Promoting Excellence : Huntington's Disease Recommendations to the Field : Introduction

A successful corporate accountant begins uncontrollably fidgeting. He loses motor control, and then he loses his job. Another woman, unable to speak, grieves the death of her son by screaming down a nursing home's halls. A man stumbles down the street and is arrested for intoxication. He isn't drunk. He has Huntington's Disease.

These scenarios are not atypical for the 30,000 people in the United States with Huntington's Disease (HD), an inherited neurodegenerative disorder with onset usually between 30 to 50 years of age, and a disease course spanning two or more decades to death. It involves progressive motor, cognitive and behavioral symptoms from onset until death. No cure exists. The disease, which is often misinterpreted, is underreported and understudied.

Its tragedies, however, are many. Each child of a person with Huntington's Disease is at risk to develop HD. As the disease progresses, a veil drops between people with HD and their friends and family, slowly robbing them of their ability to communicate and prompting, according to one study,* almost 30 percent to attempt suicide at least once, generally in the early stages of the disease.

Perhaps most disturbing is that the disease seems wrought with conditions that are neither costly nor difficult to correct. However, many staff, burned out by patients' demands, fail to understand the special requirements of HD, such as the need for more frequent snacks to combat the weight loss associated with the disease.

A major consequence of the combination of cognitive and behavioral problems and the long course of the disease is that many people with HD have consumed most financial resources by the time they need them most - toward the end of life.

*See Appendix C, Research and Policy Subcommittee Report.

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Promoting Excellence in End-of-Life Care was a national program of the Robert Wood Johnson Foundation dedicated to long-term changes in health care institutions to substantially improve care for dying people and their families. Visit PromotingExcellence.org for more resources.

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