Promoting Excellence : Huntington's Disease Recommendations to the Field : Huntington's Disease and Palliative Care

The HD Peer Workgroup chose to define the beginning of the end of life in HD as the point at which the person is no longer able to live independently. Palliative care is defined as interdisciplinary team-based care, for people with life-threatening illness or injury, which addresses physical, emotional, social and spiritual needs and seeks to improve quality of life for the ill person and his or her family. Patients need palliative care well before they are near the end of life. In fact, the World Health Organization defines the beginning of the need for palliative care for people with cancer as the time of diagnosis. Demonstration projects and pilot studies funded by The Robert Wood Johnson Foundation showed that early introduction of palliative care to people with incurable diseases can enhance the quality of life throughout illness while reducing hospital admissions, allowing more patients to be cared for at home and die at home.[10] Preliminary findings indicate that care can be improved while diminishing the overall medical expense.

In a similar fashion, the need for palliative care in Huntington's Disease begins when a diagnosis of HD is first considered. Because a patient's ability to communicate diminishes over the course of the disease, it is at the beginning that the need for information about HD is the greatest. The future can be discussed and plans made for later in the course of the illness when the need for palliative care intensifies. A palliative care health worker can then coordinate care according to the patient's preferences, and stand ready to handle problems before crises arise. A team of clinicians can work to improve care and quality of life, emphasizing continuity of care, communication, symptom management, crisis prevention and early crisis management. Early introduction of palliative care, a seamless provision of services of gradually increasing intensity, is imperative.

The progression of HD is divided into five stages defined by the patient's score on the Total Functional Capacity (TFC) Scale, which details level of function in the domains of workplace, finances, domestic chores, activities of daily living and requirements for unskilled or skilled care.[11,12] The interdisciplinary palliative care team assists patients and their families in articulating their wishes and the goals of care, and developing a plan to achieve those goals. When a person with HD is no longer able to live independently, palliative care turns toward adaptation to advanced illness and disability and issues of life completion and closure, while continuing to provide comfort and support for the person with HD and his or her family.

Total Functional Capacity (TFC) Scale
Engagement in occupation Capacity to handle financial affairs Capacity to manage domestic responsibilities Capacity to perform activities of daily living Care can be provided atů
Stage I (TFC 11-13) (0-8 years) Usual level Full Full Full Home
Stage II (TFC 7-10) (3-13 y) Lower level Requires slight assistance Full Full Home
Stage III (TFC 3-6) (5-16 y) Marginal Requires major assistance Impaired Mildly impaired Home
Stage IV (TFC 1-2) (9-21 y) Unable Unable Unable Moderately impaired Home or extended care facility
Stage V (TFC 0) (11-26 y) Unable Unable Unable Severely impaired Total care facility only

No cure is presently available for Huntington's Disease. Researchers, however, have made great strides in understanding the basic mechanisms causing HD that may lead to potential treatments, through studies funded publicly and through HDSA. The Huntington Study Group (www.Huntington-study-group.org), as well as other individuals and groups, have conducted clinical studies aimed at reducing disease progression and the impact of symptoms. Those involved in research and in care hope that a treatment and cure for HD are developed in the near future. In the interim, the goal must be to provide the best possible care for people with HD and their families. National organizations such as the Huntington's Disease Society of America (www.HDSA.org) and the Huntington Society of Canada (www.HSC-ca.org) are invaluable in providing education and support to those with HD as well as funds for care and research. Health care alone cannot provide the necessary support to HD patients and families. Integrated care and connections that utilize resources from the entire community provide the best care.

The burden of caregiving and the process of seeking out community resources to ensure quality care as the disease progresses must not fall solely on HD family members. Non-traditional approaches that integrate attention to personal care and comfort in addition to curative treatment and efforts to modify the course of the disease can improve overall quality of life for HD patients and their families.

" Less attention...is paid to caregiver grief, that relentless ongoing process that is brought about, not by a loved one's death, but by the changed aspects of their life, and inevitably of our own." --A Caregiver

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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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