Annals of Long Term Care

Located in suburban San Diego, Edgemoor, a distinct part of the San Diego County Psychiatric Hospital, has become a referral site for patients with the neurological condition Huntington’s disease (HD). Best known for its choretic movements and its affliction of folk singer Woody Guthrie, HD is a hereditary and relentless disease that usually begins in persons at middle age and results in progressive decline over approximately 20 years.1 Staff at the University of California San Diego (UCSD) Huntington’s Disease care program, designated a Center of Excellence by the Huntington’s Disease Society of America since 2000, refers patients with late-stage HD to Edgemoor. Edgemoor staff has cared for generations of patients from the same families.

Although the patient with HD is initially treated for many years in an outpatient setting, as the disease progresses and family resources wane, institutionalization is not unusual. The duration of the disease often exhausts insurance benefits and personal resources, and limits the options for care settings. Additionally, escalating behavioral problems and care needs will strain family or other caregivers physically and emotionally, creating the need for placement in a facility. Caught in this conflict, families choosing to place the patient in a long-term care setting may face difficulty finding a facility and staff that can manage their family member.2

In the period of 2000 to 2008, Edgemoor cared for 53 patients with late-stage HD, ranging in age from 29 to 78 years at time of death. Patients who have died or were discharged averaged 48 years of age at admission and stayed an average of 11 years. Table I summarizes demographic characteristics of patients with HD at Edgemoor. As understanding of this disease process has grown, Edgemoor staff has sharpened its capability to anticipate the needs of the patient with HD. This experience allows for more successful work with these patients via person-centered care strategies, consistent assignments, individualization, risk-sharing, and with maintenance of autonomy as a primary goal.

Etiology of Huntington’s Disease

HD is an autosomal dominant disease with both physical and behavioral manifestations (Table II).3-5 The gene for HD is a mutated autosomal dominant gene located on the short arm of chromosome 4 and is associated with an expanded trinucleotide repeat.1 Children of parents with HD have a 50% risk of developing the disease.6 Defining characteristics of HD include abnormal movements, progressive decline in cognitive and physical abilities, and serious psychiatric-behavioral disturbances. The cognitive decline associated with HD has been classified as a subcortical dementia characterized by slowness of mental processing, forgetfulness, impaired cognition, impulsivity, impairment in affective regulation, apathy, and depression, as opposed to the traits associated with Alzheimer’s dementia,1 such as progressive deterioration of memory, aphasia, apraxia, and agnosia.

The course of HD has been divided into three stages, each estimated to last about 5 years (Table II3-5). Each patient’s disease progression is unique. Without exception, the disease eventually progresses into a late-stage pattern resembling that of Alzheimer’s disease. Patients have aphasia, dysphasia, dementia, and require complete physical care before death. Patients are institutionalized in Stage 2 or 3/4.7,8

Concerns and Care Strategies

Planning and preparation are essential for a facility prior to accepting a patient with HD. Such patients may be high risk for a facility. The decision to admit a younger, ambulatory, and potentially violent patient for what is likely a lifetime of care should not be made without assuring that the appropriate resources to handle him or her are available. Edgemoor staggers these admissions to allow time for assessment and adjustment of patients with HD.