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Mapping the Literature: Palliative Care Within Adult and Child Neurology Alexis Dallara, Anca Meret and John Saroyan J Child Neurol 2014 29: 1728 originally published online 9 April 2014 DOI: 10.1177/0883073814527159 The online version of this article can be found at: http://jcn.sagepub.com/content/29/12/1728

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Topical Review Article

Mapping the Literature: Palliative Care Within Adult and Child Neurology

Journal of Child Neurology 2014, Vol. 29(12) 1728-1738 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0883073814527159 jcn.sagepub.com

Alexis Dallara, MD1, Anca Meret, MLS1, and John Saroyan, MD2

Abstract Objectives of this review were to examine definitions and background of palliative care, as well as address whether there is an increased need for palliative care education among neurologists. The review also explores what literature exists regarding palliative care within general neurology and child neurology. A literature review was conducted examining use of palliative care within child neurology. More than 100 articles and textbooks were retrieved and reviewed. Expert guidelines stress the importance of expertise in palliative care among neurologists. Subspecialties written about in child neurology include that of peripheral nervous system disorders, neurodegenerative diseases, and metabolic disorders. Adult and child neurology patients have a great need for improved palliative care services, as they frequently develop cumulative physical and cognitive disabilities over time and cope with decreasing quality of life before reaching the terminal stage of their illness. Keywords palliative care, end-of-life care Received October 24, 2013. Received revised December 04, 2013. Accepted for publication February 13, 2014.

Although hospice and palliative care were first developed for terminal cancer, its principles are applicable to neurologic illness as well. Unfortunately, less than 5% of all journal articles in palliative care are written for noncancer conditions.1 In contrast to cancer, neurologic illnesses have a longer and more variable time course, and it is often hard to determine exactly when a patient is entering the terminal stages of life.1 A mixture of preventive, therapeutic, and palliative care is often required throughout multiple stages of the neurologic illness. Previous studies have shown shortfalls in usage of palliative care in patients with long-term neurologic conditions,2 which can lead to poorer controlled physical and psychological symptoms, as well as spiritual distress, among a subpopulation already at risk for long-term suffering. A previous review of 53 articles regarding palliative care in general neurology concluded that there was minimal data examining use of palliative care prior to the last 6 months of life.3 Definitions of palliative care, however, often recommend that palliative care be initiated at time of diagnosis.1,4 Lack of education and experience regarding palliative care among neurologists is one of the more cited reasons1 for the underutilization of palliative care for patients with long-term neurologic conditions. Although previous studies have examined use of palliative care in adult neurology diseases such as amyotrophic lateral sclerosis, Parkinson, and Alzheimer, there is minimal literature surrounding palliative care in child neurology. Pediatric palliative care is an emerging field, and child neurology patients are one of the most common subgroups for which pediatric

palliative care consultations are called.5 The purpose of this literature review was to identify definitions of palliative care, determine whether there is a need for increased palliative care education among neurologists, and examine previous publications regarding the practice of palliative care in child neurology and general neurology as it can pertain to child neurology.

Methods The methods involved searches on PubMed, OVID Medline, Scopus, and the Cochrane Library. MeSH terms on PubMed and keywords utilized included brain neoplasms, neuromuscular diseases, muscular dystrophies, muscular dystrophy, Duchenne, neurodegenerative diseases, rare diseases, nervous system diseases, brain diseases, palliative care, quality of life, hospices, terminal care; newborn, infant, child, and adolescent. Studies and editorials from all health care disciplines, including medicine, nursing, and psychology, were considered. The bibliographies of identified manuscripts were reviewed. Additional methods included textbook searches, online and by recommendation of hospice

1

New York Presbyterian Hospital, Columbia University Medical Center, New York, NY, USA 2 BAYADA Hospice, Palliative Care, Norwich, VT, USA Corresponding Author: Alexis Dallara, MD, New York Presbyterian Hospital / Columbia University Medical Center, 622 West 168th St New York, NY 10032, USA. Email: [email protected]

