Huntington's Disease and Dementia

The Challenges of Home Care

Huntington's disease is a progressive, and inherited, neurological disease that breaks down nerve cells in the brain, leading to a condition commonly referred to as Huntington's Dementia.

The disease is characterized by uncontrollable jerking muscle movements called Huntington's Chorea. It was first identified in 1872 by Dr. George Huntington. The gene responsible for this disorder was discovered in 1993, allowing people to be tested earlier in life and before child bearing age, allowing people to make the choice as to whether to have children or not if they carry the defective gene.

About 1 in 10,000 people have Huntington's Disease, but the statistics may be much higher due to the fact that it often goes undiagnosed in third world countries.

After the initial onset of symptoms, the disease process lasts anywhere from 5 - 20 years and is always fatal. Symptoms typically begin between the ages of 30 - 45 and the younger the symptoms occur, the faster the disease seems to progress.

The Cause of Huntington's Disease

The cause is a defect in one single gene. It is considered an autosomal dominant disorder. What that means is that an individual needs just one copy of the defective gene to get the disease. Each child receives two copies of each gene, one from each parent (except for the sex genes). A parent with a defective copy of the gene that causes Huntington's disease could either pass on the healthy gene, or the defective gene.

Therefore, every child of a parent with this disease has a 50/50 risk of inheriting the defective gene.

With the discovery of the specific gene, people can now get tested and make the very personal decision as to whether to have biological children or not.

In recent years, amniocentesis has been utilized to screen for this disease early in pregnancy, giving the parents the option to terminate the pregnancy if this is their choice.

Due to the absolutely devastating process of Huntington's Disease, the option to terminate must be left to the parents alone, and not judged by outsiders.

Common Symptoms of Huntington's Disease

The disease causes movement, cognitive and psychiatric disorders. The rate of progression and symptomology varies greatly among people with this disorder. Some may have all symptoms, some just a few, movement disorders may dominate or psychiatric disorders may dominate.

As the progressive damage continues in the brain, most people will suffer from depression and all will eventually develop dementia and the associated dementia symptoms.

Due to the motor function decline, most people are institutionalized in order to have 24 hour care.

Other symptoms common in this disease include:

• Muscle Rigidity
• Jerking or writhing movements (Huntington's Chorea)
• Swallowing difficulties, which can lead to repeated pneumonias
• Psychiatric Disorders, most commonly depression, but also obsessive-compulsive disorder, bi-polar and mania disorders
• Physical disorders of speech
• Lack of awareness of behavior
• Lack of impulse control

There are other, multiple symptoms that can occur with this disease process.

A good reference if you or a loved one has been diagnosed with Huntington's Chorea is The Mayo Clinic which provides up to date information on all aspects of the disease.

The type of dementia that occurs with Huntington's Disease is considered subcortical, with the prevailing symptoms involving motor changes, frequent falls, and mood and personality disorders.

With no known cure and little effectiveness with current treatments, the diagnosis can be devastating for the person diagnosed, children and family and friends.

Home care should focus on injury prevention, including preventing falls and protecting the airway. Thickened liquids are generally necessary as the risk of aspiration is so high. Bed alarms and chair alarms can be effective for fall prevention.

As the disease progresses, interventions such as a helmet, wheelchair seat belt and padded rooms become necessary in most cases.

The other main concern is protecting the care givers. Accidental injuries occur more often than people may think, and due to the patient's lack of understand as to their abilities, caring for them can be extremely difficult.

If you find yourself in this position, you may want to consider either full time help in your home or placement in an extended care facility. The safety of both the person with the disease and the family must be the top priority. In later stages, a person with Huntington's Disease must never be left alone.

To compare nursing homes in your area, go to Medicare.gov.

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