Palliative Neurology

Palliative care medicine is a multidisciplinary approach to improving quality of life for individuals and their families at any stage of serious illness, from diagnosis through the end of life. Although often associated with diseases such as amyotrophic lateral sclerosis (ALS) or glioblastoma, fundamental palliative care skills of managing symptoms, providing basic psychosocial and spiritual support, and communicating effectively¹ are broadly applicable across for all neurologic diseases, even those that are not imminently life-limiting. Core palliative care skills (Table 1) should be considered an essential part of a neurologist’s tool kit. In a case-based approach, this review will help readers develop these skills and apply them in practice.

Breaking Serious News

From the perspective of a neurologist, a new diagnosis of Parkinson disease (PD) may not seem like bad or even serious news. After all, PD is among the most treatable of neurodegenerative illnesses, and people may live well for decades with PD. However, uncertain prognosis, symptom burden rivaling that of advanced metastatic cancer,² and functional dependence may all limit quality of life, underscoring palliative care needs over the course of PD. Sharing a new PD diagnosis clearly and empathetically promotes prognostic awareness, fosters a therapeutic patient-physician relationship, and paves the way for shared decision-making as the illness advances. A 6-step protocol, SPIKES (Table 2),³ is helpful for breaking serious news and can be applied to any life-altering neurologic diagnosis (eg, ALS, Alzheimer disease [AD] or multiple sclerosis [MS]).

The SPIKES steps are intended to progress in order, but individual steps may be omitted as a clinical situation dictates. Setting up the scene ensures the environment is conducive to discussing serious news. In the outpatient clinic, this typically means sitting down, ensuring good eye contact, and minimizing interruptions. Give patients a moment to put on their shoes, if needed, and consider rearranging seating to minimize the intrusion of computer monitors or other electronic devices. Next, assess the patient’s perception with an open-ended question (eg, “What have your other doctors suggested might be going on?” or “What worries you?”) These types of questions allow you to tailor your response accordingly, correct misinformation, and address any specific concerns that may have arisen during the visit. You may, for example, learn that a patient with early PD inaccurately believes they will need nursing home care in a matter of months, allowing you to offer specific reassurance. The final step before sharing the diagnosis, often omitted, involves asking for an invitation to proceed, ensuring patients are open to receiving more information. Consider inserting a “warning shot” if you expect a person to be surprised: “I’m afraid I have some serious news.”

Sharing knowledge should be succinct—ideally, a short 1-sentence headline (eg, “Your history and neurologic examination are diagnostic of Parkinson disease.”) Importantly, this should be followed by a pause. When faced with silence, patients fill the void and may express emotions (eg, anxiety or perhaps relief). Acknowledging any emotion is the next step, with statements like“I wish I had better news” or “I can see you’re upset, tell me what is on your mind.” The NURSE mnemonic (Table 3) gives helpful prompts for responding empathetically to emotion. If silence goes unanswered, consider an open-ended question (eg, “For some people, that diagnosis feels like serious news; how did it land for you?”) Recognizing and responding to emotion facilitates a transition from an emotional to a cognitive space, allowing a final step of summarizing and strategizing next steps. Avoid the pitfall of discussing further testing or treatment before emotions are acknowledged. Breaking bad news in the setting of diagnostic uncertainty adds complexity; however, limiting knowledge shared initially to a succinct headline still applies. Consider parkinsonism with atypical features, in which you could say, “You have parkinsonism. I’d like to explain what that means, how it differs from Parkinson disease, and where we go from here.”

Psychosocial Needs

In addition to physical symptoms, the psychologic burden of PD is high for individuals with PD and their caregivers. From the time of diagnosis, fear of functional dependence, need for burdensome care from family members, and grief over changes in identity are pervasive.⁴ Caregiver burden has a physical and psychologic component; spouses describe their own loss of independence and mental exhaustion from constant vigilance.^5,6 Unaddressed psychologic symptoms may lead to social isolation, further diminishing quality of life for patients and caregivers. As a physician, recognizing and acknowledging these symptoms, often with a statement of empathy (Table 3), has a large positive effect in addition to considering caregivers’ needs in optimizing a care plan. Chaplains and social workers may provide added spiritual support, help identify support groups, and maximize in-home resources.

Communicating Effectively to Navigate Conflict

Communicating effectively to navigate conflict is a key palliative care skill. In Mrs. G’s case, conflict exists between her stated preferences, her clinical team’s obligation to honor those, and her surrogate decision-maker’s requests. Although guidelines suggest careful hand feeding of adults with advanced dementia is at least as good as tube feeding for outcomes of aspiration pneumonia, functional status, comfort, and death,⁷ many physicians feel obligated to offer feeding-tube placement if requested by surrogate decision-makers.⁸ Effective communication can mitigate this conflict and ensure meeting goals of the patient, surrogate decision-makers, and the neurologist.

Prioritize Building Rapport

When there is potential conflict, establishing a good relationship with patients and families smooths the way to address difficult topics on subsequent visits. Ask open-ended questions, such as “Tell me about your mom” or “What should I know about her to take good care of her health?” These types of questions put the patient at the forefront of the discussion while providing valuable insight into perceptions and preferences that will inform surrogate decision-making. Ask about an unusual or unique object in the patient’s room, (eg, a photograph or drawing). Acknowledge emotion and offer empathy using NURSE statements (Table 3).

