Huntington 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 if the family has been a caregiver to a parent, brother, sister, son, daughter, aunt, uncle, grandparent, or grandchild at one time or another.
Similarly, this person in your care must 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 is 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 to his children may need this same kind of care. Visits by children, bothers, 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 themselves.
Although people can first exhibit signs of HD at any age, most people first show them when they 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 anticipating 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 HD.