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. Life threatening complications may result from aspiration, chronic infections, poor nutrition, falls, or cardiovascular disease.
- ~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
- First-line strategies are non-pharmacologic and include gait/balance training, speech therapy, and orthotics and leg weights to assist with upright posture.
- 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 US. Haloperidol and other antipsychotics are also used for chorea, although trials evaluating their effectiveness have shown mixed results.
- Psychiatric manifestations are present in over half of HD patients.
- 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.
- 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.
- Low doses of scheduled benzodiazepines before meals or propranolol are used to control motor manifestations and anxiety related to eating.
- 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 There are no evidence-based criteria for determining a 6 month prognosis in HD; web-based reference 13, however, provides some guidance regarding hospice eligibility. A retrospective, multi-centered study of over 100 HD patients, suggested that the hospice length of stay is longer than non-HD hospice patients and only a significant minority of HD patients are able to die at home. Labored breathing, excessive secretions, and restlessness are common terminal symptoms – see Fast Facts #1, 60, 109, 158, and 176.
- 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 Huntington disease: a randomized controlled trial. Neurology. 2006; 66(3):366-72.
- 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. Baltimore, MD: The Johns Hopkins University Press; 1989.
- 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 United States. Neurology. 1988; 38:769.
- 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 Huntington’s disease on family life. Psychosomatics. 2007; 48(5):400-404.
- Wong MT, Chang PC, Yu YL, et al. Psychosocial impact of Huntington’s disease on Hong Kong Chinese families. Acta Psychiatry Scand. 1994; 90:16-18.
- Hospice Eligibility: Huntington’s Disease. Hospice of the Valley Inc. Available at: http://www.hospiceofthevalley.net/med-pros/eligibility-guidelines. Accessed July 30, 2015.
- 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.
- Mendlik M, Frank S, Casarett D. Utilization of Hospice Services in a Population of Patients with Huntington’s Disease (TH307-C). Journal of Pain and Symptom Management 2015; 2: 333-334.
Version History: Originally published April 2008. Version re-copy-edited in June 2009; then again July 2015 – reference #15 added and incorporated into text, web resources updated.
Fast Facts and Concepts are edited by Sean Marks MD (Medical College of Wisconsin) and associate editor Drew A Rosielle MD (University of Minnesota Medical School), with the generous support of a volunteer peer-review editorial board, and are made available online by the Palliative Care Network of Wisconsin (PCNOW); the authors of each individual Fast Fact are solely responsible for that Fast Fact’s content. The full set of Fast Facts are available at Palliative Care Network of Wisconsin with contact information, and how to reference Fast Facts.
Copyright: All Fast Facts and Concepts are published under a Creative Commons Attribution-NonCommercial 4.0 International Copyright (http://creativecommons.org/licenses/by-nc/4.0/). Fast Facts can only be copied and distributed for non-commercial, educational purposes. If you adapt or distribute a Fast Fact, let us know!
Disclaimer: Fast Facts and Concepts provide educational information for health care professionals. This information is not medical advice. Fast Facts are not continually updated, and new safety information may emerge after a Fast Fact is published. Health care providers should always exercise their own independent clinical judgment and consult other relevant and up-to-date experts and resources. Some Fast Facts cite the use of a product in a dosage, for an indication, or in a manner other than that recommended in the product labeling. Accordingly, the official prescribing information should be consulted before any such product is used.