Palliative Care of End Stage Parkinsonism: An Overview Including the Five Pillars Framework

Parkinson's Disease: More than a Movement Disorder

Although the focus of this article is on end‐stage parkinsonism, it is important to note that palliative care is not limited to advanced disease/end‐of‐life and that palliative care needs in parkinsonism can occur at any stage of the illness. ¹ Many patients liken the experience of being diagnosed with Parkinson's disease (PD) to having a bomb dropped on them: a theme evocative of destruction, turmoil, and fear. ² They look to their clinicians, families, and faith at this time, trying to make sense of “a new normal.” Although motor symptoms are often well controlled with current dopaminergic medications in early PD (the honeymoon phase) and can be adequately managed in many patients through medications and more advanced therapies for years to decades in the motor complications phase, significant disability and morbidity can arise as the disease progresses from recalcitrant motor symptoms (eg, balance), as well as non‐motor symptoms, and emotional, and spiritual issues. In fact, the symptom burden in patients with advanced PD (Hoehn and Yahr 3–5) is likely similar to or potentially worse than that those entering palliative care with cancer diagnoses, ³ highlighting the great need for a palliative approach for those with PD. ⁴

In addition to motor symptoms of bradykinesia, rigidity, and imbalance, patients with advanced PD increasingly experience difficult to control secondary symptoms such as chronic pain, mood disorders, dysphagia, and cognitive changes. ¹ , ⁵ Often these symptoms are a source of considerable distress for patients and their families. Further, PD affects not only the patient, but also the patient's family and caregivers who struggle with the logistics of caring for someone with a progressive neurological disease including coordinating specialist appointments, enlisting support services, and patient safety. Complicating matters, they must manage their own fatigue, burnout, grief, and worries for the future.

Limitations to Current Models of Care

Current systems of care for advanced PD generally include a primary care physician and a neuro specialist or movement disorders specialist, who both connect to the patient but may have little connection to each other. ¹ , ⁶ , ⁷ Although these care systems may manage primary motor symptoms well, it often falls short with other non‐motor symptoms and psychosocial support. ⁸ This model also struggles in identifying and easing caregiver burnout, addressing patient goals of care regularly, and preparing and referring the patient appropriately for hospice care. ⁹

Need for Integrated Palliative Care

In discussing palliative care for parkinsonism, it is critical to distinguish the palliative care approach that can be delivered by any healthcare provider from the specialty of palliative medicine. Meeting the palliative care needs of people with parkinsonism on a population level requires collaboration across multiple entities including specialist palliative care, primary care/neurologists, and community support organizations. ¹⁰ As the palliative needs of persons with parkinsonism change over time, there is no single model of palliative care that will best meet all needs; rather there are several complementary models that can be used to most effectively and efficiently meet the needs of patients. ¹¹ The practice of primary palliative care is essential to proactively meet the majority of palliative care needs experienced by patients and families. ¹² Team‐based specialist palliative care has proven effective for patients with moderate to high palliative care needs, ¹³ and end‐of‐life palliative care/hospice may be necessary for persons with end‐stage parkinsonism.

Five Pillars of Palliative Care for Parkinson's Disease

The Parkinson Foundation developed the following five pillars (Table 1) to operationalize palliative care as a new standard across its center of excellence network: routine assessment and support for family care partners, recognition and support for emotional and spiritual concerns, systematic assessment and management of non‐motor symptoms, annual advanced care planning conversations, and timely referral to hospice (and specialist palliative care). ¹⁴ There is a need for continued involvement of the movement disorders specialists with all pillars. These pillars are applicable throughout the course of parkinsonism, but may become more pressing in advanced disease.

TABLE 1.

Five pillars of team‐based palliative care for Parkinsonism

Pillar	Rationale and role of Movement Disorder Specialist (MDS)
1. Care partner support	Care partners are essential for optimal patient outcomes and are at high risk for burnout and other adverse events. The MDS can screen caregivers, refer to social work, and provide guidance to plan for the future.
2. Nonmotor Symptoms (NMS)	NMS are major determinants of quality of life and care partner distress. The MDS can systematically screen for these symptoms and lead or coordinate treatment.
3. Spiritual and Emotional Support	Spiritual and emotional challenges beyond the psychiatric disorders of anxiety and depression are common sources of suffering. The MDS can provide validation for these experiences and refer to supportive counseling.
4. Advance Care Planning (ACP)	ACP is essential to providing goal and value concordant care. The MDS can initiate these conversations and provide patients/family with a roadmap of their illness.
5. Timely referral to specialist palliative care or hospice	Specialist palliative care may be needed for complex symptom management (eg, chronic severe pain) or end‐of‐life care. The MDS can initiate referrals and stay involved to guide neurologic aspects of care (eg, dopaminergic therapies near end‐of‐life).