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Table 1. Identification of Guidelines Regarding Palliative Care of Patients Living With Neurologic Diseases. Year published Conditions

Title

Origin

Palliative Care in Neurology (AAN Position Statement)

United States 1996

Study Group of Bioethics and Palliative Care in Neurology

Italy

Clinical Practice Guidelines for Quality Palliative Care (National Consensus Project)

United States 2009

2000

Guideline Development Group for Great Britain 2008 Long-term neurological conditions

The care of the patient with amyotrophic lateral sclerosis (AAN practice parameter)

United States 2009

Taskforce on the development of guidelines and a core curriculum for the palliative care for people with neurological disease (European association for palliative care)

Europe

Ongoing

Significance

First and only position statement States that neurologists have a duty to provided by the AAN on palliative provide adequate palliative care care Identifies the ethical problems Includes information on the associated with chronic neurologic International Working Group on diseases; defines palliative care and Palliative Care in Neurology, a application of palliative care Research Group of the World treatment in the neurologic field Federation of Neurology Goal was to provide a blueprint for Identifies 8 domains of quality palliative care: structure, physical, the foundation of palliative care and psychiatric, social, spiritual, cultural, hospice programs in the United and ethical/legal aspects of care, as States well as care of the imminently dying patient Convened by the National Council for Neurology, rehabilitation, and palliative care services should work Palliative Care and the British closely together; a person with an Society of Rehabilitation Medicine LTNC should be referred to with the Royal College of Physicians specialist palliative care services if they have a limited life span, distressing symptoms, or desire for end of life planning There is a paucity of evidence-based Revised recommendations for the guidelines for management of commanagement of ALS based on plications of ALS, especially in review and analysis of peerregards to level A evidence reviewed literature Taskforce established in 2008; still in Aims to develop guidelines for care of patients with ALS, MS, Stroke, process, awaiting final Huntington, and Parkinson disease, documentation as well as form a core curriculum on neurologic palliative care for palliative care and neurology professionals

Abbreviations: AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; LTNC, long-term neurologic conditions; MS, multiple sclerosis.

and palliative medicine faculty (JS, AM). Consultation with a librarian (AM) and pediatric palliative care expert (JS) aided in further refining the search. More than 100 articles and textbooks were retrieved and reviewed. No date limitations were used. Single case reports, literature not in English, abstracts without full-text availability, and unpublished articles were excluded. Publications solely about pain medicine, ethics, withdrawal of life support, and the elderly were also excluded. This included exclusion of diseases not typically encountered in childhood, such as dementia, amyotrophic lateral sclerosis, Parkinson disease, and stroke. Of the publications found, 51 were considered to be pertinent to the scope of this review.

regarding palliative care. The remaining articles or textbook chapters reviewed palliative care within adult and child neurology, with 21 and 15 sources respectively. Twenty-two of the 51 articles were original research articles, whereas the remaining 29 were review articles, editorials, or position statements. Of the original research, there was one prospective observational study, 13 survey- or questionnairebased studies, and 8 retrospective analyses. No randomized controlled trials were retrieved.

Identification of Guidelines Regarding a Palliative Approach to the Care of Patients Living With Literature Overview. The 51 identified publications were published between 1991 and 2012 and originated from the United Neurologic Diseases Results

States, Canada, the United Kingdom, Germany, France, and Australia. There were 6 guidelines and/or position statements regarding palliative care in neurology, none of which referenced the palliative care needs of children with neurologic conditions. Nine articles reviewed neurologist’s education

Multiple statements have been made by the American Academy of Neurology regarding need for palliative care within neurology (Table 1).6,7 Bernat wrote, ‘‘Another principal responsibility of neurologists to dying patients is to permit death to occur in the most comfortable manner possible,

