Behavioral Problems in Huntington's disease

The three most profound behavioral problems in Huntington's disease come from the dementia (loss of the ability to reason), the altered perception of the world and the changes in family dynamics.

Demenita

The dementia is technically called a sub-cortical dementia. Translated into real world terms this means that a person with Huntington's disease has difficulty performing a sequence of tasks, especially when distracted, while their ability to form new memory and to recall old memories is relatively well preserved. Thus a patient maybe able to do the wash if there is a list of instructions posted in the laundry room, but may fail at doing the wash if they are also trying to make a meal.

Distracting influences can impair the ability of a person with Huntington's disease to accomplish a task. For example, one may be able to eat easily in a quiet environment, but have great difficulty eating in a noisy, crowded room with both a television set and a radio playing.

Realistically evaluate what a person with Huntington's disease is capable of and what they are interested in doing. If they can no longer sort the clothes properly when doing the wash, perhaps they can still do the wash if someone else sorts the clothes (or wash everything in cold water).

As a person can do less and less for themselves, their sense of self worth may be lessened. Work to find tasks that the person is interested in doing and that they are capable of doing. They may not be able to paint as well as they used to, but they may still enjoy the act of painting. Activities of everyday life, such as bathing, food preparation and other acts may become more difficult for them to accomplish. If these acts can be modified, the person can still maintain the feelings of accomplishment and independence that come with the ability to help themselves.


When confronted with an unwanted behavior, it is important to look at what the behavior represents and what it accomplishes. As Huntington's disease advances the ability to communicate diminishes, First there is slowing in the production of words. As words become more sparse, the content is still there, sometime stripped of pronouns and adjectives. Eventually the person may not be able to use speech to communicate. It is at this point that many obnoxious behaviors occur.

Example: a person spits out the food that they are given to eat. What are the possible reasons:

Lets face it, canned peas taste pretty bad. Maybe they would like fresh peas instead.

In this case the behavior can convey many different meanings. Solving the behavior for the meaning can eliminate the problem. Remember that someone with Huntington's disease is still a person. They may not be the same as they were several years ago, but they deserve to be treated with respect and understanding to help preserve the quality and dignity of their life.

Compromise Solutions

Sometimes the solution is to relax one's expectations. Another example, provided by Dr. Rubin, was a middle aged man with Huntington's disease who could feed himself, but preferred to be fed by an attendant. His rational was that it took such a long time to feed himself that he did not have time left over to go fishing, an activity that he enjoyed much more than feeding himself.

Aggression

As part of the sub-cortical dementia in Huntington's disease, a person with Huntington's disease loses their ability to gait emotion. When confronted with the denial of their desire a person with Huntington's disease may respond with a temper tantrum instead of responding with a more polite and socially acceptable response "why not?" Aggression is a typical response in children and in some adults when their immediate desires are not met. This can be severely worsened in Huntington's disease by the person's inability to gait or control wide fluctuations in emotional response. Just as they can rapidly escalate into severe anger and belligerence, they can also calm down remarkably faster than their caregivers. Understanding that this is part of the disease can help in learning how to modulate this behavior.

Once a pattern of behavior is established it may become difficult to break. If you initially decline a request (e.g., to allow them to watch a television show), they respond angrily and then you give in to their original demand, you have taught them that being aggressive and belligerent allows them to accomplish their goals. Along the same line of teaching behaviors, if you constantly remind someone that they were inappropriate (e.g., "you were out of control") you may also be reinforcing the behavior. Try not to concentrate on talking about the detrimental behaviors, try to focus on the beneficial behaviors. If you think that this sounds like a lesson in child rearing, you are correct. Children learn from everything that they see and do. So do adults, irregardless of whether they have Huntington's disease or not. By focusing on the inappropriate behaviors you may actually be reinforcing them and de-emphasizing the desired behaviors.

When aggression reaches the point where you have a concern about harm to yourself, to the affected person or to others involved in the care of a person with Huntington's disease, you must get professional help as soon as possible. Behavioral modification techniques can work, but there are times when medications may be clearly needed as an adjunct to other techniques. Health care providers with expertise in Huntington's disease can be most helpful in this area.

Irritability

We all can become irritable if we are tired, cold, hungry, isolated in pain... These are the basic necessities of life. When these needs are met, then we can work towards other goals and aspirations. If someone with Huntington's disease is irritable, it is imperative that you try to figure out why. This is especially true when the disease has advanced to the point that communication is difficult. So, see if they might be hungry or thirsty, look to see if something may be causing pain or discomfort. If that fails there are other things that should be considered.

Apathy, withdrawal from activities, social isolation, changes in appetite and weight, wide mood swings are all components of depression. About one out five adults in North America will develop at least one episode of clinical depression in their lifetime, making it the most common psychiatric disorder. Depression can be caused by either a reaction to an external event (e.g., death of someone who is close to you, finding out one is seriously ill) or endogenous (from within). Depression is frequently an initial manifestation of Huntington's disease. Depression can also occur in the middle to later stages of Huntington's disease as a person is confronted with their diminished ability to do things and by social isolation. As one of our patients said; "Why don't they come and visit me? I'm not contagious." Depression can be treated in many ways including:

Sometimes these efforts are not successful and psychotherapy and possibly medications may be needed. Depression should be aggressively treated in people with Huntington's disease. Not only can depression be easily treated, but by treating it the quality of a person's life can be greatly improved. The consequences of not treating depression can include: worsening the depression, loss of contact with friends and family and the very real risk of suicide.

