Five Questions to Help Prompt End-of-Life Planning in Neuromuscular Disease

Abstract

Patients with neuromuscular disease are living longer lives but continue to have significant and often unpredictable morbidity and mortality. End-of-life planning for these patients is thus an essential part of their medical care. This planning should include the following topics: health care surrogates, swallowing and nutrition, daytime respiratory support, and all aspects of when end of life is near. Adult-onset and early-onset diseases may require different approaches to these topics. All patients with neuromuscular disease will benefit from these discussions to best reach patient-centered goals. We present health care providers these five questions and explanations as a guide.

Keywords

neuromuscular - end of life - goals - planning - surrogates - conversation

Publikationsverlauf

Artikel online veröffentlicht:
19. Juli 2024

• References

• 1 Blair E, Langdon K, McIntyre S, Lawrence D, Watson L. Survival and mortality in cerebral palsy: observations to the sixth decade from a data linkage study of a total population register and National Death Index. BMC Neurol 2019; 19 (01) 111
• 2 Landfeldt E, Thompson R, Sejersen T, McMillan HJ, Kirschner J, Lochmüller H. Life expectancy at birth in Duchenne muscular dystrophy: a systematic review and meta-analysis. Eur J Epidemiol 2020; 35 (07) 643-653
• 3 Rose L, McKim D, Leasa D. et al. Trends in incidence, prevalence, and mortality of neuromuscular disease in Ontario, Canada: a population-based retrospective cohort study (2003-2014). PLoS One 2019; 14 (03) e0210574
• 4 D'Cruz RF, Kaltsakas G, Suh E-S, Hart N. Quality of life in patients with chronic respiratory failure on home mechanical ventilation. Eur Respir Rev 2023; 32 (168) 220237
• 5 Abbott D, Prescott H, Forbes K, Fraser J, Majumdar A. Men with Duchenne muscular dystrophy and end of life planning. Neuromuscul Disord 2017; 27 (01) 38-44
• 6 Martinez NP, Herrero MM, Rebollo CG, Repiso EM. How our amyotrophic lateral sclerosis patients die. Eur Respir J 2020; 56 (Suppl. 64) 3419
• 7 Ceriana P, Surbone S, Segagni D, Schreiber A, Carlucci A. Decision-making for tracheostomy in amyotrophic lateral sclerosis (ALS): a retrospective study. Amyotroph Lateral Scler Frontotemporal Degener 2017; 18 (7–8): 492-497
• 8 Kripke C. Adults with developmental disabilities: a comprehensive approach to medical care. Am Fam Physician 2018; 97 (10) 649-656
• 9 Finder JD, Birnkrant D, Carl J. et al; American Thoracic Society. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004; 170 (04) 456-465
• 10 Andersen PM, Abrahams S, Borasio GD. et al; EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)–revised report of an EFNS task force. Eur J Neurol 2012; 19 (03) 360-375
• 11 Kruser JM, Clapp JT, Arnold RM. Reconsidering the language of serious illness. JAMA 2023; 330 (07) 587-588
• 12 Childers JW, Back AL, Tulsky JA, Arnold RM. REMAP: a framework for goals of care conversations. J Oncol Pract 2017; 13 (10) e844-e850
• 13 Phillips J, Wendler D. Clarifying substituted judgement: the endorsed life approach. J Med Ethics 2015; 41 (09) 723-730
• 14 Katz AL, Webb SA. COMMITTEE ON BIOETHICS. Informed consent in decision-making in pediatric practice. Pediatrics 2016; 138 (02) e20161485
• 15 Francis L. Supported decision-making: the CRPD, non-discrimination, and strategies for recognizing persons' choices about their good. Journal of Philosophy of Disability. 2021; 1: 57-77
• 16 Zietlow K, Dubin L, Battles A, Vitale C. Guardianship: a medicolegal review for clinicians. J Am Geriatr Soc 2022; 70 (11) 3070-3079
• 17 Bantekas I, Stein MA, Anastasiou D. The convention on the rights of persons with disabilities: a commentary. Oxford Commentaries on Interna. Oxford: Oxford University Press; 2018
• 18 Stratton RJ, Ek AC, Engfer M. et al. Enteral nutritional support in prevention and treatment of pressure ulcers: a systematic review and meta-analysis. Ageing Res Rev 2005; 4 (03) 422-450
• 19 Schaible UE, Kaufmann SHE. Malnutrition and infection: complex mechanisms and global impacts. PLoS Med 2007; 4 (05) e115
• 20 Hsu YH, Chou MY, Chu CS. et al. Predictive effect of malnutrition on long-term clinical outcomes among older men: a prospectively observational cohort study. J Nutr Health Aging 2019; 23 (09) 876-882
• 21 Ngan OMY, Bergstresser SM, Sanip S, Emdadul Haque ATM, Chan HYL, Au DKS. Cultural considerations in forgoing enteral feeding: a comparison between the Hong Kong Chinese, North American, and Malaysian Islamic patients with advanced dementia at the end-of-life. Developing World Bioeth 2020; 20 (02) 105-114
• 22 Bergman T, Lize N, Beijer S, Raijmakers N, Metselaar S. A care ethics approach to a reduced ability to eat. Nurs Ethics 2023; (e-pub ahead of print)
• 23 Miller RG, Jackson CE, Kasarskis EJ. et al; Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009; 73 (15) 1218-1226