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achieved by artfully minimizing sources of patient discomfort, by carefully planning for the moment of dying, and by attending to the psychological and spiritual needs of the dying patient and family.’’7 The 1996 position statement has yet to be updated; however, there was more recently the creation of an American Academy of Neurology Pain and Palliative Care Section division. In 2004, the first edition of the Clinical Practice Guidelines for Quality Palliative Care was developed by the National Consensus Project to describe the core precepts and structures of clinical palliative care programs in the United States.8 The National Consensus Project was a product of 4 palliative care organizations, including the American Academy of Hospice and Palliative Medicine, Center to Advance Palliative Care, Hospice and Palliative Nurses Association, and the National Hospice and Palliative Care Organization. Two years later, the National Quality Forum adopted the Clinical Practice Guidelines, commenting that the guidelines set forth by the National Consensus Project reflect the goals of the Institute of Medicine’s 6 dimensions of quality care: safe, effective, timely, patient-centered, efficient, and equitable.9 The Clinical Practice Guidelines for Quality Palliative Care was more recently updated in 2009. Additionally in 2009, an American Academy of Neurology practice parameter was released regarding care of the patient with amyotrophic lateral sclerosis.10 This parameter examined the existing evidence base regarding multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with amyotrophic lateral sclerosis. Supporting studies included 2 Class I studies, 8 Class II studies, and 30 Class III studies. Ideas for future research were to study use of terminal symptom management, advanced directives, hospice, spiritual care, and effective communication. In Europe, there have been multiple position statements published emphasizing the importance of access to palliative care for neurology patients. The European Association for Palliative Care was established in 2008 to develop guidelines for the delivery of palliative care to people with neurologic disease.11 The Guideline Development Group convened by the National Council for Palliative Care and the British Society of Rehabilitation Medicine (in association with the Royal College of Physicians), also in 2008, has published practical advice for neurologists regarding how to care for patients with chronic illness.12

Identification of Articles Summarizing Palliative Care Education for the Neurologist Table 2 summarizes the relevant articles regarding education of the neurologist and palliative care. In 1999, Carver et al13 surveyed 600 neuro-oncologists, amyotrophic lateral sclerosis specialists, and neurologists to assess ethical and legal questions in end-of-life care regarding withdrawal/withholding of life-sustaining measures, physician-assisted suicide and euthanasia, and advance directives and health care proxies. A second American Academy of Neurology survey that same year by

Galer found that only 30% of neurologists felt adequately trained to diagnose pain disorders, 20% felt adequate to treat them, and 90% of neurologists stated that more pain medicine education was needed for resident training.14 In 2004, Borasio and Voltz16 translated the Carver et al13 American Academy of Neurology survey and sent it to all medical directors of neurologic departments in Germany. Almost half of the respondents believe that their training in end-of-life care was insufficient, and greater than 90% expressed interest in education programs on palliative care. Despite the fact that the vast majority of the neurologists regarded advance directives as helpful, less than 5% of their patients had advance directives in place.16 Research also exists regarding the teaching of palliative care topics in neurology residency training. Neurology programs lag behind internal medicine and family medicine regarding consistently educating their residents in palliative care,17 despite its required inclusion by the Accreditation Council for Graduate Medical Education.22 Additionally, neurology ranks in the lowest quartile for amount of attention devoted to end-of-life care in textbooks compared to other medical subspecialties, with minimal to no content on palliative symptom control, ethics, and care of the imminently dying patient,15 ranking along with surgery, pulmonary, and infectious disease as one of the specialties with the lowest average percentages for using ‘‘end of life keywords.’’15 Authors noted that, even for Alzheimer disease, there was a greater amount of helpful end-of-life information in family and primary care medicine texts (average helpful content score, 50.8%), than in neurology texts (average helpful content score, 15.4%).15 A more recent study found that two-thirds of neurology residents agree, however, that even monthly palliative care clinical case discussions could help them acquire basic skills regarding end-of-life care and provide emotional support when working with dying patients.20

