Defining Unique Needs for Palliative and Hospice Care in Huntington Disease

hospital patient nurse caregiver
hospital patient nurse caregiver
Research has shown significant gaps in knowledge on palliative services among neurology residents, resulting in low referral rates.

The course of Huntington disease (HD) can take decades to fully evolve from the early hallmarks of chorea, mood disturbances, and mild cognitive impairment to end-stage disease in which patients often die from complications of dementia and/or significant decline in motor function.1 Palliative and hospice care play an important role in the long-term management of HD, including ongoing symptom relief measures, caregiver support, and end-of-life care.2

Palliative Care vs Hospice

The services offered by palliative care and hospice often overlap significantly, particularly in imminently terminal conditions such as cancer and heart disease, where progression is much more rapid than in HD. Palliative care and hospice both focus on comfort measures and end-of-life care when the benefits of disease-modifying treatments have been exhausted. By definition, patients must be given a prognosis of 6 months’ survival or less by 2 physicians to receive Medicare benefits for hospice.3,4,5

The Accreditation Council for Graduate Medical Education IV.A.5.b).(3).(c) states that neurology residents “must demonstrate knowledge of palliative care, including adequate pain relief as well as psychosocial support and counseling for patients and families.”6 Despite this, a review by Tarolli et al2 identified significant gaps in resident training in palliative care principals, and found that neurologists in general have low rates of referrals for palliative care services, suggesting they do not often discuss these options with patients and caregivers.7-9 Of the patients referred to hospice care for dementia, less than 30% have access to care for 3 days or more.10,11 In addition, a survey conducted by Downing and colleagues found that only 38.2% of patients with HD had signed advanced directives, and less than half had discussed end-of-life plans.12

Special Needs of HD

HD produces a complex compendium of behavioral, psychiatric, movement, and cognitive issues that escalate in late stages of the disease, often overwhelming the abilities of family caregivers, and ultimately leading to the death of the patient. The extensive needs of these patients requires special end-of-life care and specialty social work services for suitable placement in skilled nursing facilities to provide high-quality care.13 A 2014 overview by Boersma et al14 referred to a need for a paradigm shift in the approach to palliative care for patients with neurologic diseases. “In our clinical experience regarding spiritual well-being, neurology patients experience their disease as something intrinsic to their person, which clearly differs from patients with cancer who see ‘the cancer’ as something outside of themselves,” the authors wrote.

Recent data from Johnson et al15 found that patients with HD are younger (57 vs 76 years old) and spend more time in hospice than other hospice patients (median, 42 vs 17 days). “The high rates of nursing-home placement for HD patients likely reflects the additional burden of high rates of behavioral issues (eg, irritability, anger, impulsiveness) seen in this population. As a result, patient and family safety often overshadow other goals of care,” Benzi Kluger, MD, MS, chief of the neurology supportive and palliative care section at the University of Colorado School of Medicine (and coauthor of the Boersma study14), told Neurology Advisor. “Perhaps even more impressive than the 42-day median stay is that 28 of the 101 patients were either still alive or discharged from hospice because they no longer met hospice criteria,” she added.

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Providing palliative measures for HD is challenging for many reasons, primarily because there are no specific prognostic criteria and the disease progression is extremely variable. Dr Kluger pointed out that patients with HD have patterns of disability and dementia at much younger ages than are typical in the healthcare system. “This presents psychosocial challenges for social workers, counselors, home health care providers, nursing homes, and hospices that are prepared to deal with these issues in older adults with Alzheimer’s but not younger adults.”

Specialty Palliative Care for HD

“When we see HD patients in our neuropalliative care clinic, we cover the same basic issues (medical symptom management, psychosocial support, spiritual well-being, advance care planning), but anticipate several unique challenges depending on the symptoms, stage, and goals of care of the HD patient and their family,” Dr Kluger explained. “As HD affects cognition, mood, and behavior, we need to assess capacity and may need to place limitations on independence to maintain safety. We also work intensively, and frequently consult psychiatry, to manage complex mood and behavioral symptoms. Other challenges include finding creative ways to help relatively young patients and families find ways to stay connected to their community and activities they find meaningful. And there are many more resources for older adults with these issues than for younger adults,” she said.

Caregiver Burnout

Aside from the physical demands of caring for a patient with HD, caregivers have the challenge of managing their own psychological issues related to the patient’s cognitive decline, delusions, and excessive mood swings. Bereavement for these caregivers is complicated by a sense of losing their loved one in small doses.

“The field of palliative care is evolving to recognize that palliative care needs for many, and perhaps most, illnesses begin at the time of diagnosis, if not earlier. HD is a great example of an illness where palliative care needs often come before the diagnosis for both patients and families, due to subtle cognitive and personality changes preceding diagnosis, the stress of seeing other family members live and die with HD, and the stress of living with the possibility of developing HD regardless of whether they pursue presymptomatic testing,” Dr Kluger said.

At this time, there are no specific protocols for palliative care for HD. Dr Kluger pointed to the need for more empirical studies to guide clinicians, caregivers, patients, and families in best practices for assessment and management of HD over the full course of the disease. She and her colleagues are at work on a small grant to better define the palliative care needs and preferences of patients and families with HD to create a standardized, patient- and family-centered approach to palliative care in HD.


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