Journal of Neurology

, Volume 265, Issue 11, pp 2730–2736 | Cite as

Ethical challenges in tracheostomy-assisted ventilation in amyotrophic lateral sclerosis

  • Morten MagelssenEmail author
  • Trygve Holmøy
  • Morten Andreas Horn
  • Ove Arne Fondenæs
  • Knut Dybwik
  • Reidun Førde
Original Communication


The special nature of amyotrophic lateral sclerosis (ALS) and tracheostomy with invasive ventilation (TIV) leads to challenges that can be difficult in two senses: not only to handle well, but also to discuss with patients and other involved stakeholders. Because of the delicate nature of interpersonal relations and communication in ALS, some of the downsides to TIV may almost take on a nature of taboo, making them difficult to raise for open discussion. Yet these ethical challenges are important to be aware of, not only for health professionals and managers but, arguably, also for patients and next of kin. They are important also for a wider professional and societal debate about whether and to whom TIV should be offered. In this paper we highlight and examine ethical challenges in TIV for ALS, with a special emphasis on those that are hard to discuss openly and that therefore might fail to be addressed. The analysis is structured by the four core principles of healthcare ethics: beneficence, nonmaleficence, respect for patient autonomy, and justice.


Amyotrophic lateral sclerosis Ethics Beneficence Invasive ventilation Patient autonomy Tracheostomy 


Compliance with ethical standards

Conflicts of interest

Dr. Magelssen has received a fee for a lecture for Sanofi-Aventis. Dr. Holmøy has received unrestricted research grants from The Norwegian ALS foundation.

Ethical standards

Ethics approval was not required for this study as it did not involve the generation of new data.


