FAST FACT AND CONCEPT #201
Palliative Care for Patients with Huntington’s Disease
Sean Marks MD, Serena Hung MD, and
Background Huntington’s disease (HD) is an incurable neurodegenerative disorder inherited in an autosomal dominant fashion. It is characterized by progressive movement disorders, psychiatric manifestations, behavioral abnormalities, and cognitive impairment. This Fast Fact will focus on supportive and terminal care for patients with HD and their families.
Natural History and Prognosis
· Symptom onset is usually between 33 and 44 years; subtle cognitive and motor changes may precede diagnosis by many years. Mean duration of illness from onset to death is 15-20 years with average age of death of 60 years; there are no proven therapies which slow the progression of HD.
· Patients show signs of progressive dementia and become unable to walk, talk, take in nutrition, and care for themselves. L
· ~1/3 of all patients with HD are institutionalized in long-term care facilities.
Impact on Families HD often begins during a time when family life is most complex and therefore most disruptive to the family structure (e.g. child-rearing, career development). Children can be particularly affected: distress is aggravated by concerns about their own genetic susceptibility, and as many as 40% of children of HD patients describe HD as splitting their family apart. Careful assessments of familial coping and psychosocial needs are an integral part of ongoing care for the HD patient.
Common Symptoms and Supportive Care Patients are best served by an interdisciplinary team familiar with caring for patients with HD.
· Motor Manifestations. Abnormal involuntary movements include: chorea, dystonia, rigidity, bradykinesia, tremor, and myoclonus; other motor manifestations include gait and balance problems leading to frequent falls, slurred speech and swallowing difficulties
o First-line strategies are non-pharmacologic and include gait/balance training, speech therapy, and orthotics and leg weights to assist with upright posture.
o Chorea is the most frequently targeted symptom for pharmacologic therapy. Tetrabenazine (a dopamine depleting agent) has been shown to reduce chorea in a well-designed placebo controlled trial; it is undergoing approval in the
· Psychiatric manifestations are present in over half of HD patients.
o Depression is a significant psychiatric problem and rates of suicide are higher in HD patients than the general population. Case reports support using tricyclic as well as newer antidepressants.
o Agitation is also common, and a small number of patients develop psychosis. Atypical neuroleptics are commonly used. Emotional lability (including episodes of extreme anger) can respond to propranolol.
o Low doses of scheduled benzodiazepines before meals or propranolol are used to control motor manifestations and anxiety related to eating.
o Establishing strict daily and hourly routines can help lessen anxiety, short-term memory deficits, intrusive thoughts, and fear of abandonment.
· Cognitive Deficits: gradual loss of memory and executive function are common. Consequently, increasing impairments in initiating movements and conversation occur. Yes/no questions may be preferable over open-ended questions when cognitive impairments become severe.
Advance Care Planning Advanced care planning should be performed as early as possible, prior to cognitive impairment. Of particular importance is establishing a health care power of attorney, as well as documenting guidance to families for likely decisions they will face (such as tube feeding and mechanical ventilation). Some states require clear evidence that a patient would want tube feeding withheld or withdrawn at the end of life and patients should be instructed to document this if consistent with their wishes. See also Fast Facts #12, 65, 162, and 178 for further discussion of advance care planning.
Terminal Care Hospice services should be considered for all patients with advanced disease. There are no evidence-based criteria for determining a 6 month prognosis; web-based reference 13 however provides some guidance. Labored breathing, secretion management, and restlessness are common terminal symptoms – see Fast Facts #1, 60, 109, 158, and 176.
References
- Moskowitz CB, Marder K. Palliative care for people with late-stage Huntington’s disease. Neurologic Clinics. 2001; 9(4):849-865.
- Bonelli RM, Wenning GK. Pharmacological management of Huntington’s disease: an evidence-based review. Curr Pharm Design. 2006; 12(21):2701-20.
- Huntington Study Group. Tetrabenazine as antichorea therapy in
- Paulsen JS, Hoth KF, Nehl C, Stierman L. Critical periods of suicide risk in Huntington’s disease. Am J Psychiatry. 2005 ; 162(4):725-31.
- Folstein SE. Huntington’s disease: a disorder of families. The
- Nance MA, Sanders G. Characteristics of individuals with Huntington’s disease in long-term care. Movement Disorders. 1996; 11:542-548.
- Sorensen SA, Fenger K. Causes of death in patients with Huntington’s disease and in unaffected first degree relatives. J Med Genet. 1992; 29:911-914.
- Lanska DJ, Lavine L, Lanska MJ, Schoenberg, BS. Huntington’s disease mortality in the
- Myers RH, Sax DS, Koroshetz WJ, et al. Factors associated with slow disease progression in Huntington’s disease. Arch Neurol. 1991; 448:800-804.
- Mitchell S, Buchanan J, Littlehale S, Hamel M. Tube-feeding versus hand-feeding nursing home residents with advanced dementia: a cost comparison. J Am Med Direct Assn. 2003; 4(1):27-33.
- Vamos M, Hambridge J, Edwards M, Conaghan J. The impact of
- Hospice Eligibility Flipchart: Huntington’s Disease. VistaCare Inc. Available at: http://www.vistacare.com/eligibility/flipchart. Accessed December 7, 2007.
- Foroud T, Gray J, Ivashina J, Conneally PM. Differences in duration of Huntington’s disease based on age at onset. J Neurol Neurosurg Psych. 1999; 66:52-56.
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