What is Huntington’s disease?

Huntington’s disease diagram

Huntington’s disease is an inherited disorder that causes degeneration of brain cells in the motor control regions of the brain and other areas. Symptoms of the disease, which become progressively worse, include uncontrolled movements, abnormal body postures, and changes in behaviour, 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 the age of 20. More than 30,000 Americans have Huntington’s disease.

How is Huntington’s disease inherited?

A mutation causes Huntington’s disease 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 rare cases, an individual with Huntington’s disease does not have a parent with the disorder.

How is Huntington’s disease diagnosed? Woman suffering from Huntington’s disease

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 the presumed risk of passing Huntington’s disease to a child, it’s advisable to meet with a genetic counsellor. A genetic counsellor will discuss the potential risks of a positive test result, which would also indicate if the parent will develop the disease. 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:

  • The Food and Drug Administration approves Tetrabenazine (Xenazine) to suppress the involuntary jerking and writhing movements (chorea) associated with Huntington’s disease.
  • Antipsychotic drugs, such as haloperidol (Haldol) and chlorpromazine, have the 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 behavioural problems, develop coping strategies, manage expectations during the progression of the disease and facilitate effective communication among family members.

Speech therapy:

Young women with Huntington’s disease

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 using 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, emphasizes mobility technologies specific to the needs of those with Huntington’s disease.

Quantum powerchairs incorporate power-adjustable seating for user repositioning and comfort; speciality 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 powerchairs feature the latest advanced technologies to increase the independence of those with Huntington’s disease. iLevel® seat elevation technology allows users 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. Quantum powerchairs are designed to provide optimal medical comfort and maximum independence for those with Huntington’s disease.

Solution

Quantum Rehab power bases consist of a wide array of models, each incorporating various components to accommodate the comprehensive needs of individuals requiring a complex rehab product. The Quantum Edge 3 with industry-first 4.5 mph at iLevel offers the most advanced power chair experience. Q6 powerchair series, which includes the Edge 3 and Q6 Edge 2 powerchairs, provide highly adjustable mid-wheel drive power bases. The Q6 Edge 2.0 accept the optional iLevel technology, which offers up to 12 inches of lift at 4.5 mph. The 4Front is a quiet, more responsive front-wheel drive power chair that features automotive-grade suspension with unprecedented comfort and rides quality.

Some of our related products