker liquids are easier to swallow. Caregivers should know the Heimlich maneuver.

Keeping in mind that a person with Huntington’s has an altered sense of reality, the caregiver must think through what can be reached, how he or she will think of it, and whether or not it would be dangerous to them.

Install grab bars in bathtubs and shower stalls. Shower benches and flexible shower heads prolong independence. Textured strips should be put on the floor of the tub and shower for traction. Finally, raised toilet seats and handles give the patient something to hold onto.

In the kitchen, put chemicals out of reach. Supervision is important such as making sure the stove isn’t turned on by accident. Put items to be used within easy reach, and keep the counters free of clutter.

Minimize furniture so that your loved one doesn’t have to maneuver around it. Remove rugs and carpets where possible since they are stumbling hazards. Pre-programmed telephones make dialing easier.

A tendency towards paranoia might mean that your loved one may try to lock you out of your home. Prevent this by installing locks that can be opened from the outside as well as the inside. Keyless entries are a good idea. For those who tend to leave home, nametags sewn inside clothing with their name and phone number are useful. Also, door alarms will allow you to know when someone is coming and going.

Because Huntington’s disease is progressive, the symptoms exacerbate as the disease advances. Late stage symptoms of the disease include dystonia, sustained abnormal postures that include facial grimaces, twisted neck and arched back; chorea or choreoathetosis, a condition in which involuntary movements like jerking, twisting and writhing become more pronounced; a slowness of voluntary movements to include abnormal eye movements and gait disturbances; an inability to regulate the speed or force of movements; an inability to initiate movement; slowed reactions; difficulty speaking and swallowing due to involvement of the throat muscles; localized or generalized weakness and impaired balance ability; and rigidity.

In most people, chorea peaks and then declines while rigidity and slowness in movement may become more significant. Rigidity and spasticity are late stage problems that can affect gait, lead to falls and eventually lead to the need for a wheelchair. While an enumeration of these complications paints a bleak picture and must illustrate the feeling of helplessness faced by caregivers, it is important for others to understand not only what the patient is living but what their caregivers face daily.

There is great reason for hope in combating Huntington’s, however. Since 1991, Columbia University’s HDSA Center for Excellence has been working tirelessly to provide help to those who have the disease .  One day at a time, with the help of people who care and want to find answers, through the courage of those living with the disease and because of the 24/7 dedication of caregivers, there will be answers. Those who live it know that Huntington’s disease does not have to have the last word. It can be a statement of victory from those who learn in each moment to overcome overwhelming odds.

Helpful Hints for Caregivers

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