Identification of Articles Regarding Palliative Care Within Adult and Child Neurology and Its Subspecialties Regarding the spectrum of palliative care in general neurology (ie, not restricted to child neurology), there were 18 relevant articles pertinent to this review, as well as 3 textbooks (Tables 3 and 4). The majority of articles were review articles, with limited other study types, including 3 retrospective chart reviews and one cross sectional survey.2,21,31,57 The 3 retrospective studies all examined care of the neuro-oncology patient,21,25,29,57 which concluded that a relative paucity of research exists examining the palliative care needs of people with neurooncologic disease.21,31 Regarding articles restricted to child neurology, there were 13 relevant articles pertinent to this review and 1 textbook. The majority of articles were retrospective cohort studies or review articles. The only prospective study identified was a 1991 article that used a standardized form given to 45 families of children with life-threatening illnesses over a 10-year period in Britain. The majority of children were intellectually impaired and faced a steady, slow, decline to death without prospect of curative treatment. The authors separated the children’s

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Table 2. Identification of Articles Summarizing Education of the Neurologist Regarding Palliative Care. Title

Carver et al13

End-of-life care: a survey of U.S. United States neurologists’ attitudes, behavior, and knowledge

1999

Galer et al14

Pain medicine education among American neurologists: a need for improvement An evaluation of the end-of-life care content in leading neurology textbooks

United States

1999

United States

2000

Borasio et al15

Attitudes towards patient care at the end of life: a survey of directors of neurological departments

United States

2004

Schuh et al16

Neurology resident learning in an United States end-of-life/palliative care course

2007

United States Weissman et al44 Outcomes from a national multispecialty palliative care curriculum development project Schuh et al45 United States Education research: neurology residency training in the new millennium

2007

Khot et al19

Coping with death and dying on a United States neurology inpatient service: death rounds as an educational initiative for residents

2011

Gofton et al21

Identifying the palliative care needs United States of patients living with cerebral tumors and metastases

2012

Rabow et al18

Origin

Year Research Published Design

Author

2009

What this study adds

Cross-sectional Survey of AAN members; suggested there survey is a need for improved education of neurologists regarding knowledge of basic palliative care principles Cross-sectional Survey of AAN members; concluded survey neurologists need improved education in diagnosis and management of pain Descriptive Review of 50 major medical textbooks; study displayed neurology as ranking in the lowest quartile in coverage of palliative care and end-of-life concerns Cross-sectional Survey of neurology department directors survey in Germany; again suggested a need for improved education of neurologists regarding knowledge of basic palliative care principles Survey þ Surveyed neurology residency program Intervention directors; found that less than half of programs offer any didactics regarding palliative care or end-of-life care Cross-sectional A standardized palliative care curriculum survey can improve resident education in palliative care Cross-sectional Creation of an palliative care course was survey successful in helping neurology residents improve knowledge regarding palliative care Cross-sectional Neurology residents found monthly 1 survey hour clinical case discussions helpful in teaching principles of end-of-life care and in providing emotional support regarding caring for dying neurology inpatients Retrospective Described symptom prevalence and analysis whether patients had proxies, hospice discussions, or DNRs in place; concluded adult brain tumor patients warrant increased palliative care usage

Abbreviations: AAN, American Academy of Neurology; DNRs, do-not-resuscitate orders.

illnesses into 6 groups, the largest being degenerative disorders, and others including brain tumors and peripheral nervous system disorders.39 The majority of child neurology articles concentrated on specific subspecialties, the most common being that of peripheral nervous system disorders. These patients account for a large percentage of pediatric palliative care consultations and children’s hospices.53 The majority of these articles addressed medical management at end of life, rather than from time of diagnosis.40,43,50 Another common subspecialty retrieved was that of pediatric neurodegenerative diseases.41,42,52 Other studies retrieved examining the use of palliative care in child neurology included a study of children who had died of brain tumors, 49 and a compilation of short articles regarding palliative care for pediatric neurology patients, including review of

pain in children with neuro-irritability and difficult-to-treat epilepsy.51 The pediatric palliative care textbook reviewed included 1 chapter devoted exclusively to palliative care in neurologic illnesses, and a second chapter, to neuromuscular diseases.55

Discussion Definitions and Background of Palliative Care Palliative Care is defined by the World Health Organization as ‘‘the active total care of patients whose disease is not responsive to curative treatment. Control of pain, other symptoms, and of psychological, social, and spiritual problems is paramount. The goal of palliative care is the achievement of the

