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HD InsightsCreating a Springboard for Neuropalliative Care

Survey shows meaning and purpose are the strongest ally for families with HD

Even in the throes of a devastating disease, meaning and purpose remain fundamental to the human experience. A survey co-led by first author Leonard Sokol, MD, found that people with HD who have strong meaning and purpose in their lives fare best in terms of emotional health, lower suicidality behavior and less desire to hasten death. This mirrors findings among people with chronic cancer, a group that has been studied extensively.

“While not surprising that meaning and purpose would rank high, its prioritization as the top need for people at all stages of the disease was striking,” Sokol said. “Having this now in black and white may be what we need to drive changes in palliative care in a population for which it has been nearly absent.”

Funded by the Huntington’s Disease Society of America, the NIH National Institute of Neurological Disorders and Stroke, and the National Center for Advancing Translational Sciences, the aim of this two-year, 300-participant survey (HDQLIFE) was to understand the state of palliative care for people with HD. This is important because there appear to be wide disparities in its provision compared with that in chronic cancer or Alzhiemer’s disease, for example. The study, published in the Annals of Clinical and Translational Neurology, is entitled “Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life.”

The survey findings by Sokol and his colleagues establish an evidence base that can be used
to drive improvements in HD palliative care and close these gaps.

“Basically, we convened hundreds of family members and several HD professionals and asked, ‘What are the physical, the mental, and the spiritual symptoms at each stage?’” Sokol said. “Families need a constant level of resources and involvement because it is such a devastating disease. So that was the first step — to find and shine a spotlight on what these individual and family-related needs are.”

The Emergence of Neuropalliative Care for HD

Palliative care, which focuses on the physical, mental, social, spiritual and existential needs of those with serious illness, as well as those of their caregivers, is a relatively new subspecialty, recognized by the American Board of Medical Specialties in 2013.

Many confuse palliative care with hospice, which is provided in the last six months or so of life. Palliative care can be used at any point for patients with life-limiting illness, and it can be provided with curative intent as well as to care for those with terminal illness.

Sokol says that long before patients with HD near the end of their lives, they can benefit from palliative services.

“When we started this initiative, we looked at palliative teams of physicians, chaplains, social workers, psychologists, pharmacists and nurses working together with families facing cancer. We asked, how do we apply this model to people with Parkinson’s to ALS, Alzheimer’s… to HD?”

For patients with HD, palliative medicine means focusing on planning for the future in terms of medical needs, both for the individual with HD and their family, but also on existential distress and suicidal thoughts and behavior.

“It’s no surprise that studies show Huntington’s disease is one of the leading neurological diseases associated with suicidal thoughts and actual attempts. Whether this behavior is governed by the disease itself, by outside factors, or by a combination of both is very hard to tease apart. Yet, whatever the case, we hoped the answers to the survey questions would have actionable significance for improving this mindset,” he said.

Clinician Feedback on Gaps

Despite board recognition of palliative care as a specialty, the actual provision of palliative care to patients with HD is still thin. In a  2018 study by Downing et al., only 11% of people with Huntington’s disease reported having a conversation with a clinician about death and dying or where to die. Many clinicians report having inadequate time, comfort and skill to help patients with HD with issues related to death anxiety, planning for the future and making decisions about their end-of-life wishes.

In fact, while 81% of the clinicians surveyed felt it was in their purview to treat existential suffering, less than 50% felt skilled at doing so.

Underscoring these needs, the HDQLIFE survey found that 34% of clinicians felt their patients exhibited existential suffering “quite” or “very” often. Fifty-seven percent “mostly” or “strongly” agreed that lack of meaning contributed to their requests to hasten death.

Three Palliative Care Targets

Sokol says the three palliative needs that raised the antenna of the researchers were: meaning and purpose, concerns with death and dying, and advanced care planning.

“We wanted to know how these associated with health-related quality of life changes, ”Sokol said. “To answer that question, we separately analyzed each of these three measures that had jumped out at us.” 

Meaning and Purpose

A 2016 study by Carlozzi, et al. showed that meaning and purpose is an HD-validated patient-reported outcome. What the researchers hadn’t known was that it topped the list of unmet needs.

Sokol’s team found that higher meaning and purpose was associated with greater enjoyment and life satisfaction and less depression, regardless of the of the magnitude of symptom burden. Baseline meaning and purpose predicted this across all disease stages.

“We found that having meaning and purpose was absolutely essential to helping people with HD live the most productive life that they could possibly live,” Sokol said.