Sometimes depression can be managed without medications, even caffeine has some antidepressant effects along with its stimulating effects. As one of the longest continually used stimulants, caffeine has a good track record and relatively few bad effects, such as withdrawal. For imformation on other methods of treating depresion, including medications, look at depression at Yahoo for more information on depression. If you are worried that the one you care for is contemplating suidice visit the home safety page of this site for information about making your home safe for the person with Huntington's disease.

Obsessions and Compulsions

An obsession is a constant concern or worry about one or more things that may or may not be grounded in reality. An example may be worrying that one's disability status may be taken away. While this may in some cases be a reality based worry, in most cases once disability is granted for Huntington's disease in the United States it is not taken away. A compulsion is a feeling of needing to o actually performing a task over and over. Compulsions, like obsessions can be grounded in reality. If you realize that your memory is not what it used to be, you may need to check to be certain that you have locked the door or turned off the stove.

When obsessions or compulsions get to the point that they are consuming too much of one's time or they get to the point where the compulsion of obsession is in itself worrisome, something should be done. Sometimes, calm rational discussions with the person can help them to confront the fear that has led to their obsession and allow them to cope. In the above example of disability, the caregiver as well the health care provider can help to reassure the person with Huntington's disease that there disability status is not in jeopardy.

A compulsive behavior in a patient who has trouble communicating can be difficult. Carol Moskowitz and Lori Quinn have taken care of a women with Huntington's disease with excessive thirst. They found that when they cooled her room to around 60 degrees, her thirst diminished. Her compulsive thirst was apparently a reaction to a feeling of being hot. In a colder environment the thirst abated.

Hallucinations

Hallucinations can vary between benign misperceptions of reality to horrifying experiences that have no basis in reality. They can involve sounds (especially voices), visions, olfactory (smells), gustatory (taste) and tactile (feeling of being touched. An example of a benign tactile hallucination is that of a patient who feels that the chorea of his legs is caused by ghosts. A severe frightening visual hallucination could be the vision of a yawning mouth with bloody teeth. People with Huntington's disease may often have benign, or non-bothersome (to them) hallucinations. For example, a patient may stare off into space and laugh at something that appears to be just in front of them. Treatment for this type of problem may be as simple as teaching the caregiver not to be upset by the laughter. In the example above about the ghosts moving a young man's legs, convincing him to avoid frightening movies and television shows.

Paranoia can be associated with hallucinations or even trigger hallucinations. One of our patients who was in a nursing home became paranoid that people were trying to kill him, when the nursing placed a series of terminal patients as his roommate. When he was matched with a roommate who more closely matched his age and general health the paranoia diminished. The spouse of this patient taught him to orient himself by looking at all four corners of his room upon awakening and labeling where his clothing and shoes should go. By training him to place his possessions in the same place every day, they were able to decrease his misperception that people were stealing his shoes, when in fact he had not put them away where they belonged.

Medications may be needed to help to alleviate or control some of the more severe behavioral problems. The medications should be a second line of treatment after attempts have been made to understand or modify the behavior. One excellent source for health care providers is the second editions of "A Physicians Guide to the Management of Huntington's Disease" by Adam Rosenblatt, M.D., Neal Ranen, M.D., Martha Nance, M.D., and Jane Paulsen Ph.D., printed by the Huntington's Disease Society of America.

Family Dynamic Alterations

The tradition roles in the family involve the parents that provide leadership, nurturing, care, comfort and necessities and that of the child that must rely on the parent to provide these items. As we grow and mature the child takes on the roles of the parent as they become more independent. Eventually the child becomes a parent in their own right. They then have children of their own to take care of and also their parents may also require help as the grandparents now need assistance.

When a member of a family is ill, these roles can rapidly shift. While a child who is ill still remains a child, their level of independence diminishes. When the parent is ill, the child will need to take on adult roles while the ill adult becomes more child like in their need to be cared for. If the illness is short lived, the roles will be reestablished rapidly. If the illness is life long, as is Huntington's disease, once a person is symptomatic, the roles may never be restored.

When the parent is ill When a parent is ill and is no longer able to care for their child (children) the child's reactions may be:

Some of the results of the parent's illness may be: When these problems become so severe that the family has trouble adapting, then counseling is needed. Counseling sessions can help to teach family members techniques to deal or work with the problems. Oftentimes only one or two sessions are needed in order to get the family working smoothly again.

Another excellent source of support can be found from other families with Huntington's disease.

WWW Resources of note:

 Alzheimer-Europe

This page was last updated on August 21, 2000
Return to the Introduction page of Caring for People with Huntington's Disease
KUMC Pulse This home page is supported by the Department of Neurology at the Kansas University Medical Center and by the Huntington's disease support groups. 
Suggestions and comments are welcome. Please mail them to me at: Dubinsky@kuhub.cc.ukans.edu