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Palliative therapy in neurology

The neurologist and the dying patient

Palliative therapy in the terminal stage of neurological disease

Palliative care in patients with primary brain tumors

Palliative care of patients with primary malignant brain tumour: case review of service use and support provided

Palliative care in specific neurologic conditions (AAN Continuum Edition) Integrating palliative and neurological critical care

Voltz and Borasio23

Bernat et al7

Voltz24

Taillibert et al25

Faithfull et al26

Kurent27

Palliative care beyond cancer care for all at the end of life United Kingdom

Palliative care needs of patients with neurologic or neurosurgical conditions

Murray and Sheikh1

Chahinea et al30

United States

From diagnosis to death: exploring the interface between United Kingdom neurology, rehabilitation and palliative care in managing people with long-term neurological conditions

Turner-Stokes et al2

France

Palliative care in patients with brain metastases

United States

United States

United Kingdom

France

United States

United States

Germany

Origin

Taillibert and Delattre29

Owens and Flom28

Title

Author

Table 3. Identification of Articles and Textbooks Reviewing Use Palliative Care in Neurology.

2008

2008

2007

2005

2005

2005

2005

2004

1997

1996

1994

What this study adds

(continued)

Overview of treatments for common symptoms occurring in late-stage neurologic disease (ie, restlessness, drowsiness, death-rattle, shortness of breath, pain, seizures, raised intracranial pressure, thirst/hunger) Editorial Reinforced that neurologists have a duty to provide adequate palliative care and should encourage improved physician education on palliative care Review, Reviewed current treatment recommendations for recommendations symptoms arising in end-stage neurologic disease, as well as common ethical dilemmas at end of life Review Distressing symptoms of patients with primary brain tumors include pain, epilepsy, side effects of antiepileptic drugs; thromboembolic disease resulting in physical disability, cognitive disease and psychological distress Retrospective case Many services within the community exist to support review patients with primary malignant brain tumors, but only a small proportion receive inpatient hospice, while many others are admitted to hospitals at end of life Review Reviewed palliative care in ALS, persistent vegetative state, Alzheimer disease, and other adult neurologic conditions Editorial Reviewed palliative care principles for critical care nurses working in neurologic intensive care units Review Reviewed common side effects of brain metastases; concludes that palliative management of brain metastasis requires a multidisciplinary approach Cross-sectional sur- Questionnaires mailed to consultants in neurology, vey, review rehabilitation, and palliative medicine; found all need improved education in palliative care of neurologic patients Review Neurologic/neurosurgical diseases were the second most common subpopulation consulted on by a palliative medicine service after cancer; however, there is no consensus regarding when to initiate palliative care services for neurology patients Retrospective Neurologic/neurosurgical diseases were the second most review common subpopulation seen by the palliative medicine service after cancer; reasons for consultation included comfort measures, pain management, and hospice candidacy

Review

Year published Research design

1733

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Supportive care in neurooncology

‘‘The choice for death’’ and neurology

Symptomatic management and palliative care in neurodegenerative disease

Identifying the palliative care needs of patients living with cerebral tumors and metastases

Palliative care in neurology

Palliative neurology

Palliative care in neurological disease

Pace et al33

Durnova´34

Bede et al35

Gofton et al21

Voltz et al36

Maddocks et al37

Byrne et al38

United Kingdom

Europe/United States United Kingdom

United States

Ireland

Austria

Italy

United States

2009

2006

2004

2012

2011

2010

2010

2010

2009

2008

2008

Textbook

Textbook

Textbook

Retrospective review

Review

Editorial

Review

Editorial

Review

Retrospective review

Review

Year published Research design

Abbreviations: AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; LTNC, long-term neurologic conditions.

