How can you help? You can help the Oklahoma Chapter of the Huntington's Disease Society by clicking on the Donate/Membership link to your left. The national chapter can be found at www.hdsa.org.

 

 


Introduction to Huntington's Disease

Huntington's Disease is best known for crippling native Oklahoman, folksinger, and songwriter Woody Guthrie and causing complications that led to his death. It is an inherited progressively degenerative disorder of the nervous system characterized by involuntary movements (chorea) and the loss of cognitive ability (dementia).

It affects over 30,000 Americans and over another 150,000 are at risk. These numbers do not reflect the hundreds of thousands around the world who are also affected or at risk.

Over a period of 10 to 25 years, the ability to think, speak, and walk is greatly diminished. Treatment of the disease is symptomatic and supportive, however, there is no cure.

Early symptoms include involuntary movements of the body and limbs, marked personality changes -- depression, euphoria, and increased forrgetfulness. As the disease progresses, speech becomes slurred, swallowing difficult, and gait unsteady (leading to falls and injuries). Reasoning and judgment becomes impaired. Eventually, there is death.

Each child of a parent with HD has a 50/50 chance of inheriting the disease. Children as young as 2 and adults as old as 80 have developed symptoms of the disease, however, symptoms usually appear between the ages of 30 and 50, after a person has married and had children and perhaps passed on the gene. This disease affects members of both sexes as well as all races and ethnic groups.

 

A Compilation of the Stages of HD

 Early/ Early Intermediate Stages

Stated in positive terms from the person's point-of-view.

Defiance

"I'm not denying the diagnosis, I'm defying the verdict! I'm not refusing to accept it, I'm just boldly resisting the inevitable!"

Perseverance

"I'm continuing on...in spite of all the difficulties this damn disease puts in front of me."

 

Early Stages

 

Early in the disease, manifestations include subtle changes in coordination, perhaps some involuntary movements, difficult thinking through problems, and often, a depressed or irritable mood.

At this stage, medications are often effective in treating depression and other emotional symptoms. It is a good time to begin planning for the future. Financial plans should be made and legal documents drawn up.

Early Stage

 

The person is diagnosed as having HD and can function fully both at home and work.

 

In addition to the physical symptoms of HD, there are often subtle cognitive signs as well.

 

Initial stages

 

People with early Huntington's may find they have difficulty organizing routine matters or coping effectively with new situations. Difficulty recalling information may make them appear forgetful.

Work activities may become more time-consuming, and decision-making and attention to detail may be impaired.


Early emotional symptoms may be equally subtle. Individuals at this stage may experience more periods of depression, apathy, irritability, or impulsiveness, or perhaps changes in personality.


At this stage, people with Huntington's can function quite well at work and home.

Early Intermediate Stage

 

The person remains employable but at a lower capacity. They are still able to manage their daily affairs despite some difficulties.

 

In addition to the physical symptoms of HD, there are often subtle cognitive signs as well.

 

Intermediate stages


As the disease progresses, the symptoms become worse. The initial motor symptoms will gradually develop into more obvious involuntary movements such as jerking and twitching of the head, neck, arms and legs. These movements may interfere with walking, speaking and swallowing.


People at this stage of Huntington's often look as if they're drunk: they stagger when they walk and their speech is slurred.

 

They have increasing difficulty working or managing a household, but can still deal with most activities of daily living.

 

Late Intermediate Stage

Stated in positive terms from the person's point-of-view.

 

Compassion

"I'm sorry for the trouble I'm causing my family and everyone else who cares for me.

I wish I could do something to help them."

Intermediate stages


As the disease progresses, the symptoms become worse. The initial motor symptoms will gradually develop into more obvious involuntary movements such as jerking and twitching of the head, neck, arms and legs. These movements may interfere with walking, speaking and swallowing.


People at this stage of Huntington's often look as if they're drunk: they stagger when they walk and their speech is slurred. They have increasing difficulty working or managing a household, but can still deal with most activities of daily living.

Late Intermediate Stage

 

The person can no longer work and/or manage household responsibilities. They need considerable help or supervision to handle daily financial affairs. Other daily activities may be slightly difficult but usually only require minor help.

Middle Stages

 

In the middle stage involuntary movements (chorea) may become more pronounced. A staggering gait can sometimes be mistaken for drunkenness.

Speech and swallowing will begin to be affected. It is important to consult a speech therapist who will be able to offer suggestions and strategies for improving communication and swallowing abilities. Likewise, occupational and physical therapists can develop programs to help maintain the highest level of functioning and thereby improve quality of life.

 

Thinking and reasoning skills will also gradually diminish. At this stage it may become increasingly difficult to hold a job and to carry out household responsibilities.

 

Here again, simple strategies may be employed to help decrease frustration, increase functioning and prolong independence. For example, disorientation and short-term memory loss can be addressed by labeling drawers, maintaining a daily routine and posting a calendar appointments and events.

 

Early Advanced Stage

Stated in positive terms from the person's point-of-view.

 

Stamina

"I'm not sure just what it is but something keeps me going! It keeps me going through all my fatigue and all the problems and hardship this damn disease presents me."

Early Advanced Stage

 

The person is no longer independent in daily activities but is still able to live at home supported by their family or professional carers.

 

Advanced Stage

Stated in positive terms from the person's point-of-view.

 

Grace

"I've quietly resigned myself to needing others to care for me, to sustain me. I can't show them, but I'm more concerned for the welfare of those around me than I am for myself. We know we're there for each other."

Advanced Stage

 

The advanced stages of Huntington's typically involve fewer involuntary movements and experience more rigidity. People in this stage of HD can no longer manage the activities of daily living, and they usually require professional nursing care.

 

Difficulties with swallowing, communication and weight loss are common.

Choking on food becomes a major concern, as does the weight loss. At this stage people with HD are totally dependent on others for all aspects of care, they can no longer walk, and not able to communicate their needs.

Although cognitive abilities are severely impaired, it is important to remember that the person is generally still aware of his/her environment, remains able to comprehend language, and remains an awareness of loved ones and others. He/she may continue to enjoy looking at photographs and hearing stories of family and friends.

People do not die from HD itself but rather from a complication of the disease, such as choking, pneumonia, heart failure or infection developing from the body's weakened condition. Death generally occurs about 15 to 20 years after onset.