Context: Huntington's disease (HD) is a neurodegenerative disorder characterized by mid-life onset, cognitive decline, and behavioral disturbance. Objective: We conducted a review of the end-of-life (EOL) experience of HD patients and their families. Methods: We searched 5 electronic databases. Eligible studies were published in English and contained outcomes related to PC, end-of-life (EOL), advance directives (ADs), symptom management, or hospice use for HD adults. Results: We screened 1566 studies, assessed 244 studies, and included 27 studies. Symptom Prevalence: Decedent data showed greater likelihood of pneumonia, choking, nutritional deficiencies, and skin ulcers. HD patients in hospice experienced pain, anxiety, nausea, and dyspnea. Psychiatric symptoms included dysphoria, agitation, irritability, apathy, and anxiety. Psychosis is associated with worse cognition, function, and behavioral disturbance. Symptom Management: Electroconvulsive therapy (ECT) and venlafaxine improved depression. Suicidal ideation improved with talking, self-management, medication, and discussing EOL wishes. Tetrabenazine improved chorea. Experience as Illness Progressed: HD patients require home care within two years of diagnosis. Only one study reported use of palliative care services (4%). HD patients are admitted to the hospital late in disease course and are often discharged to long-term care facilities (LTCF). Advance Care Planning: Two studies created tools to navigate EOL decisions. Most HD patients had EOL wishes; only familiarity with HD predicted having EOL wishes. Few had ADs or discussed EOL wishes with their families. Clinicians drive EOL discussions. Views on physician-assisted death (PAD) and euthanasia varied widely. Conclusions: Research is needed to further assess the PC needs of HD patients and to provide care recommendations.
Keywords: Huntington's disease; advance directives; end-of-life care; hospice; palliative care; symptom management.