Feb. 17, 2022 | Huntington’s disease is a progressive, degenerative disease that causes certain nerve cells in the brain to waste away. Named for Dr. George Huntington, who first described this hereditary disorder in 1872, Huntington’s disease, or HD, is now recognized as one of the more common genetic disorders, with more than a quarter of a million Americans having the disease or at risk of inheriting it from an affected parent. Every child of a parent with HD has a 50-50 chance of inheriting the gene that causes the disease, which affects as many people as cystic fibrosis and muscular dystrophy. If a child does not inherit the gene, he or she cannot pass it on. If the child does inherit the gene, he or she can pass it on and will develop the disease if that child lives long enough. In 1993, the HD gene was isolated and a genetic test was developed which can determine whether a person carries the gene although it cannot predict when symptoms will begin.

Feb. 17, 2022 | Huntington’s disease is an inherited disease. While its symptoms usually develop in middle age, younger people with Huntington’s often have a more severe case, and their symptoms may progress more quickly. The signs and symptoms of HD can vary significantly from person to person. The neurological disease usually develops slowly, and the severity of signs and symptoms is related to the degree of nerve cell loss. Death occurs about 10 to 30 years after the symptoms first appear although the disease may progress faster in younger people. Early signs of Huntington’s often include decreased cognitive abilities, such as difficulty making decisions and remembering important information. Sudden jerky movements throughout the body and swallowing problems may appear later on. Young people who develop HD may have symptoms that mimic Parkinson’s disease, including muscle rigidity, tremors and slow movements.

Feb. 17, 2022 | Depression is the most common psychiatric disorder associated with Huntington’s disease. The depression is not a reaction to receiving a diagnosis of the disease but rather the result of an injury to the brain and subsequent changes in brain function. Some of the symptoms of depression may include feelings of irritability, social withdrawal and loss of energy. Weight loss is also common in people with Huntington’s, especially as the disease progresses. The depression associated with Huntington’s may increase the risk of suicide. Some research suggests that the greater risk of suicide occurs before a diagnosis is made and in middle stages of the disease when a person has begun to lose independence. Anyone displaying any of these signs should see a physician, who will review the patient’s symptoms and medical history. A referral to a neurologist will be necessary to properly diagnose a potential neurological disorder.

Feb. 17, 2022 | Currently, there is no way to stop or reverse the course of Huntington’s disease, no treatment to halt its progression, which leads to death after ten to 25 years. Some approaches can control signs and symptoms, but HD eventually causes physical and mental disability. As the disease progresses, long-term nursing home care may be necessary. Tetrabenazine is prescribed for treating Huntington’s-associated chorea. It is the only drug approved by the U.S. Food and Drug Administration specifically for use against HD. Antipsychotic drugs may help to alleviate chorea and may also be used to help control hallucinations, delusions, and violent outbursts. Drugs may be prescribed to treat depression and anxiety. Drugs used to treat the symptoms of HD may have side effects such as fatigue, decreased concentration, restlessness, or hyperexcitability, and should be only used when symptoms create problems for the individual.

Feb. 17, 2022 | Most people with Huntington’s disease do not die as a direct result of the disease but rather from medical problems that arise from its effects on the body, such as infections, choking and pneumonia. Because Huntington’s disease affects a person’s cognitive abilities, drawing up a will and a living will early in the course of the disease is recommended. Most people with Huntington’s disease eventually need assistance in performing daily functions. In these cases, group housing can provide a comfortable living situation that allows the patient to maintain their independence. Early discussions about this type of care enable the patient to communicate his or her preferences. Managing Huntington’s is demanding on the person with the disorder, family members and caregivers. As the disease progresses, the person will become more dependent on caregivers. A number of issues will need to be addressed, and strategies to cope with them will evolve.