  1. 1.
    Rooney J, Byrne S, Heverin M, Corr B, Elamin M, Staines A et al (2013) Survival analysis of Irish amyotrophic lateral sclerosis patients diagnosed from 1995 to 2010. PLoS One 8(9):e74733CrossRefGoogle Scholar
  2. 2.
    Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W et al (2009) 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 73(15):1218–1226. CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P et al (2012) EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)—revised report of an EFNS task force. Eur J Neurol 19(3):360–375. CrossRefPubMedGoogle Scholar
  4. 4.
    Radunovic A, Annane D, Rafiq MK, Mustfa N (2013) Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 10:CD004427Google Scholar
  5. 5.
    Berlowitz DJ, Howard ME, Fiore JF, Vander Hoorn S, O’Donoghue FJ, Westlake J et al (2016) Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort. J Neurol Neurosurg Psychiatry 87(3):280–286CrossRefGoogle Scholar
  6. 6.
    Connolly S, Galvin M, Hardiman O (2015) End-of-life management in patients with amyotrophic lateral sclerosis. Lancet Neurol 14(4):435–442CrossRefGoogle Scholar
  7. 7.
    Lerum SV, Solbrække KN, Frich JC (2017) Healthcare professionals’ accounts of challenges in managing motor neurone disease in primary healthcare: a qualitative study. Health Soc Care Community 25(4):1355–1363CrossRefGoogle Scholar
  8. 8.
    Weber C, Fijalkowska B, Ciecwierska K, Lindblad A, Badura-Lotter G, Andersen PM et al (2017) Existential decision-making in a fatal progressive disease: how much do legal and medical frameworks matter? BMC Palliat Care 16(1):80CrossRefGoogle Scholar
  9. 9.
    Dybwik K, Tollåli T, Nielsen EW, Brinchmann BS (2010) Why does the provision of home mechanical ventilation vary so widely? Chronic Respir Dis 7(2):67–73CrossRefGoogle Scholar
  10. 10.
    Tollefsen E, Midgren B, Bakke P, Fondenes O (2010) Amyotrophic lateral sclerosis: gender differences in the use of mechanical ventilation. Eur J Neurol 17(11):1352–1357CrossRefGoogle Scholar
  11. 11.
    Chio A, Calvo A, Ghiglione P, Mazzini L, Mutani R, Mora G et al (2010) Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry 81(10):1141–1143. CrossRefPubMedGoogle Scholar
  12. 12.
    Tagami M, Kimura F, Nakajima H, Ishida S, Fujiwara S, Doi Y et al (2014) Tracheostomy and invasive ventilation in Japanese ALS patients: decision-making and survival analysis: 1990–2010. J Neurol Sci 344(1–2):158–164. CrossRefPubMedGoogle Scholar
  13. 13.
    Dreyer P, Lorenzen CK, Schou L, Felding M (2014) Survival in ALS with home mechanical ventilation non-invasively and invasively: a 15-year cohort study in west Denmark. Amyotroph Lateral Scler Frontotemporal Degener 15(1–2):62–67. CrossRefPubMedGoogle Scholar
  14. 14.
    Heritier Barras AC, Adler D, Iancu Ferfoglia R, Ricou B, Gasche Y, Leuchter I et al (2013) Is tracheostomy still an option in amyotrophic lateral sclerosis? Reflections of a multidisciplinary work group. Swiss Med Wkly 143:w13830. CrossRefPubMedGoogle Scholar
  15. 15.
    Dreyer PS, Felding M, Klitnæs CS, Lorenzen CK (2012) Withdrawal of invasive home mechanical ventilation in patients with advanced amyotrophic lateral sclerosis: ten years of Danish experience. J Palliat Med 15(2):205–209CrossRefGoogle Scholar
  16. 16.
    Paganoni S, Karam C, Joyce N, Bedlack R, Carter GT (2015) Comprehensive rehabilitative care across the spectrum of amyotrophic lateral sclerosis. NeuroRehabilitation 37(1):53–68. CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Calvo A, Moglia C, Ilardi A, Cammarosano S, Gallo S, Canosa A et al (2011) Religiousness is positively associated with quality of life of ALS caregivers. Amyotroph Lateral Scler 12(3):168–171. CrossRefPubMedGoogle Scholar
  18. 18.
    Chio A, Gauthier A, Montuschi A, Calvo A, Di Vito N, Ghiglione P et al (2004) A cross sectional study on determinants of quality of life in ALS. J Neurol Neurosurg Psychiatry 75(11):1597–1601. CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Simmons Z, Bremer BA, Robbins RA, Walsh SM, Fischer S (2000) Quality of life in ALS depends on factors other than strength and physical function. Neurology 55(3):388–392CrossRefGoogle Scholar
  20. 20.
    Slevin ML, Plant H, Lynch D, Drinkwater J, Gregory WM (1988) Who should measure quality of life, the doctor or the patient? Br J Cancer 57(1):109–112CrossRefGoogle Scholar
  21. 21.
    Kubler A, Winter S, Ludolph AC, Hautzinger M, Birbaumer N (2005) Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabilit Neural Repair 19(3):182–193. CrossRefGoogle Scholar
  22. 22.
    Lule D, Ehlich B, Lang D, Sorg S, Heimrath J, Kubler A et al (2013) Quality of life in fatal disease: the flawed judgement of the social environment. J Neurol 260(11):2836–2843. CrossRefPubMedGoogle Scholar
  23. 23.
    Rothwell PM, McDowell Z, Wong CK, Dorman PJ (1997) Doctors and patients don’t agree: cross sectional study of patients’ and doctors’ perceptions and assessments of disability in multiple sclerosis. BMJ 314(7094):1580–1583CrossRefGoogle Scholar
  24. 24.
    Rousseau MC, Pietra S, Blaya J, Catala A (2011) Quality of life of ALS and LIS patients with and without invasive mechanical ventilation. J Neurol 258(10):1801–1804. CrossRefPubMedGoogle Scholar
  25. 25.
    Bruno M-A, Bernheim JL, Ledoux D, Pellas F, Demertzi A, Laureys S (2011) A survey on self-assessed well-being in a cohort of chronic locked-in syndrome patients: happy majority, miserable minority. BMJ Open 1(1):e000039CrossRefGoogle Scholar
  26. 26.
    Rousseau MC, Baumstarck K, Billette de Villemeur T, Auquier P (2016) Evaluation of quality of life in individuals with severe chronic motor disability: a major challenge. Intractable Rare Dis Res 5(2):83–89. CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Lerum SV, Solbrække KN, Holmøy T, Frich JC (2015) Unstable terminality: negotiating the meaning of chronicity and terminality in motor neurone disease. Sociol Health Illn 37(1):81–96CrossRefGoogle Scholar
  28. 28.
    Dybwik K, Tollåli T, Nielsen EW, Brinchmann BS (2011) “Fighting the system”: families caring for ventilator-dependent children and adults with complex health care needs at home. BMC Health Serv Res 11(1):156CrossRefGoogle Scholar
  29. 29.
    Kaub-Wittemer D, Steinbuchel N, Wasner M, Laier-Groeneveld G, Borasio GD (2003) Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manag 26(4):890–896CrossRefGoogle Scholar
  30. 30.
    Dybwik K, Nielsen EW, Brinchmann BS (2011) Home mechanical ventilation and specialised health care in the community: between a rock and a hard place. BMC Health Serv Res 11:115. CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Woolley SC, Rush BK (2017) Considerations for clinical neuropsychological evaluation in amyotrophic lateral sclerosis. Arch Clin Neuropsychol 32(7):906–916. CrossRefPubMedGoogle Scholar
  32. 32.
    Lulé D, Böhm S, Müller H-P, Aho-Özhan H, Keller J, Gorges M et al (2018) Cognitive phenotypes of sequential staging in amyotrophic lateral sclerosis. Cortex 101:163–171CrossRefGoogle Scholar
  33. 33.
    Oliver DJ, Turner MR (2010) Some difficult decisions in ALS/MND. Amyotroph Lateral Scler 11(4):339–343. CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Menzel P (2014) Statistical versus indentified lives. Why not to use the “R” word. In: Danis M, Hurst S, Fleck L, Førde R, Slowther A (eds) Fair resource allocation and rationing at the bedside. Oxford University Press, New York, pp 238–252CrossRefGoogle Scholar
  35. 35.
    Nonoyama ML, McKim DA, Road J, Guerriere D, Coyte PC, Wasilewski M et al (2018) Healthcare utilisation and costs of home mechanical ventilation. Thorax. CrossRefPubMedGoogle Scholar
  36. 36.
    Magelssen M, Nortvedt P, Solbakk JH (2016) Rationing at the bedside: immoral or unavoidable? Clin Ethics 11(4):112–121. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Centre for Medical Ethics, Institute of Health and SocietyUniversity of OsloOsloNorway
  2. 2.Department of NeurologyAkershus University HospitalLørenskogNorway
  3. 3.Institute of Clinical MedicineUniversity of OsloOsloNorway
  4. 4.Department of NeurologyOslo University HospitalOsloNorway
  5. 5.National Advisory Unit on Longterm Mechanical VentilationHaukeland University HospitalBergenNorway
  6. 6.Department of Anaesthesia and Intensive Care MedicineNordland HospitalBodøNorway
  7. 7.Nord UniversityBodøNorway

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