Semin Neurol
DOI: 10.1055/s-0044-1787809
Review Article

End-of-Life and Hospice Care in Neurologic Diseases

Claudia Z. Chou
1   Division of Community Internal Medicine, Geriatrics, and Palliative Care, Mayo Clinic, Rochester, Minnesota
,
Elyse A. Everett
2   Department of Medicine, Washington University in St. Louis, St. Louis, Missouri
3   Department of Neurology, Washington University in St. Louis, St. Louis, Missouri
,
Jessica McFarlin
4   Department of Neurology, University of Kentucky, Lexington, Kentucky
,
Usha Ramanathan
5   Division of Neurology, University of Toronto, Toronto, Ontario, Canada
› Author Affiliations

Abstract

The care of a patient with neurologic disease at end-of-life requires expertise in addressing advance care planning, hospice, symptom management, and caregiver support. Neurologists caring for patients with advanced neurologic disease often identify changes in disease trajectory, functional status, or goals of care that prompt discussions of advance care planning and hospice. Patients nearing end-of-life may develop symptoms such as dyspnea, secretions, delirium, pain, and seizures. Neurologists may be the primary clinicians managing these symptoms, particularly in the hospitalized patient, though they may also lend their expertise to non-neurologists about expected disease trajectories and symptoms in advanced neurologic disease. This article aims to help neurologists guide patients and caregivers through the end-of-life process by focusing on general knowledge that can be applied across diseases as well as specific considerations in severe stroke and traumatic brain injury, amyotrophic lateral sclerosis, Parkinson's disease, and dementia.



