WHAT IS HUNTINGTON’S DISEASE?
Huntington’s disease (HD), also called Huntington’s chorea, is a rare, fatal disease that causes nerve cells in the brain to slowly waste away. The loss of nerve cells causes chorea. Chorea is the medical word for uncontrolled jerky movements of the face, arms, neck, and trunk. Huntington’s disease also causes dementia. Dementia is a gradual loss of mental function.
HOW DOES IT OCCUR?
The disease is passed from parent to child by a faulty gene. If one parent has this gene, his or her children have a 50% chance of inheriting the gene and having the disease.
WHAT ARE THE SYMPTOMS?
The disease develops slowly. Sometimes it is hard to recognize. Symptoms usually affect movement, speech, personality, and mental function.
Symptoms related to movement and speech may include:
· repeated, irregular movement of eyebrows and forehead
· facial grimaces
· rigid muscles
· loss of balance
· trouble walking
· uncontrolled jerking movements of arms, legs, and trunk
· halting speech
Symptoms related to personality and mental function may include:
· having hallucinations (seeing or hearing things that are not really there)
· having delusions (false beliefs or ideas)
· being suspicious without cause
· neglecting personal appearance and hygiene–for example, failing to change clothes or bathe
· neglecting duties, such as not paying bills or going to work
· being depressed
· being irritable
· behaving irresponsibly, impulsively, or violently, such as driving recklessly, getting drunk, or starting fights
· losing the ability to remember, think logically, or exercise judgment
· not knowing who or where you are or what time, date, or day it is
HOW IS IT DIAGNOSED?
Your healthcare provider will examine you and ask about your symptoms and medical and family history. You will have a blood test to check for the gene that causes the disease.
HOW IS IT TREATED?
There are no medicines that can stop or reverse the symptoms of Huntington’s. Some medicines, however, can help control symptoms. For example, tetrabenazine (Nitoman) is used to help control the writhing and flailing movements that HD causes.
Other medicines can be used to help control behavior symptoms. Violent outbursts, mood swings, or depression can often be lessened or controlled with medicine.
HOW LONG WILL THE EFFECTS LAST?
The symptoms usually start between the ages of 35 and 50 and get worse over time. Most people live for about 15 years after they start having symptoms, but some live up to 30 years later. If symptoms start at a young age, the disease gets worse more quickly. As the disease gets worse you will likely need help with what are called the activities of daily living, or ADLs. Examples of ADLs are eating, dressing, and using the bathroom. Family members can try to do as much as they can, but it can be helpful to have personal attendants to help with your daily tasks. There may come a time when you may need 24-hour care in a nursing facility.
HOW CAN I HELP TAKE CARE OF MYSELF?
You want to do what you can to live as independently as possible. Occupational therapists can help you learn ways you can stay physically independent. You need to stay as strong as you can, so exercise is an important part of taking care of yourself. Your therapists will help you create an exercise program.
Also important is finding a counselor or mental health therapist who can help you sort out the usual ups and downs of life with Huntington’s disease. They can also help you with the depression.
Work closely with your healthcare provider to find the medicines that are most helpful for your movement problems and emotional problems.
Join a Huntington’s support group and encourage your family members to join also. Supportive personal relationships can develop in these groups. They may also be the best way to learn helpful tips for making everyday life easier. You can also learn about community resources for people with Huntington’s and their families.
HOW CAN I PREVENT HUNTINGTON’S DISEASE?
Because Huntington’s is a genetic disease, genetic counseling is strongly recommended for all family members. The counselor will discuss the pros and cons of being tested for the gene that causes HD. People who have the gene may pass the disease on to their children. Being tested for the gene is a very personal decision. The genetic counselor may also be able to tell you about the latest research on ways to prevent the illness in future generations.
For more information, contact:
The Huntington’s Society of Canada