Neurogenetic Disease Patients and Caregivers May Benefit from End-of-Life Conversations
—A French study based on questionnaires and interviews showed a willingness among patients with neurogenetic diseases and their primary caregivers to talk about sensitive issues like advance directives and end-of-life care.
Despite the long-held belief by many clinicians that discussing end-of-life issues with patients and their caregivers increases their emotional distress, such conversations may actually lead to improved quality of life—both for patients and their caregivers, a recent study suggests.1
Investigators in France showed that when patients with neurogenetic diseases were asked to engage in end-of-life discussions with their physicians, they did not feel depressed, worried, or sad; in fact, they said that they would welcome these conversations.
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The study, led by Anne-Claire Dorsemans, PhD, from the Sorbonne University Paris Brain Institute, was developed to explore patients’ and caregivers’ perceptions, needs, and expectations around end-of-life discussions and advance directives. It was an observational, cross-sectional, mixed-methods study from a single center, and included patients with severe neurogenetic diseases and their caregivers. Assessment tools included disease severity scales, psychosocial and emotional scales, in-house questionnaires, and semi-directed (also known as “semi-structured”) interviews, which use primarily open-ended questions.
Vulnerable patients, important discussions
Study participation was originally proposed to 237 patients and caregivers between October 2020 and March 2022.1 Dr. Dorsemans and her colleagues had hypothesized that patients with severe neurogenetic diseases would be particularly concerned about end-of-life issues and advance directives, and would be prone to talk about these subjects because of the serious nature of the diseases and their associated consequences. However, only 34% of the eligible patients agreed to participate in the study; it wasn’t known why the rest declined.
The final study included 81 patients with severe disease—Huntington’s disease (n=30), autosomal dominant cerebellar ataxia with polyglutamine expansion (n=35), or Friedreich ataxia (n=16)—and 43 primary caregivers. A majority of the patients (54.3%) were men, and primary caregivers were slightly older than patients (an average of 57.5 versus 54.6 years old, respectively).
What the French investigators discovered
Upon reviewing the completed psychosocial and emotional scales, the investigators found that patients had little or no depression or anxiety, although the depression scores were slightly higher in patients than in caregivers. Caregivers considered their burden in caring for the patient to be mild or moderate.
More than 60% of the patients responded that a discussion of end-of-life issues would not be distressing, but rather, would be reassuring and helpful, and that they would specifically request such consultations. That said, patients usually wished to talk to their friends or family members first, then to their healthcare teams.
However, only 7% of the study participants—including both patients and caregivers—had written their advance directives and barely 1 in 4 (28%) had designated a surrogate decision-maker. In both cases, those rates were significantly lower than those in the general French population (18% and 44%, respectively; P<.05).
Are patients and caregivers ready to act?
“[This] study highlights that, despite a seeming lack of interest and little knowledge about end-of-life issues and legislation, patients and caregivers affected by neurogenetic diseases welcome this information when proposed within a certain context,” the investigators wrote. “They expect comprehensive and progressive support for end-of-life discussions among themselves and with healthcare professionals.”1
Although the current study enrolled French citizens and their caregivers, in the U.S., Medicare has instituted physician reimbursement for advance care planning conversations, including discussions of advance directives, as of 2016. Nevertheless, only about 1 in 3 Americans is estimated to have an advance directive for end-of-life situations, a rate that’s similar between patients with chronic illnesses and healthy adults.2
“There is a lack of guidelines around these topics at an organizational level and a general discomfort or lack of familiarity with palliative options on the part of neurologists,” Dr. Dorsemans and her colleagues concluded. “The stakes are high for patients at risk of losing communication and cognitive abilities to anticipate these inescapable future decisions.”1
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