Publication History

Article published online:
25 June 2024

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  • References

  • 1 Robinson MT, Holloway RG. Palliative care in neurology. Mayo Clin Proc 2017; 92 (10) 1592-1601
  • 2 Creutzfeldt CJ, Gooley T, Walker M. Are neurology residents prepared to deal with dying patients?. Arch Neurol 2009; 66 (11) 1427-1428
  • 3 Cook T, Arnold R, Jeong K, Childers J. Opinion & special article: next steps in palliative care education for neurology residents. Neurology 2021; 97 (24) 1134-1137
  • 4 Mehta AK, Najjar S, May N, Shah B, Blackhall L. A needs assessment of palliative care education among the United States adult neurology residency programs. J Palliat Med 2018; 21 (10) 1448-1457
  • 5 Sudore RL, Lum HD, You JJ. et al. Defining advance care planning for adults: a consensus definition from a multidisciplinary Delphi panel. J Pain Symptom Manage 2017; 53 (05) 821-832.e1
  • 6 National POLST. Accessed November 15, 2023 at: https://polst.org/
  • 7 Tilburgs B, Vernooij-Dassen M, Koopmans R, Weidema M, Perry M, Engels Y. The importance of trust-based relations and a holistic approach in advance care planning with people with dementia in primary care: a qualitative study. BMC Geriatr 2018; 18 (01) 184
  • 8 Taylor LP, Besbris JM, Graf WD, Rubin MA, Cruz-Flores S, Epstein LG. Ethics, Law, and Humanities Committee, a joint committee of the American Academy of Neurology, American Neurological Association, and Child Neurology Society. Clinical guidance in neuropalliative care: an AAN position statement. Neurology 2022; 98 (10) 409-416
  • 9 Llewellyn H, Neerkin J, Thorne L. et al. Social and structural conditions for the avoidance of advance care planning in neuro-oncology: a qualitative study. BMJ Open 2018; 8 (01) e019057
  • 10 Kluger BM, Miyasaki JM. Key concepts and opportunities. Handb Clin Neurol 2022; 190: 3-15
  • 11 Macchi ZA, Lum HD. Advance care planning in neurologic illness. Handb Clin Neurol 2022; 190: 129-148
  • 12 Weisbrod N, Quill TE. Neuropalliative Care: A Guide to Improving the Lives of Patients and Families Affected by Neurologic Disease. Creutzfeldt CJ, Kluger BM, Holloway RG. eds. 2019. Springer;
  • 13 Creutzfeldt CJ, Kluger BM, Holloway RG. eds. Neuropalliative Care: A Guide to Improving the Lives of Patients and Families Affected by Neurologic Disease Neuropalliative Care: A Guide to Improving the Lives of Patients and Families Affected by Neurologic Disease. 1st ed.. Chap. 15. Cham, Switzerland: Springer; 2018: 205-218
  • 14 Murray SA, Kendall M, Sheikh A. et al, eds. End of Life Care in Long Term Neurological Conditions: A Framework for Implementation. 2008 ; National End of Life Care Programme
  • 15 Hoff T, Trovato K, Kitsakos A. Hospice satisfaction among patients, family, and caregivers: a systematic review of the literature. Am J Hosp Palliat Care 2024; 41 (06) 691-705
  • 16 Turner R, Rosielle DA. Fast Facts and Concepts #82: Medicare Hospice Benefit - Part 1: Eligibility and Treatment Team. Accessed November 13, 2023 at: https://www.mypcnow.org
  • 17 Hospice. 28 Sept 2023 . Accessed December 19, 2023 at: https://www.cms.gov/medicare/payment/fee-for-service-providers/hospice
  • 18 NHPCO Facts and Figures 2023 Edition. Accessed June 3, 2024 at: https://www.nhpco.org/wp-content/uploads/NHPCO-Facts-Figures-2023.pdf
  • 19 Risco JR, Kelly AG, Holloway RG. Prognostication in neurology. Handb Clin Neurol 2022; 190: 175-193
  • 20 Local Coverage Determination (LCD): Hospice Determining Terminal Status. Accessed November 15, 2023 at: https://www.cms.gov/medicare-coverage-database/view/lcd.aspx?lcdid=34538&ver=16&bc=0
  • 21 McCluskey L, Houseman G. Medicare hospice referral criteria for patients with amyotrophic lateral sclerosis: a need for improvement. J Palliat Med 2004; 7 (01) 47-53
  • 22 Luchins DJ, Hanrahan P, Murphy K. Criteria for enrolling dementia patients in hospice. J Am Geriatr Soc 1997; 45 (09) 1054-1059
  • 23 Salpeter SR, Luo EJ, Malter DS, Stuart B. Systematic review of noncancer presentations with a median survival of 6 months or less. Am J Med 2012; 125 (05) 512.e1-512.e6
  • 24 Lynn J, Schuster JL, Kabcenell A. Improving Care for the EOL: A Sourcebook for Health Care Managers and Clinicians. 1st ed. 2000. Oxford:: Oxford University Press;
  • 25 Downar J, Goldman R, Pinto R, Englesakis M, Adhikari NK. The “surprise question” for predicting death in seriously ill patients: a systematic review and meta-analysis. CMAJ 2017; 189 (13) E484-E493
  • 26 Glover TL, Kluger BM. Palliative medicine and end-of-life care. Handb Clin Neurol 2019; 167: 545-561