• 24 Birnkrant DJ, Bushby K, Bann CM. et al; DMD Care Considerations Working Group. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol 2018; 17 (03) 251-267
• 25 Brumbaugh D, Watne L, Gottrand F. et al. Nutritional and gastrointestinal management of the patient with Duchenne muscular dystrophy. Pediatrics 2018; 142 (Suppl. 02) S53-S61
• 26 Stavroulakis T, Baird WO, Baxter SK, Walsh T, Shaw PJ, McDermott CJ. Factors influencing decision-making in relation to timing of gastrostomy insertion in patients with motor neurone disease. BMJ Support Palliat Care 2014; 4 (01) 57-63
• 27 Sulistyo A, Abrahao A, Freitas ME, Ritsma B, Zinman L. Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2023; 8 (08) CD004030
• 28 Yuen JK, Chan FHW, Chan TC. et al. Hospital careful hand feeding program reduced feeding tube use in patients with advanced dementia. J Nutr Health Aging 2023; 27 (06) 432-437
• 29 Khan A, Frazer-Green L, Amin R. et al. Respiratory management of patients with neuromuscular weakness: an American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report. Chest 2023; 164 (02) 394-413
• 30 Annunziata A, Coppola A, Polistina GE. et al. Daytime alternatives for non-invasive mechanical ventilation in neuromuscular disorders. Acta Myol 2021; 40 (01) 51-60
• 31 Cesareo A, LoMauro A, Santi M, Biffi E, D'Angelo MG, Aliverti A. Acute effects of mechanical insufflation-exsufflation on the breathing pattern in stable subjects with Duchenne muscular dystrophy. Respir Care 2018; 63 (08) 955-965
• 32 Chatwin M, Ross E, Hart N, Nickol AH, Polkey MI, Simonds AK. Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J 2003; 21 (03) 502-508
• 33 Carnevale FA, Alexander E, Davis M, Rennick J, Troini R. Daily living with distress and enrichment: the moral experience of families with ventilator-assisted children at home. Pediatrics 2006; 117 (01) e48-e60
• 34 Sancho J, Servera E, Díaz JL, Bañuls P, Marín J. Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival. Thorax 2011; 66 (11) 948-952
• 35 Spataro R, Bono V, Marchese S, La Bella V. Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis. J Neurol Sci 2012; 323 (1-2): 66-70
• 36 Laakso K, Markström A, Idvall M, Havstam C, Hartelius L. Communication experience of individuals treated with home mechanical ventilation. Int J Lang Commun Disord 2011; 46 (06) 686-699
• 37 Evans R, Catapano MA, Brooks D, Goldstein RS, Avendano M. Family caregiver perspectives on caring for ventilator-assisted individuals at home. Can Respir J 2012; 19 (06) 373-379
• 38 DeCourcey DD, Silverman M, Oladunjoye A, Wolfe J. Advance care planning and parent-reported end-of-life outcomes in children, adolescents, and young adults with complex chronic conditions. Crit Care Med 2019; 47 (01) 101-108
• 39 Seeber AA, Pols AJ, Hijdra A, Grupstra HF, Willems DL, de Visser M. Advance care planning in progressive neurological diseases: lessons from ALS. BMC Palliat Care 2019; 18 (01) 50
• 40 Martinez NP, Bueno SM, Muriana AP, Ballesteros FM, Collado NF, Gamez BJ. Survival and decision making for tracheotomy in ALS. Eur Respir J 2018; 52 (Suppl. 62) PA2393
• 41 POLST. Accessed 12 June 2024 at: https://polst.org/
• 42 Lunn JS. Spiritual care in a multi-religious context. Journal of pain & palliative care pharmacotherapy. England: Informa Healthcare; 2003: 153-166
• 43 Steinhauser KE, Christakis NA, Clipp EC, McNeilly M, McIntyre L, Tulsky JA. Factors considered important at the end of life by patients, family, physicians, and other care providers. JAMA 2000; 284 (19) 2476-2482
• 44 Grossoehme DH, Thienprayoon R, Sawnani H. et al. Assessment of the relationship between disease progression and goals of care by individuals with Duchenne muscular dystrophy and their caregivers. Muscle Nerve 2022; 65 (06) 646-651
• 45 Glare P, Virik K, Jones M. et al. A systematic review of physicians' survival predictions in terminally ill cancer patients. BMJ 2003; 327 (7408) 195-198
• 46 Wahlgren L, Kroksmark AK, Tulinius M, Sofou K. One in five patients with Duchenne muscular dystrophy dies from other causes than cardiac or respiratory failure. Eur J Epidemiol 2022; 37 (02) 147-156
• 47 Lunn MR, Wang CH. Spinal muscular atrophy. Lancet 2008; 371 (9630) 2120-2133
• 48 Mandler RN, Anderson Jr FA, Miller RG, Clawson L, Cudkowicz M, Del Bene M. ALS C.A.R.E. Study Group. The ALS Patient Care Database: insights into end-of-life care in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 2001; 2 (04) 203-208
• 49 Messer B, Armstrong A, Doris T, Williams T. Requested withdrawal of mechanical ventilation in six patients with motor neuron disease. BMJ Support Palliat Care 2020; 10 (01) 10-13
• 50 de Visser M, Oliver DJ. Palliative care in neuromuscular diseases. Curr Opin Neurol 2017; 30 (06) 686-691
• 51 Saetrang T, Bjørk IT, Capjon H, Rasmussen M. Parent-child communication and timing of interventions are challenges in the Duchenne muscular dystrophy care. Acta Paediatr 2019; 108 (03) 535-540