Family finds new hope in battling rare disease

(ARA) - Matt and Karen Douglas had only been married for a few years when Matt started exhibiting early symptoms of Huntington’s disease (HD). His symptoms started with small, uncontrolled movements, such as his hands twitching when he didn’t want them to; but the movements soon worsened and became so severe that Matt was unable to drive his car.

HD is an inherited neurodegenerative disease that over time results in uncontrolled movements, emotional changes and mental deterioration. In the United States, about 25,000 people have HD. Each child of a parent with the disease has a 50 percent chance of inheriting the gene and ultimately developing the disease. This means at least 150,000 others are at risk of developing the disease, along with thousands of their relatives.

The most common, and perhaps most devastating symptom of HD is chorea. Chorea is characterized by jerky, involuntary movements throughout the body, often appearing as writhing, twisting and turning in a constant, uncontrollable dance-like motion. As chorea progresses, the jerk-like movements worsen and make it difficult for patients to carry out many activities associated with daily living.

“Patients can lose the ability to button a shirt, drink a glass of water, or even sit in a chair without falling off,” says Dr. Amy Colcher, a neurologist at Pennsylvania Hospital. “As the disease progresses, the danger for falls and other injuries increases greatly.”

Currently, there is no cure for HD and the disease is ultimately fatal. However, in 2008, the U.S. Food and Drug Administration approved Xenazine (tetrabenazine) to treat chorea associated with Huntington’s disease, making this the first product ever approved for a symptom of HD. “Xenazine is not a cure for HD, but as a physician, I am grateful for this medical milestone and that we now have an option to potentially help patients better manage the uncontrolled movements they experience as a result of the disease,” says Colcher.

Serious side effects reported with the use of Xenazine include depression and suicidal thoughts and actions. Xenazine should not be used in patients who are actively suicidal or in patients with untreated depression. Concerns about the risk of suicide are heightened in all patients with HD.

For Matt and Karen Douglas from Delaware, Xenazine offers new hope in managing his chorea associated with HD.

“Before Xenazine, chorea used to limit Matt’s life in so many ways,” says Karen. “Since Matt started on Xenazine as part of a clinical trial almost four years ago, we have been able to help control his movements. He will continue experiencing chorea, but every bit of his independence helps extend our quality of life as a family.”

“The availability of Xenazine in the United States represents a significant development for the patients, families and caregivers affected by HD,” says Louise Vetter, chief executive officer of the Huntington’s Disease Society of America. “HDSA and the scientific community continue to work towards a cure for HD, and while we do, we are working to educate as many people as possible about the disease, which is why we have an extensive network of local chapters across the country that are working tirelessly to raise awareness for this devastating disease and the exciting advancements that are being made to help manage it.”

If you are interested in learning more about HD, its symptoms, and local awareness events in your community, visit www.hdsa.org. For more than 40 years, HDSA has been supporting patients with HD and their family members by providing vital support, information and educational services to improve the lives of those affected by HD. For full Prescribing Information including Boxed Warning for Xenazine, visit www.xenazineusa.com.

Courtesy of ARAcontent