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Semin Respir Crit Care Med
DOI: 10.1055/s-0044-1787994
Review Article

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

Benjamin J. Lipanot
1   Division of Pulmonary, Critical Care, Allergy, and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana
,
Gabriel Bosslet
1   Division of Pulmonary, Critical Care, Allergy, and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana
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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


Publication History

Article published online:
19 July 2024

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  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 6 Martinez NP, Herrero MM, Rebollo CG, Repiso EM. How our amyotrophic lateral sclerosis patients die. Eur Respir J 2020; 56 (Suppl. 64) 3419
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 8 Kripke C. Adults with developmental disabilities: a comprehensive approach to medical care. Am Fam Physician 2018; 97 (10) 649-656
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 11 Kruser JM, Clapp JT, Arnold RM. Reconsidering the language of serious illness. JAMA 2023; 330 (07) 587-588
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 13 Phillips J, Wendler D. Clarifying substituted judgement: the endorsed life approach. J Med Ethics 2015; 41 (09) 723-730
    CrossrefPubMedGoogle Scholar
  • 14 Katz AL, Webb SA. COMMITTEE ON BIOETHICS. Informed consent in decision-making in pediatric practice. Pediatrics 2016; 138 (02) e20161485
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 16 Zietlow K, Dubin L, Battles A, Vitale C. Guardianship: a medicolegal review for clinicians. J Am Geriatr Soc 2022; 70 (11) 3070-3079
    CrossrefPubMedGoogle Scholar
  • 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
    Google Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 19 Schaible UE, Kaufmann SHE. Malnutrition and infection: complex mechanisms and global impacts. PLoS Med 2007; 4 (05) e115
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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) DOI: 10.1177/09697330231197708.
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 41 POLST. Accessed 12 June 2024 at: https://polst.org/
    PubMedGoogle Scholar
  • 42 Lunn JS. Spiritual care in a multi-religious context. Journal of pain & palliative care pharmacotherapy. England: Informa Healthcare; 2003: 153-166
    Google Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 47 Lunn MR, Wang CH. Spinal muscular atrophy. Lancet 2008; 371 (9630) 2120-2133
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 50 de Visser M, Oliver DJ. Palliative care in neuromuscular diseases. Curr Opin Neurol 2017; 30 (06) 686-691
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar