What is Huntington’s Disease?

Huntington's disease is an inherited disorder that causes degeneration of brain cells in the motor control regions of the brain, as well as other areas. Symptoms of the disease, which become progressively worse, include uncontrolled movements, abnormal body postures, and changes in behavior, emotion, judgment, and cognition. Huntington’s disease also causes impaired coordination, slurred speech, and difficulty feeding and swallowing. Symptoms typically begin between the ages of 30 and 50. A juvenile form occurs under age 20. More than 30,000 Americans have Huntington’s disease.

How is Huntington’s Disease Inherited?

Huntington’s disease is caused by a mutation in the gene for a protein called “huntingtin.” An affected person usually inherits the altered gene from one affected parent. Each child of a parent with Huntington’s disease has a 50-50 chance of inheriting the gene. If a child does not inherit the gene, he or she will not develop the disease and generally cannot pass it to subsequent generations. There is a small risk that someone who has a parent with the mutated gene, but who did not inherit the gene, may pass a possibly harmful genetic sequence to his or her children. A person who inherits the gene will eventually develop the disease. In very rare cases, an individual with Huntington disease does not have a parent with the disorder.

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How is Huntington’s Disease Diagnosed?

A genetic test, coupled with a complete medical history and neurological and laboratory tests, helps physicians diagnose Huntington’s disease. People with a known family history of Huntington's disease are understandably concerned about whether they may pass the Huntington gene on to their children. These people may consider genetic testing and family planning options.

For prospective parents of presumed risk of passing Huntington’s disease to a child, it’s advisable to meet with a genetic counselor. A genetic counselor will discuss the potential risks of a positive test result, which would indicate if the parent will develop the disease, as well. Prenatal testing for the gene is also available.

Treatment for Huntington’s Disease

No treatment can yet stop the progression of Huntington’s disease. However, medications and therapies can help with symptoms.

Medications for physical symptoms:

• Tetrabenazine (Xenazine) is specifically approved by the Food and Drug Administration to suppress the involuntary jerking and writhing movements (chorea) associated with Huntington's disease.
• Antipsychotic drugs, such as haloperidol (Haldol) and chlorpromazine, have a side effect of suppressing movements.
• Other medications include amantadine, levetiracetam (Keppra, others) and clonazepam (Klonopin).

Medications for psychiatric symptoms:

• Tetrabenazine (Xenazine) is specifically approved by the Food and Drug Administration to suppress the involuntary jerking and writhing movements (chorea) associated with Huntington's disease.
• Antipsychotic drugs, such as haloperidol (Haldol) and chlorpromazine, have a side effect of suppressing movements.
• Other medications include amantadine, levetiracetam (Keppra, others) and clonazepam (Klonopin).

Mental health practices:

Mental health professionals can provide talk therapy to help a person manage behavioral problems, develop coping strategies, manage expectations during progression of the disease and facilitate effective communication among family members.

Speech therapy:

A speech therapist can help improve the ability to speak clearly or teach the use of communication devices, as well as address difficulties with muscles used in eating and swallowing.

Physical therapy:

A physical therapist can teach appropriate and safe exercises that enhance strength, flexibility, balance and coordination. These exercises can help maintain mobility as long as possible and may reduce the risk of falls.

Occupational therapy:

Occupational therapy assists the person with Huntington's disease, family members and caregivers on the use of assistive devices that improve functional abilities.

Prognosis of Huntington’s Disease

At this time, there’s no cure for Huntington’s disease, with a lifespan of 15 to 20 years from initial symptoms.

Mobility for those with Huntington’s Disease

Many with Huntington’s disease experience the progressive loss of the ability to walk, necessitating the use of a power wheelchair. Quantum Rehab®, the global leader in individualized power chairs, puts an emphasis on mobility technologies specific toward the needs of those with Huntington’s disease.

Quantum Power Chairs® incorporate power-adjustable seating for user repositioning and comfort; specialty drive controls, including using a single finger or one’s head to operate the power chair; and, a highly-adaptable design to meet an individual’s current and future needs.

Quantum Power Chairs feature the latest advanced technologies to increase the independence of those living with Huntington’s disease. iLevel® seat elevation technology allows a user to operate the power chair at seated or standing height. Bluetooth® is also integrated into Quantum’s Q-Logic 3 electronics, so those with Huntington’s disease can operate much of their environment with the power chair drive control, itself.

For those with Huntington’s disease, Quantum Power Chairs are designed to provide optimal medical comfort and maximum independence. Please click here for more information on Quantum Power Chair solutions for those living with Huntington’s disease.

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