Academic journal article Care Management Journals

Huntington's Disease: A Caring Approach to the End of Life

Academic journal article Care Management Journals

Huntington's Disease: A Caring Approach to the End of Life

Article excerpt

This article describes the challenges of end-of-life care encountered in a specialized long-term care program for people with Huntington's disease (HD). The Promoting Excellence in End-of-Life Care Huntington's Disease Workgroup defines the initiation of palliative care as the point at which independent living is no longer possible. Mobility and lifestyle accommodations for people in the nursing home setting with an early-onset disease are a major feature of this program. The primary end-of- life considerations are advance directives decision-making and anticipating end-stage care needs. Disease progression, denial, family conflict, and clinician blind-spots may impede the development of timely advance directives. The unpredictable and idiosyncratic nature of disease progression impacts decision making for end-of-life care settings and approaches: hospitalization, nursing home stay, and in-house hospice care are the available options. The Workgroup has delineated several priority areas for patient care in HD: autonomy; dignity; meaningful social interaction; communication; comfort; safety and order; spirituality; enjoyment, entertainment, and well-being ; nutrition; and functional competence. This review also includes a description of the program features in each of these areas.

Keywords: Huntington's disease; advance directives; long-term care; chronic disease

Huntington's disease (HD) is a neurodegenerative disorder. The disease is genetic-each child of a gene carrier parent has a 50% chance of inheriting the fatal mutation and developing the illness. HD involves psychiatric imbalance, motor dysfunction, and cognitive impairment. The usual course spans 17 to 20 years (Myers, 2005 ). During the course of illness, functionality erodes significantly, and the impact on the patient and his or her family is often traumatic. People affected with HD lose the capacity to work outside of the home, to parent actively and effectively, and to perform activities of independent living such as driving and shopping. Eventually, most families consider transfer from the home environment to an institutional community in order to ensure the necessary 24-hour supervision and care support for issues such as mobility, safety, nutrition, and emotional wellbeing.

Terence Cardinal Cooke Health Care Center (TCC) has been caring for Huntington's disease patients since 1988. As with several other disease-specific programs, TCC has designated a specialized unit for 48 people with HD. There is no care requirement minimum; any patient presenting with symptoms and situational need is considered for admission. This article describes the Huntington's disease program and some of the end-of-life caregiving challenges in TCC's 19-year history.


The Huntington's Disease Peer Workgroup, sponsored by the Robert Wood Johnson Foundation, establishes the guidelines for palliative care in HD. Palliative care begins when the patient is no longer able to live independently-well before the end of life. A multidisciplinary team can accommodate the family and patients as health status changes. Continuity of care, effective communication, crisis intervention, and symptom management ("Promoting Excellence," 2001) are priorities in the effort to buffer the traumas associated with diagnosis and functional loss.

The Terence Cardinal Cooke facility has a large and diverse patient population. The 729-bed facility is a home for individuals with end-stage renal disease, AIDS, developmental disabilities, pulmonary diseases, Alzheimer's dementia, and other geriatric conditions. HD residents are encouraged to socialize and participate in recreational activities throughout the entire long-term care community, wherever appropriate. Several HD patients develop offunit friendships and relationships, some of which are great sources of rejuvenation; however, when the motives for the relationship are unclear or are essentially transactional, these social ties can be a cause for concern for TCC staff. …

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