“When people are first diagnosed or know they are at risk, it’s common to ask themselves, ‘What was the point of living? What should I do? How does society look at me? What will this do to my family?’ Should I end my life?’,” Sokol said. “More than half of the survey respondents felt that a lack of meaning contributed to a desire to hasten their death.”

When they looked at how meaning and purpose associated with changes in quality of life down the line, they found that even in the face of a horrific symptom burden like chorea and dysphagia, many of those who had higher levels of meaning and purpose still reported that they had an enjoyment of life – some even reported the capacity for joy. In fact, higher baseline scores predicted better health-related quality of life, mentally and socially as well, at 12 months and at 24 months out.

“The big takeaway from this is that we have a potential therapeutic target. Not to oversimplify, but if the patient, the caregivers and the providers know the power of this and work to help patients reconnect to their life’s meaning after the trauma of learning they are at-risk or have HD — or if they can find new purpose, whether it is joining in the HD community’s work or something else — maybe they could improve their quality of life from early on,” Sokol said.

Death Anxiety

High baseline death anxiety, or the fear of worsening illness and what it will bring, predicted poorer meaning and purpose down the line, as well as other negative QoL indicators. Sokol says one of the key takeaways here is that this is not just anxiety in these cases; it is specific to death and dying, making it a better-defined target for palliative care.

“We saw that people with death anxiety had higher levels of suicidal behavior at the follow-up dates. They had worse mental health across all stages of disease and at baseline, one and two years,” Sokol said. “So, if you’re developing a therapeutic intervention, you want to try to influence not only the sense of meaning and purpose, but the degree of death anxiety.”

End-of-Life Planning

Advance care planning was identified as a third highly unmet need. Sokol describes end-of-life planning as an act based on sharing your values, your goals, your hopes, with your family and the medical team that will guide you now and into the future. This is a quintessential family-based process within healthcare, but the HD patient has an especially acute need to decide who will speak for them when they cannot.

“It’s not something that is really happening often in the HD world,” he said. “One of the fears has been that if people with HD were encouraged to do end-of-life planning, it might increase what is already a high level of suicidal thoughts in this population. The concern has been that families may not want to talk about it, feeling that maybe behavior will get worse if they do.”

“On flip side, patients are also thinking, ‘Who in my family, what trusted person, is going to make decisions for me now? What are my medical preferences when my HD gets so bad that I can’t swallow? If I can’t think, I can’t chew, do I want to live, and who’s going to take care of all this? What am I going to do with my money, my estate? Who’s going to be my financial power of attorney? Who is going to care for my kids? How do I plan the timeline for when that will happen?’”

Sokol says there has been little structure around helping families with these weighty questions, so oftentimes it’s done so late that it becomes a non-issue because the person is already too cognitively compromised.

“I’ve seen it, and it is extremely distressing to the families not to know, for example, if their loved one wanted to return to the hospital if they got pneumonia,” he said. “Some families naturally feel that if they talk about the future in those terms it will cut off hope.”

To the contrary, studies looking at cancer show that with advance planning there’s a reduction of stress and increased levels of peace among patients, family members and other caregivers. Through the survey, Sokol and his colleagues sought to learn whether the same would be true in such a different disease.

“We know they need the advanced planning, but if it’s causing more suicidal thoughts and actions, then you really have a conundrum there. I was holding my breath through the study.”

The news was strongly reassuring, with the finding that there was no association between doing any form of advanced care planning with family members and suicidal thoughts and behaviors.

“There was no uptick at all, in any stage of the disease,” Sokol said. “So that data, I think, added this layer of peace and reassurance that when we work from a clinical angle, families and the doctors in the room, and the social workers and other providers, can have this kind of conversation about these topics without feeling like we’re going to do harm.”

“The message is: we need to talk to patients and families about how to hope for the best and at the same time, plan for the worst.”

The State of Palliative Care Today

Sokol says he has seen evidence that talking about these findings and the emergence of neuropalliative care for HD are starting to change the way providers talk with their patients.

“At the end of the day, that to me is the most rewarding aspect of doing this study. This patient-reported clinical research is having an impact on the face of clinical care. And it’s changed my practice — the way I approach things when I’m seeing patients with degenerative disease. I think it’s really awesome to have this framework to enhance care.”

About HD Insights

Our mission is to promote, disseminate, and facilitate research on Huntington’s disease. To fulfill this mission, we are guided by an outstanding editorial board that includes representatives from three continents, academia, industry, and the HD community.