Explore Values and Preferences Before Decisions

Use open-ended questions like “When you think about the future, what’s important to your mom? What does she value?” or “What scares you, and what is she hoping to avoid?” Faith and spirituality often play a role. The FICA spiritual history tool⁹ (Table 4) can be used to explore religious or spiritual faith and how it affects making decisions about health. The FICA tool also helps identify any role of a faith community in understanding how spiritual issues should be addressed during care.

Find Common Ground

Once values and preferences are established, it often becomes clear that patients, families, and physicians initially thought to be in conflict have more in common than initially appreciated. Prioritizing a discussion of values over positions regarding what to do (eg, whether to place a feeding tube) leads to creative solutions that promote goal-concordant care.

Make a Recommendation

With values and preferences established, neurologists may ask permission to recommend treatments honoring those values. These difficult conversations may span multiple visits. In the case of Mrs. G, rapport was established when Dr. W praised Mrs. G’s family for their care of her and found common values of minimizing suffering and unnecessarily invasive procedures. Mrs. G’s family and Dr. W agreed these goals could be met with aggressive treatment of constipation and other physical symptoms that might limit oral intake, with careful hand feeding and a plan to judiciously offer subcutaneous fluids as needed if there was objective evidence of discomfort from dehydration, obviating the need for feeding tube placement. Mrs. G was discharged with support of hospice.

Discussing Prognosis

Discussing prognosis in neurologic disease typically requires attention to survival and functional status. Adding to the challenge, disease trajectories often vary among individuals, creating uncertainty. The duration of recovery over weeks to months, or longer, limits practicality of time-limited trials that are commonly used on medical or cardiac intensive care units.

Use the SPIKES and NURSE Protocols for Prognosis

The SPIKES (Table 2) and NURSE (Table 3) tools are as useful for discussing prognosis as for sharing a diagnosis. Share the knowledge succinctly, “His long-term prognosis is uncertain. I’d like to discuss the range of possible outcomes.” Beware of statements or requests that can sidetrack the conversation, such as “Isn’t there something you can do to help him recover more quickly?” Underlying this statement is emotion; respond with empathy, “I wish I had a medicine that would help him regain strength on his right side more quickly.” Avoid the cognitive trap of explaining the physiology of stroke recovery.

Emphasize What Is Known First

Short-term outcomes are often better known and can be discussed concretely before describing best, worst, and most likely long-term outcomes, using ranges for uncertainty. “He has a serious brain injury. In the short term, he will need a feeding tube and placement in a rehabilitation facility. In the best case, over weeks, months, or longer, he may be able to walk independently, recover some language, take food by mouth, and live at home with assistance. In the worst case, he could suffer complications such as pneumonia that will limit his recovery, leave him in a wheelchair and dependent on others for care, require artificial nutrition and nursing home care for the rest of his life. Most likely, he’ll get back some strength, but not enough to walk independently. He may take some food by mouth, but not enough to meet his nutritional needs. Communication will remain impaired, and he would need a substantial amount of care at home or long-term care in a nursing home.” Saying “I worry” conveys the gravity of a situation, without committing to a specific prognosis. “I worry that even in the best case, we may not get him to where he was before the stroke.”

Shared Decision-Making

Implicit in Mr. C’s case is a choice to proceed with gastrostomy tube placement and care focused on stroke recovery or to pursue a comfort-focused care plan. Even when a patient’s values and preferences surrounding a decision are relatively clear, the burden of surrogate decision-making can be agonizing for family members. The Best Case/Worst Case tool facilitates this discussion and can be employed with patients or surrogates.^10,11

The Best Case/Worst Case Tool

The Best Case/Worst Case tool centers around a graphic aid (Figure),typically drawn by hand during a family meeting. Using the graphic as a guide, the neurologist describes in detail the best, worst, and most likely outcome for each treatment plan. Boxes are used to represent the best and worst cases; a star is placed on a line that runs from best to worst to indicate the most likely scenario when a range of outcomes is possible. Outcomes should be described as a detailed patient-centered narrative, as if telling a story. After completing the graphic and eliciting preferences using open-ended questions (“When you think about the future, what do you think is important to your husband?”), neurologists may, with permission, make a recommendation based on the patient’s ascertained values. In the case of Mr. C, treatment A is care focused on stroke recovery and treatment B is comfort-focused care: “We would shift our focus to keeping your husband comfortable. This means continuing medicines for pain and shortness of breath and stopping treatments that aren’t adding to his comfort, which might even be prolonging his death. In this case, we recommend against a feeding tube, and would instead offer small sips of water by mouth. My hope is that you’ll have some time for friends and family to say goodbye. I anticipate he’ll die comfortably in the coming days to a short few weeks.” With further discussion, the neurologist learned that Mr. C would have considered even short-term artificial nutrition unacceptable, and his wife opted for comfort-focused care.

Conclusions

Palliative care medicine covers a broad skill set of managing symptoms, providing basic psychosocial and spiritual support, and communicating effectively. Communication barriers resulting from cognitive impairment, uncertainty in disease trajectory, and existential distress related to loss of identity are just some of the unique palliative care challenges that emerge in neurologic disease.¹² Leaders in the emerging field of neuropalliative care identify improved palliative care education for neurologists and reducing the stigma surrounding palliative care as priorities for incorporating palliative care into neurologic practice. Recognizing that the use of palliative care may improve quality of life for patients and families across the spectrum of neurologic illness, neurologists should consider palliative care a fundamental component of their tool kits.