  • 27 Daly FN, Ramanathan U. End-of-life and hospice care for neurologic illness. Handb Clin Neurol 2022; 190: 195-215
  • 28 Bodtke S, Ligon K. Hospice and Palliative Medicine Handbook: A Clinical Guide. Amazon Digital Services; 2016
  • 29 Moens K, Higginson IJ, Harding R. EURO IMPACT. Are there differences in the prevalence of palliative care-related problems in people living with advanced cancer and eight non-cancer conditions? A systematic review. J Pain Symptom Manage 2014; 48 (04) 660-677
  • 30 Abernethy AP, McDonald CF, Frith PA. et al. Effect of palliative oxygen versus room air in relief of breathlessness in patients with refractory dyspnoea: a double-blind, randomised controlled trial. Lancet 2010; 376 (9743) 784-793
  • 31 Galbraith S, Fagan P, Perkins P, Lynch A, Booth S. Does the use of a handheld fan improve chronic dyspnea? A randomized, controlled, crossover trial. J Pain Symptom Manage 2010; 39 (05) 831-838
  • 32 Kamal AH, Maguire JM, Wheeler JL, Currow DC, Abernethy AP. Dyspnea review for the palliative care professional: treatment goals and therapeutic options. J Palliat Med 2012; 15 (01) 106-114
  • 33 Mahler DA, O'Donnell DE. Recent advances in dyspnea. Chest 2015; 147 (01) 232-241
  • 34 Lokker ME, van Zuylen L, van der Rijt CC, van der Heide A. Prevalence, impact, and treatment of death rattle: a systematic review. J Pain Symptom Manage 2014; 47 (01) 105-122
  • 35 Wee BL, Coleman PG, Hillier R, Holgate SH. The sound of death rattle II: how do relatives interpret the sound?. Palliat Med 2006; 20 (03) 177-181
  • 36 Wee B, Hillier R. Interventions for noisy breathing in patients near to death. Cochrane Database Syst Rev 2008; 2008 (01) CD005177
  • 37 Prommer E. Anticholinergics in palliative medicine: an update. Am J Hosp Palliat Care 2013; 30 (05) 490-498
  • 38 Buss MK, Vanderwerker LC, Inouye SK, Zhang B, Block SD, Prigerson HG. Associations between caregiver-perceived delirium in patients with cancer and generalized anxiety in their caregivers. J Palliat Med 2007; 10 (05) 1083-1092
  • 39 Breitbart W, Alici Y. Agitation and delirium at the end of life: “We couldn't manage him”. JAMA 2008; 300 (24) 2898-2910 , E1
  • 40 American Psychiatric Association. Practice guideline for the treatment of patients with delirium. Am J Psychiatry 1999; 156 (05) 1-20
  • 41 Inouye SK, Zhang Y, Jones RN, Kiely DK, Yang F, Marcantonio ER. Risk factors for delirium at discharge: development and validation of a predictive model. Arch Intern Med 2007; 167 (13) 1406-1413
  • 42 Sykes N, Thorns A. The use of opioids and sedatives at the end of life. Lancet Oncol 2003; 4 (05) 312-318
  • 43 Von Gunten C. Fast Facts and Concepts #8: Morphine and Hastened Death. Accessed May 4, 2023 at: https://www.mypcnow.org
  • 44 Portenoy RK, Sibirceva U, Smout R. et al. Opioid use and survival at the end of life: a survey of a hospice population. J Pain Symptom Manage 2006; 32 (06) 532-540
  • 45 Mercadante S, Portenoy RK. Opioid poorly-responsive cancer pain. Part 3. Clinical strategies to improve opioid responsiveness. J Pain Symptom Manage 2001; 21 (04) 338-354
  • 46 Albert RH. End-of-life care: managing common symptoms. Am Fam Physician 2017; 95 (06) 356-361
  • 47 Quijada E, Billings JA, Bukowy EA. Fast Facts #60: Pharmacologic Management of Delirium Update on Newer Agents. Accessed November 15, 2023 at: https://www.mypcnow.org
  • 48 Connelly J, Weissman DE. Fast Facts and Concepts #229: Seizure Management in the Dying Patient. 2010 . Accessed November 13, 2023 at: https://www.mypcnow.org
  • 49 Tradounsky G. Seizures in palliative care. Can Fam Physician 2013; 59 (09) 951-955 , e401–e405
  • 50 Goostrey K, Muehlschlegel S. Prognostication and shared decision making in neurocritical care. BMJ 2022; 377: e060154
  • 51 Teno JM, Clarridge BR, Casey V. et al. Family perspectives on end-of-life care at the last place of care. JAMA 2004; 291 (01) 88-93
  • 52 Bender MA, Hurd C, Solvang N, Colagrossi K, Matsuwaka D, Curtis JR. A new generation of comfort care order sets: aligning protocols with current principles. J Palliat Med 2017; 20 (09) 922-929
  • 53 Creutzfeldt CJ, Longstreth WT, Holloway RG. Predicting decline and survival in severe acute brain injury: the fourth trajectory. BMJ 2015; 351: h3904
  • 54 Everett EA, Pedowitz E, Maiser S. et al. Top ten tips palliative care clinicians should know about amyotrophic lateral sclerosis. J Palliat Med 2020; 23 (06) 842-847
  • 55 Moens K, Houttekier D, Van den Block L. et al. Place of death of people living with Parkinson's disease: a population-level study in 11 countries. BMC Palliat Care 2015; 14: 28