Shaw32

A palliative approach to neurological care: a literature review Special report: bringing palliative care to neurology

Gofton et al3 Canada

United States

Psychosocial and supportive-care needs in high-grade glioma

Catt et al31

Origin United Kingdom

Title

Turner-Stokes et al12 Long-term neurologic conditions: management at the interface between neurology, rehabilitation and palliative care

Author

Table 3. (continued)

Explored the interaction between neurology, rehabilitation and palliative care services, and how they might best work together to provide long-term support for patients and caregivers; included a checklist for the clinician caring for the patient with an LTNC Having a brain tumor is associated with a decreased likelihood of receiving palliative care compared to other cancer subtypes; there exists a lack of research on the palliative care needs of people with high-grade glioma Literature review retrieving 53 articles regarding palliative care in adult neurology General neurologists were interviewed about how they can integrate palliative care into their practices While there has been progress in medical management, there remains a need for improved education regarding supportive care and end-of-life issues in brain tumor patients Reviewed controversial issues in palliative neurology including ‘‘choices for death,’’ euthanasia, and doctorassisted suicide Examined 4 neurodegenerative diseases (ALS, Huntington, dementia, and parkinsonian disorders); also provided a review of patient support groups Documented the physical symptom burden (ie, motor deficits and cognitive changes) of adults with primary brain tumors and cerebral metastases Reviewed multiple adult neurologic disorders and the application of palliative care practices to these groups Reviewed dementia, stroke, Parkinson disease, ALS, Huntington disease, muscular dystrophies, and multiple sclerosis Reviewed Parkinson disease, motor neuron disease, multiple sclerosis, and Huntington disease

What this study adds

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Table 4. Identification of Articles Regarding Palliative Care Within Child Neurology and Its Subspecialties. Author

Title

Origin

Woolley et al39 Cornerstone care for families of children with life-threatening illness

United Kingdom

Hilton et al40

End of life care in Duchenne muscular United States dystrophy

Hunt and Burne41

Medical and nursing problems of children with neurodegenerative disease

Davies42

Living with dying: families coping with Canada a child who has a neurodegenerative genetic disorder The role of palliative care in advanced Australia muscular dystrophy and spinal muscular atrophy

Parker et al43

Steele44

Dawson45

United Kingdom

Canada Trajectory of certain death at an unknown time: children with neurodegenerative life-threatening illnesses. Australia Mapping the journey: family carers’ perceptions of issues related to end-stage care of individuals with muscular dystrophy or motor neurone disease

Year published Research design Findings 1991

1993

1995

1996

1999

2000

2003

Weidner46

Developing an interdisciplinary palliative care plan for the patient with muscular dystrophy

United States

2005

Graham and Robinson47

Integrating palliative care into chronic United States care for children with severe neurodevelopmental disabilities

2005

Prospective cohort

Families indicated they can be better helped and services more rationally used when a ‘‘cornerstone carer’’ exists to help coordinate care Review Reviewed patient preferences, quality-of-life issues, and treatment of symptoms regarding DMD at end of life Retrospective Described the demographics of cohort children with neurodegenerative disease admitted to a pediatric hospice Retrospective; Addressed the coping strategies and questionnaire support resources for patients with neurodegenerative genetic disorders Interviews The majority of families caring for children with terminal neurologic diseases are not referred to a palliative care service or have advanced directives Interviews Families of children with neurodegenerative diseases shared their experiences as their children approach end of life Interviews Determined the needs of carers during the final stages of caring for someone with muscular dystrophy or motor neuron disease; results suggest that palliative care has much to offer, but this could currently be underrecognized Review The early introduction of a palliative team allows for a relationship of trust to develop between the doctor and the family of a child with MD Review Barriers to effective EOL care in children include professional attitudes, reconfigured parental roles, and the uncertainty of prognosis. Review Reviews management of seizures, agitation, and spasticity

Wusthoff et al48 Management of common neurologic symptoms in pediatric palliative care: seizures, agitation, and spasticity Zelcer et al49 Palliative care of children with brain tumors: a parental perspective

United States

2007

Canada

2010

Interviews

Arias et al50

United States

2011

Retrospective

Palliative care services in families of males with Duchenne muscular dystrophy

Parents of children who died from their brain tumors shared common challenges during the neurologic deterioration of their child, including balancing competing responsibilities and talking to their child about death The majority of families caring for children with DMD were not referred to a palliative care service or have advanced directives (continued)