Abbreviations: MDS, movement disorders specialist; NMS, non‐motor symptom management; ACP, advance care planning.

Palliative Care in Advanced and Terminal Parkinsonism

Care Partner Support

Advanced parkinsonism brings functional impairments in ambulation, instrumental, and basic activities of daily living, requiring progressive levels of assistance that stresses the home system. A substantial portion of home care is done by care partners like spouses, adult children, neighbors, or friends, who report feeling unprepared and under‐supported in this new role. ¹⁵ Notably, patients with an involved care partner have improved outcomes and quality of life. ³ Providing care partner support begins with determining how the care partner is doing and what needs they have. Even a few short, sincere questions (eg, what is toughest about this for you?) can demonstrate compassion and provide a sense of being seen and heard. ¹⁶ Support needed may range from emotional and spiritual to practical suggestions on care or services at home and may benefit from involvement of other disciplines such as social work. Specialist palliative care may improve some outcomes for care partners of persons with advanced disease. ¹³

Non‐motor Symptoms

PD patients experience a variety of non‐motor symptoms including fatigue, pain, constipation, and depression. These symptoms are leading drivers of quality of life, ¹⁷ , ¹⁸ and one of the most frequent causes of undertreatment is under‐recognition in clinical practice. ⁸ It is not uncommon for patients to be confused about which symptoms relate to their parkinsonism and, which come from other conditions (eg, arthritis) or aging, compounding under‐reporting. In fact, by using a simple checklist to systematically and comprehensively screen for symptoms, the involvement of a neuropalliative care team significantly improved symptom burden compared to standard care from a neurologist or movement specialist. ¹⁹ In advanced parkinsonism, psychosis is also a major driver of care partner distress and nursing home placement and merits aggressive treatment. ²⁰ Last, screening for symptoms that are not treatable medically may still be helpful in getting help to manage symptoms (eg, speech therapy for dysphagia), coping (eg, counseling around living with dementia), and guiding goals of care and prognosis discussions.

Emotional and Spiritual Concerns

Outside of the high risk of the psychiatric disorders of anxiety and depression, a person with PD experiences a series of losses related to motor and cognitive changes that can impact their close relationships, shatter their sense of self, and challenge their sense of meaning. ²¹ Recent research has shown that spiritual and emotional challenges such as demoralization are both common and distressing in PD ¹⁰ and that these aspects of the illness may be expected to drive perceived suffering. ² Simply naming these concerns can be helpful as patients often have shame around “negative” emotions and may fear having their distress pathologized as a psychiatric condition. Moreover, supportive counseling is likely to be better received, more appropriate, and more effective for these challenges when accurately recognized than antidepressants. ⁵

Advance Care Planning

Advance care planning (ACP) is a conversation between patients, families, and healthcare providers to establish values and plans of care for future changes in health and function. ⁶ In advanced parkinsonism, ACP provides an opportunity for clinicians to share a roadmap of what is ahead and to elicit the values and wishes of patients in navigating future decline. ²² , ²³ Other important outcomes of these conversations commonly include designating a healthcare proxy—particularly important to do before severe dementia—and an advance directive to avoid invasive interventions if no longer desired and increase chances of dying in their preferred manner, typically meaning dying at home rather than in a hospital or intensive care unit. ²⁴

Referral to Specialist Palliative Care or Hospice

When available, the addition of specialist outpatient or home palliative care may provide a much desired extra layer of support for patients and families and may improve outcomes such as symptom burden. ²⁵ , ²⁶ Although prognosis and disease stage may factor into these referrals, early evaluation of palliative needs is preferred as some patients—particularly with Parkinson‐plus disorders—may have high needs early in their illness. There are several possible tools to screen patients including the Brief Needs Assessment Tool (Table 2A). ¹³ , ²⁷ , ²⁸

TABLE 2.

Triggers for palliative care referrals

2A. Specialist palliative care Care partner distress Diminished quality of life because of intractable symptoms Complex symptom management (eg, severe chronic pain) Significant emotional, spiritual, or existential distress Complex goals of care or family dynamics Advanced disease with minimal help from therapeutic interventions and/or signs that terminal phase is approaching

2B. Hospice (end‐of‐life palliative care) Recurrent hospitalizations or significant decline following single hospital stay Weight loss and/or diminished appetite Dysphagia for liquids or secretions Diminished efficacy or need for dopaminergic medications Accelerated functional decline Advanced dementia often with increased daytime sleep

Consideration of hospice—or end‐of‐life palliative care—starts with recognizing that PD and related disorders are terminal conditions. PD is in fact the 14th leading cause of death in the United States. ²⁹ Although there is a need for guidelines specific to PD, research suggests that reasonable triggers to consider hospice include advanced dementia, weight loss, accelerating disability, dysphagia, recurrent hospitalizations, and diminished benefit from dopaminergic medications (Table 2B). ³⁰ , ³¹

