Huntington's disease is a neurological disorder, a disease of the brain. Cells
in the central part of the brain known as the basal ganglia die. Since so much of the brain's activity passes through this area, the death of these cells affects virtually everything about a person-including movements, moods, and thinking processes.
But because the damage caused by HD is
only inside the brain, the person with HD may look relatively able-bodied until the later stages of the disease.
Caregivers often mistakenly assume
that changes in the person are due to lack of motivation, laziness or worse, and not to the disease itself.
HD is a genetic disease that you get by inheriting a defective gene for HD from one parent. Every child of a parent with the defective gene for HD has a 50/50 chance of inheriting the gene, no matter how many children that parent has. If you do not inherit the HD gene, you cannot get the disease and you cannot pass it on to your children or their descendants. If you do get the HD gene, you will eventually get the disease. HD always manifests itself if you live long enough. It never skips a generation.
As a genetic disease, HD is referred to as "a disease of families." In many families touched by HD, more than one family member may have HD at the same time. Many relatives are at risk of later developing the disease.
Nearly every member of the family has been a caregiver to a parent, brother,
sister, son, daughter, aunt, uncle, grandparent, or grandchild at one time or
Similarly, this person in your care most likely saw one of his own parents suffer with HD. Just as the winter snow was always deeper and the summer longer through a child's eyes, this horrible disease probably appeared worse from a child's perspective. Feelings of guilt or sadness may be stirred in the person with HD. He may think about how he might have already passed the gene on to his children. Or he may worry that his brothers and sisters will get HD, too.
His family may have struggled to care for him at home for as long as possible.
They are very knowledgeable about his preferences, and have learned how to meet his care needs. They may be very helpful to you. When family members visit him in the nursing home, they may be silently thinking of the day when one of his children may need this same kind of care.
Visits by children, brothers, and sisters can be as difficult for them as they are for
the person with HD. For at-risk relatives, each visit can be another confrontation
with the disease they might get them-selves.
Although people can first exhibit signs of HD at any age, most people first show them when the are in their 30s and 40s. Thus, HD is described as an "adult-onset disease." In the prime of his life, he may discover that he has HD, that each of his children may have inherited the gene, and, if so, will eventually develop the disease. More than likely, he had settled on a career
and was, in many ways, a well-adjusted adult. These achievements will now have to be given up, one after another, along with his plans and dreams, as HD runs its destructive course.
As a progressive disease, HD begins very subtly and only the person with HD,
close friends or relatives, and the trained eye of a physician can detect its earliest signs. It progresses in stages, slowly advancing for many years.
It usually takes at least 15 years for the disease to run its course, sometimes longer. Particularly during the last half of those years, the affected person will need help with household chores and personal care.
As a caregiver, you can help by antici-pating changes in function that may trigger new concerns and preparing in advance for each new set of challenges that you and the person for whom you are caring will encounter.
There is also a juvenile form of HD. Occurring in about one in ten people
with HD, it looks different from the adult version. Usually the person is stiff,
rigid and slow, may have involuntary movements that look more like tremors, and may experience seizures. Juvenile HD worsens more rapidly than adult-onset
What Advanced-Stage HD Looks Like
Health care professionals look at HD as a disease made up of three disorders
-a movement disorder, a cognitive disorder, and an emotional disorder. Some
people with HD have a very severe movement disorder but very little
Others have profound cognitive changes but few movement problems. The emotional disorder is often depression, which comes and goes throughout the course of the disease.
We'll look at each one of the "trio of
disorders" in the following pages.
What Advanced-Stage HD Looks Like continued
Every person experiences the beginning of HD in a unique way. Some first notice
small subtle movements. Others find themselves becoming forgetful, and still
others become depressed. Every person experiences the progression of HD in
a unique way, too. For example, one might have a rapid deterioration in cognitive
function and less decline in the control of movements.
Another person may have rapidly increasing difficulty with movements, but no significant change in cognitive functioning during the same period of time.
Therefore, no two people with HD will present the same caregiving challenges. But in the most advanced stages, all symptoms converge in a predictable manner.
By the time a person with adult-onset HD comes to a long-term care facility, the movement disorder is usually quite apparent. But some years before, it began with small changes in eye movement and involuntary movements of the fingers and face.
The symptoms progress to a point where all muscles are affected, and walking takes on a "dance-like" quality. People often try
to camouflage and control the movements. As the involuntary movements, often referred to as chorea, become more exaggerated, what was once "dance-like" now looks "drunk-like". Speech becomes affected as words are slurred.
As balance deteriorates, falling occurs more often, and the affected person becomes unable to walk safely without assistance. Beds may need padded side rails to prevent the patient from bruising himself or falling out.
At the same time, it becomes increasingly difficult for the person with HD to speak and be understood. Nearly all people develop a swallowing disorder, need a special diet, and need assistance eating. At some point, a decision will have to be made whether or not to insert a feeding tube. This decision involves the affected person and family members, and it is most helpful if it is made well in advance. In addition, most residents eventually need adapted
beds and wheelchairs to accommodate their severe involuntary movements,
impaired balance, and changes in posture.
The cognitive disorder is less apparent than the movement disorder, but more disabling in many ways. Long before he came to you for care, he struggled with subtle changes that affected his work and
family. Most likely, his ability to organize and plan his work day slowly began to erode, and routine tasks, previously performed effortlessly, became more complicated to complete.
As cognitive function continued to deteriorate, he may have become quite inflexible, wanting things done a certain way. People around him may have noticed these small but significant changes in temperament. Long-term relationships may have been jeopardized. He may have been unable to see changes in himself, and vigorously denied their existence.
Now, in your care, his thinking is slower, initiating action is more difficult, learning new things is not as easy as it once was, and judgement is impaired. He may have developed difficulty waiting for things he wants immediately and become unreason-ably demanding of his family, friends, and caregivers. Now these problems challenge you as you assist him in his daily activities.
The emotional disorder is primarily made up of the depression that runs throughout the course of HD. Having seen his parent suffer with HD, knowing that only further decline and dependence is at hand, and
recognizing all that he's already "lost" to HD, it's easy to see why he might have a reactive depression.
Idiopathic depression, one that is not triggered by life's events, is also common. Some people with HD who are depressed appear irritable or angry.
Some deny depression because they lack insight. Others are unconsciously protecting their feelings. Even in the most advanced stages of HD, people who show classic signs of depression can respond
well to medication.
Suicide in HD occurs more often than in the general population. Depression paired with a lack of impulse control makes
suicide a major risk for patients in all stages of the disease.
There's no typical person with HD. Each individual has complex, unique needs. Some needs can be met easily. Others will require clever or creative solutions. And still others will require an ongoing trial-and-error approach. Taken together, though, you'll become well-versed in this
person's care, and your rewarding days will far outnumber your challenging ones!