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Table 4. (continued) Author

Title

Year published Research design Findings

Origin

Friedrichsdorf 51 Palliative care for children in paediatric neurology

Germany

2011

Malcolm et al52

Challenging symptom profiles of life- United Kingdom limiting conditions in children: a survey of care professionals and families

2011

Fraser et al53

Hospice provision and usage amongst United Kingdom young people with neuromuscular disease in the United Kingdom

2011

Fraser et al54

A cohort study of children and young United Kingdom people with progressive neuromuscular disorders: clinical and demographic profiles and changing patterns of referral for palliative care Oxford Textbook of Palliative Care United States for Children.

2012

Goldman et al55

Swaiman et al56 Swaiman’s Pediatric Neurology, Fifth United States edition

2006

2012

Review

Reviewed approach to pain in children with neuro-irritability, and management of refractory epilepsy and dysautonomia Survey; 43 care professionals and 16 families Retrospective prioritized mucopolysaccharidosis, neuronal ceroid lipofuscinosis, and leukodystrophy as neurologic diseases having difficult to manage symptoms, and identified behavioral difficulties, visual impairments and communication impairments as those symptoms being most difficult to manage and having the most impact on quality of life Questionnaire; Described demographics of children retrospective with progressive neuromuscular disease referred to a pediatric hospice Retrospective Supported the need for specialist cohort palliative care services for patients with neuromuscular disorders

Provided 1 chapter devoted to Pediatric neurology and a second chapter to palliative care neuromuscular disease textbook Child neurology Provided 1 chapter devoted to pain textbook and palliative care

Abbreviations: AAN, American Academy of Neurology; DMD, Duchenne muscular dystrophy; EOL, end of life; MD, muscular dystrophy.

best possible quality of life for patients and their families.’’58 Neurology guidelines emphasize the importance of expertise in palliative care among neurologists.6 The definitions set forth by the World Health Organization and American Academy of Neurology express that palliative care is a vital component to the care of any patient with a potentially life-threatening neurologic condition. Further, guidelines emphasize that palliative care be offered at the time of diagnosis, rather than when curative options are exhausted.59 There is minimal literature, however, devoted to palliative care use in neurology before the last 6 months of life.3 Advocating for palliative care specifically at time of diagnosis allows physicians to develop stronger and more trusting relationships with patients in anticipation of end-of-life care.46 Early initiation of palliative care has already been shown in randomized controlled trials to result in better patient outcomes, improving both quality of life and symptom management.60 In pediatrics, families should also be introduced to palliative care at the time of diagnosis. Palliative care should be introduced not only when a child receives a diagnosis where cure may not be possible but also when symptom management is

difficult or if there is significant suffering. The goal of pediatric palliative care is to comprehensively address the physical, psychosocial, and spiritual needs of the child and family.59 Whereas there exist multiple position statements regarding necessity for use of palliative care in adult neurology, there are no published position statements as yet exclusively directed toward the role of palliative care in child neurology (Table 1).

Is There a Need for Increased Palliative Care Education Among Neurologists? Patients affected by devastating neurologic diseases and injuries suffer tremendously physically, emotionally, and existentially. These patients, along with their families, face the brutal reality of worsening physical and cognitive function, an anticipated shortened life span, and an uncertain future. The results of Table 2 support that many neurologists do not have extensive familiarity in theories of palliative care. The literature demonstrates lack of didactics and clinical rotations in palliative care, reinforcing the need for increased palliative care education among neurologists. Some studies have started to address this by creating educational initiatives to enhance

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neurologists’ care of terminally ill patients.18,19 At the same time, with neurology textbooks ranking in the lowest quartile of medical specialties for having content on palliative care, this will need continued attention.