  • 56 Safarpour D, Thibault DP, DeSanto CL. et al. Nursing home and end-of-life care in Parkinson disease. Neurology 2015; 85 (05) 413-419
  • 57 Akbar U, McQueen RB, Bemski J. et al. Prognostic predictors relevant to end-of-life palliative care in Parkinson's disease and related disorders: a systematic review. J Neurol Neurosurg Psychiatry 2021; 92 (06) 629-636
  • 58 Katz M, Goto Y, Kluger BM. et al. Top ten tips palliative care clinicians should know about Parkinson's disease and related disorders. J Palliat Med 2018; 21 (10) 1507-1517
  • 59 Varanese S, Birnbaum Z, Rossi R, Di Rocco A. Treatment of advanced Parkinson's disease. Parkinsons Dis 2011; 2010: 480260
  • 60 Chang A, Fox SH. Psychosis in Parkinson's disease: epidemiology, pathophysiology, and management. Drugs 2016; 76 (11) 1093-1118
  • 61 Tse W, Frisina PG, Hälbig TD. et al. The effects of withdrawal of dopaminergic medication in nursing home patients with advanced parkinsonism. J Am Med Dir Assoc 2008; 9 (09) 670-675
  • 62 Koschel J, Ray Chaudhuri K, Tönges L, Thiel M, Raeder V, Jost WH. Implications of dopaminergic medication withdrawal in Parkinson's disease. J Neural Transm (Vienna) 2022; 129 (09) 1169-1178
  • 63 Friedman JH, Feinberg SS, Feldman RG. A neuroleptic malignantlike syndrome due to levodopa therapy withdrawal. JAMA 1985; 254 (19) 2792-2795
  • 64 Serrano-Dueñas M. Neuroleptic malignant syndrome-like, or–dopaminergic malignant syndrome–due to levodopa therapy withdrawal. Clinical features in 11 patients. Parkinsonism Relat Disord 2003; 9 (03) 175-178
  • 65 Toru M, Matsuda O, Makiguchi K, Sugano K. Neuroleptic malignant syndrome-like state following a withdrawal of antiparkinsonian drugs. J Nerv Ment Dis 1981; 169 (05) 324-327
  • 66 Yang HJ, Ehm G, Kim YE. et al. Liquid levodopa-carbidopa in advanced Parkinson's disease with motor complications. J Neurol Sci 2017; 377: 6-11
  • 67 Cooper SD, Ismail HA, Frank C. Case report: successful use of rectally administered levodopa-carbidopa. Can Fam Physician 2001; 47: 112-113
  • 68 Eisler T, Eng N, Plotkin C, Calne DB. Absorption of levodopa after rectal administration. Neurology 1981; 31 (02) 215-217
  • 69 Vogelzang JM, Luinstra M, Rutgers AW. Effect of rectal levodopa administration: a case report. Case Rep Neurol 2015; 7 (03) 209-212
  • 70 Kim W, Watt CL, Enright P, Sikora L, Zwicker J. Management of motor symptoms for patients with advanced Parkinson's disease without safe oral access: a scoping review. J Palliat Med 2023; 26 (01) 131-141
  • 71 Hasson F, Kernohan WG, McLaughlin M. et al. An exploration into the palliative and end-of-life experiences of carers of people with Parkinson's disease. Palliat Med 2010; 24 (07) 731-736
  • 72 Armstrong MJ, Alliance S, Corsentino P, Maixner SM, Paulson HL, Taylor A. Caregiver-reported barriers to quality end-of-life care in dementia with Lewy bodies: a qualitative analysis. Am J Hosp Palliat Care 2020; 37 (09) 728-737
  • 73 Kamal AH, Bull JH, Swetz KM, Wolf SP, Shanafelt TD, Myers ER. Future of the palliative care workforce: preview to an impending crisis. Am J Med 2017; 130 (02) 113-114
  • 74 Ersek M, Herr K, Neradilek MB, Buck HG, Black B. Comparing the psychometric properties of the Checklist of Nonverbal Pain Behaviors (CNPI) and the Pain Assessment in Advanced Dementia (PAIN-AD) instruments. Pain Med 2010; 11 (03) 395-404
  • 75 Chiong W, Tsou AY, Simmons Z, Bonnie RJ, Russell JA. Ethics, Law, and Humanities Committee (a joint committee of the American Academy of Neurology, American Neurological Association, and Child Neurology Society). Ethical considerations in dementia diagnosis and care: AAN position statement. Neurology 2021; 97 (02) 80-89
  • 76 Sampson EL, Candy B, Jones L. Enteral tube feeding for older people with advanced dementia. Cochrane Database Syst Rev 2009; 2009 (02) CD007209
  • 77 Weissman D. Fast Facts and Concepts #84: Swallow Studies,. Tube Feeding and the Death Spiral. Accessed November 15, 2023 at: https://www.mypcnow.org
  • 78 Hertler C, Eisele G, Gramatzki D. et al. End-of-life care for glioma patients; the caregivers' perspective. J Neurooncol 2020; 147 (03) 663-669
  • 79 Costa-Requena G, Espinosa Val MC, Cristòfol R. Caregiver burden in end-of-life care: advanced cancer and final stage of dementia. Palliat Support Care 2015; 13 (03) 583-589
  • 80 Durepos P, Wickson-Griffiths A, Hazzan AA. et al. Assessing palliative care content in dementia care guidelines: a systematic review. J Pain Symptom Manage 2017; 53 (04) 804-813