The word chorea is a Greek word that means dance. This refers to a characteristic of the disease which includes quick, incessant, jerky and involuntary movement
Today one in 10,000 Americans have Huntington’s disease and there are approximately 30,000 known cases in the United States.
Huntington’s disease is an inherited brain disease which usually develops in middle aged men and women alike, affecting both mind and body. It has been known to affect younger people and children; it is however very rare and when it does the outcome and symptoms can be very severe.
If one parent carries the genetic gene that causes Huntington’s there is a fifty percent chance of the children inheriting the disease.
Should this be the case it is a good idea to consider genetic counseling and testing before starting a family to determine the presence of the faulty gene. If there is a risk of passing on this genetic disease, adoption or forms of assisted reproduction may be an option. There is a procedure called “Vitro fertilization with pre-implantation”. This is a screening of the embryo for the Huntington’s disease gene. If a child does not inherit the faulty gene he will not develop Huntington’s and cannot pass it on to the next generation. Anyone who does inherit the gene will eventually develop the disease.
Signs and symptoms:
- Quick, incessant jerky and involuntary movement.
- Instability in both walking and moving around.
- Difficulty in swallowing, eating and speaking.
- Lack of coordination.
- Unable to perform daily functions.
- Depression.
Treatments:
- Some treatments can control the signs and symptoms at present; however there is no treatment available to stop or reverse the disease.
- Eventually people with Huntington’s disease will become both physically and mentally disabled and as the disease progresses, long term nursing care may be required.
- Since Huntington’s disease can impair speech and the ability to express complex thoughts, speech therapy may be an option.
Medications:
Medications are used to control movements, violent outbursts and hallucinations. Tranquilizers are also used to control depression, other obsessive-compulsion rituals, extreme emotions and mood swings.
- Prozac
- Sarafens
- Zoloft
- Lilhium
Like all medications, drugs used to treat Huntington’s do have side effects. They are:
- Fatigue, restlessness or they may make the patient hyper and excitable.
- Other antipsychotic drugs may mimic Parkinson’s disease symptoms such as involuntary twitching of both the face and body.
Self care:
Like most chronic illnesses people who exercise regularly tend to fare better than those who don’t. Physical activity and adequate exercise can help both physically and mentally.
Since the Huntington’s patient may in all probability be unsteady when moving around it is important to keep the home free of hard and sharp objects. The wearing of properly fitted shoes and padding would protect oneself while out walking.
Nutrition
Due to the quick involuntary movement’s people with Huntington’s can burn as many as five thousand calories per day; it is therefore important for them to get adequate nutrition to maintain body weight including the use of supplements and vitamins.
As the disease progresses assistance with eating may be required by cutting the food into small pieces or pureeing it. This is helpful in making the swallowing easier and prevents choking.
Cutting back on dairy products may also be required since they may cause excess mucus increasing the risk of choking.
There are also special eating utensils designed to assist people with disabilities in feeding themselves: they have suction on the bottom to help prevent spills, bendable straws making it easier for them to drink. Since Huntington patients are vulnerable to dehydration it is important that they drink plenty of liquids especially in warmer weather.
Once the signs and symptoms start they continue until the person is deceased. Signs do vary from person to person; over time vital functions such as swallowing, eating, speaking and walking usually deteriorate. Death however, usually occurs as a result of complications from the disease such as a fall or infection.
For more information: www.hdsa.org, www.huntingtonsociety.org, www.ahansw.ash, www.hda.org.uk