Previous Publications Regarding the Practice of Palliative Care in Child Neurology and General Neurology as It Might Pertain to Child Neurology The need for neurologists to provide palliative care will only continue to increase as the burden of chronic and neurodegenerative diseases increases in the general population. The American Academy of Neurology has already recognized this, devoting a continuum edition specifically to palliative care, concentrating again, mainly on chronic neurodegenerative conditions in adults. Of the articles found reviewing palliative care in general neurology, the majority were commentaries or review articles (Table 3), including review of common symptoms and needs at the time of diagnosis of a life-threatening illness as well as at end of life. One subspecialty that was repeatedly reviewed in the adult literature was neurooncology. With the advancements in treatments for patients with previously life-threatening brain tumors, patients are living with increasing long-term morbidity. Increasing morbidity is seen in other neurologic disorders as well, including stroke, dementia, and amyotrophic lateral sclerosis. There is an increasing need for adequate pain control in these groups, as well as for identifying how and when transition to hospice and ‘‘comfort care’’ might be appropriate. As neurologists, it should be our obligation to help patients and families with the difficult decisions that arise regarding treatment and life planning. The great deal of uncertainty and difficulty with prognostication that exists with neurologic diseases requires physicians to have particular expertise in anticipating such difficult questions about long-term goals of care. There is also minimal literature examining palliative care within child neurology. It is therefore not possible to document the prevalence of symptoms in children living with different life-threatening neurologic illnesses. Similar to the adult studies, many of the articles in the palliative care literature regarding children were those patients with neurodegenerative diseases. On the other hand, pediatric brain tumors, which comprise 25% to 30% of all childhood cancers and are the leading cause of death by cancer in children under 15, had perhaps fewer studies than would be expected.61 More research needs to be done in many of the child neurology subspecialties. For example, the majority of deaths in children occur below age 1 year, mainly in the first month of life.62 Neonatal neurology patients often have a very uncertain longterm prognosis, leading to difficult decisions occurring on a fairly common basis. Similar to adults, these children are commonly living longer, with the advancements of neuroimaging techniques, new antiepileptic treatments, and use of hypothermia. The highest proportion of seizures in children occurs in infancy; the highest proportion of stroke in children, also in

newborns. The families of patients with hypoxic ischemic encephalopathy, or intraventricular hemorrhage and periventricular leukomalacia, often have many questions and concerns that can go unanswered. It is important to devote more research to how palliative care can be better delivered in our neonatal intensive care units. Future studies should also address how to improve symptom management, relieve psychological and spiritual suffering, and deliver end-of-life care, in the child or adolescent with the lifelimiting neurologic illness in the pediatric intensive care unit. These commonly have the sickest of child neurology patients, including patients with status epilepticus, traumatic brain injury, ischemic or hemorrhagic stroke, increased intracranial pressure, and brain death. There was no literature retrieved regarding children with such common ethical dilemmas such as how to approach the family of these patients before or after being unresponsive to medical or surgical intervention. It is unclear what parents might believe are the most and least effective ways in which to begin goals of care discussions. Beyond providing diagnosis and treatments, neurologists and child neurologists are responsible for managing symptoms and optimizing quality of life. Limitations of this review included small sample sizes given the relative rarity of adults and children with life-threatening neurologic diseases. Our retrieved literature revealed observational studies and review articles but almost no prospective studies or randomized controlled trials (Table 4). This is likely because many of these illnesses are rare illnesses with low prevalence in the community. The lack of prospective trials and reliance on editorials and review articles reinforce the lack of evidence based medicine in this area. A randomized controlled trial, however, similar to that conducted by Temel, where patients are randomized to standard medical care versus standard medical care plus additional palliative care consultation, should be considered for the future. Although more literature in this review was from abroad rather than from the United States, recognition of the importance of palliative care in the United States will continue to increase as additional position statements, observational studies, and randomized controlled trials are developed. The fact that the majority of articles retrieved were somewhat more recently published since the start of the millennium reinforces the growing interest and awareness of physicians. Author Contributions AD and JS were responsible for study concept and design, critical revision of the manuscript for important intellectual content, and study supervision. AD and AM were responsible for data acquisition, analysis, and interpretation.

Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding The authors received no financial support for the research, authorship, and/or publication of this article.

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