Feeding Tube and Goals of Care Discussions

Dysphagia is common in advanced PD and may contribute to aspiration pneumonia, weight loss, and challenges in keeping up with nutrition and hydration. ³² Although percutaneous endoscopic gastrostomy (PEG) feeding appears to remedy the complications of dysphagia, the data in neurodegenerative illness does not support routine use. In general, PEG feeding in advanced dementia does not improve survival, quality of life, or improve care partner outcomes. ³³ , ³⁴ Similarly, in two large United Kingdom university hospitals, about a third of PD patients admitted from home who received PEG were still discharged to institutions, and almost a quarter experienced aspiration pneumonia. ³⁵ One reason for these suboptimal outcomes is that aspiration is related not only to food and drink, but also to secretions and reflux, which tube feeds do not effect and in fact could worsen. In the authors’ experience, PEG may be a reasonable option for very select patients with sufficient likelihood to withstand the procedure, when maintaining nutritional needs interferes with quality of life (eg, patient and spouse spending hours on meals).

Dopaminergic Medications and Deep Brain Stimulation at End‐of‐Life

Although end‐of‐life care is often led by palliative medicine specialists, there is still a need for input from movement disorders specialists, particularly concerning both motor and non‐motor symptom management. ³⁶ Notably, this should include guidance on whether and how dopaminergic therapies should be weaned, guidance for management of non‐motor symptoms, and advice for avoidance of antidopaminergic medications for agitation and nausea. In particular, dopamine medications may benefit from a “last in, first out approach,” ³⁷ , ³⁸ as their benefit wanes and side effects become more taxing. Symptomatic hypokinesia episodes can be ameliorated by frequent dosing of short acting levodopa/carbidopa paired with catechol‐o‐methyl transferase inhibitors ³⁹ or with transdermal rotigotine. ⁴⁰ , ⁴¹ Similarly, discussions about managing deep brain stimulation (DBS) should occur at the start of hospice, including considering ON/OFF testing to guide decisions as to whether to continue use and other potential complications (eg, replacing batteries) as end‐of‐life approaches. ⁴² Medications and DBS should be withdrawn thoughtfully and slowly given the risk of parkinsonism‐hyperpyrexia syndrome. ⁴³ Rectal formulations of levodopa have been proposed for persons with severe dysphagia, although in the authors’ experience complications of withdrawal are rare when death is imminent. ⁴⁴

Conclusions

We Can Improve Outcomes in Advanced Parkinsonism Through Palliative Approaches

For patients with PD, quality of life can be improved greatly by using a neuropalliative approach, including secondary symptom control, increased caregiver support, and improved engagement in life by planning for the future. ¹³ In considering how best to provide this care, it is better to consider complementary models as needed for each patient rather than expecting a single unified best model. ¹¹ Ideally this care would be provided in a proactive manner because it is easier to prevent issues or manage them when they are mild rather than to wait for the “right time” to bring in palliative care. This care would ideally be provided by a multidisciplinary or interdisciplinary team as the palliative care approach covers a wide range of domains going beyond the typical biomedical model including social work and chaplaincy.

As interest and demand for palliative care grows, movement disorders specialists may desire to pursue additional training in these areas and integrate them into fellowship training. Fortunately, there are a growing number of resources to aid in this charge including through VitalTalk, Ariadne Labs, the Center to Advance Palliative Care (CAPC), and the International Neuropalliative Care Society (INPCS) that sponsors the Education in Palliative and End‐of‐life Care Neurology Adaptation (EPEC‐N), a set of 26 slide sets on relevant topics that are linked to a textbook and now also available in both English and Spanish translations. ⁴⁵

There Is Still Much to Do

Despite advances in our understanding of the disease trajectory of parkinsonism and growing interest in applying palliative care approaches, the neuropalliative care field is still very much in its infancy. There is much work to be done from clinical, educational, advocacy, and research perspectives. In particular, there is a need for better evidence, including randomized controlled trials, to guide clinical practice and decision‐making in advanced and terminal parkinsonism.

Author Roles

(1) Research project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the First Draft, B. Review and Critique.

V.H.: 3A, 3B.

A.P.H.: 3A, 3B.

B.M.K.: 3A, 3B.

Disclosures

Ethical Compliance Statement: The authors confirm that the approval of an institutional review board and patient consent was not required for this work. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

Funding Sources and Conflicts of Interest: B.M.K. received funding for this work from the National Institute of Aging (NIA) (K02AG062745). The authors declare that there are no conflicts of interest relevant to this work.

Financial Disclosures for the Previous 12 Months: B.M.K. received research grants from National Institute of Nursing Research, NIA, and the Patient‐Centered Outcomes Research Institute; and received speaker honorarium from International Movement Disorders Society, Davis Phinney Foundation, and the Parkinson Foundation), V.H. has nothing to disclose), A.P.H. has nothing to disclose.