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Review article: Biomedical intelligence

Vol. 143 No. 3132 (2013)

Is tracheostomy still an option in amyotrophic lateral sclerosis?

  • Anne-Chantal Heritier-Barras
  • Dan Adler
  • Ruxandra Iancu Ferfoglia
  • Bara Ricou
  • Yvan Gasche
  • Igor Leuchter
  • Samia Hurst
  • Monica Escher-Imhof
  • Pierre Pollak
  • Jean-Paul Janssens
Cite this as:
Swiss Med Wkly. 2013;143:w13830


QUESTION UNDER STUDY: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with a poor prognosis. Survival and quality of life of ALS patients have improved through the implementation of multidisciplinary approaches, the use of percutaneous gastrostomy and of noninvasive (NIV) or invasive ventilation. The question of whether or not to propose invasive ventilation (by tracheostomy: TPPV) to ALS patients remains a matter of debate.

METHODS: The study reviews the medical literature, the practice in three Swiss and two large French ALS expert centres and reports the results of a workgroup on invasive ventilation in ALS.

RESULTS: Improved management of secretions and use of different interfaces allows NIV to be used 24-hours-a-day for prolonged periods, thus avoiding TPPV in many cases. TPPV is frequently initiated in emergency situations with lack of prior informed consent. TPPV appears associated with a lesser quality of life and a higher risk of institutionalisation than NIV. The high burden placed on caregivers who manage ALS patients is a major problem with a clear impact on their quality of life.

CONCLUSIONS: Current practice in Switzerland and France tends to discourage the use of TPPV in ALS. Fear of a “locked-in syndrome”, the high burden placed on caregivers, and unmasking cognitive disorders occurring in the evolution of ALS are some of the caveats when considering TPPV. Most decisions about TPPV are taken in emergency situations in the absence of advance directives. One exception is that of young motivated patients with predominantly bulbar disease who “fail” NIV.


  1. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. 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):1218–26.
  2. Mateen FJ, Carone M, Sorenson EJ. Patients who survive 5 years or more with ALS in Olmsted County, 1925–2004. J Neurol Neurosurg Psychiatry. 2010;81(10):1144–6.
  3. Weber M, Neuwirth C, Thierbach J, Schweikert K, Czaplinski A, Petersen J, et al. ALS patients with SOD1 mutations in Switzerland show very diverse phenotypes and extremely long survival. J Neurol Neurosurg Psychiatry. 2012;83(3):351–3.
  4. Chio A, Bottacchi E, Buffa C, Mutani R, Mora G. Positive effects of tertiary centres for amyotrophic lateral sclerosis on outcome and use of hospital facilities. J Neurol Neurosurg Psychiatry. 2006;77(8):948–50.
  5. Miller RG, Anderson F, Brooks BR, Mitsumoto H, Bradley WG, Ringel SP. Outcomes research in amyotrophic lateral sclerosis: lessons learned from the amyotrophic lateral sclerosis clinical assessment, research, and education database. Ann Neurol. 2009;65(Suppl 1):S24–8.
  6. Radunovic A, Mitsumoto H, Leigh PN. Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurol. 2007;6(10):913–25.
  7. Traynor BJ, Alexander M, Corr B, Frost E, Hardiman O. Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996–2000. J Neurol Neurosurg Psychiatry. 2003;74(9):1258–61.
  8. Leigh PN, Abrahams S, Al-Chalabi A, Ampong MA, Goldstein LH, Johnson J, et al. The management of motor neurone disease. J Neurol Neurosurg Psychiatry. 2003;74(Suppl 4):iv32–iv47.
  9. Mitsumoto H, Rabkin JG. Palliative care for patients with amyotrophic lateral sclerosis: “prepare for the worst and hope for the best”. JAMA. 2007;298(2):207–16.
  10. Van den Berg JP, Kalmijn S, Lindeman E, Veldink JH, de Visser M, Van der Graaff MM, et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology. 2005;65(8):1264–7.
  11. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest. 2004;126(5):1502–7.
  12. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5(2):140–7.
  13. Bourke SC, Williams TL, Bullock RE, Gibson GJ, Shaw PJ. Non-invasive ventilation in motor neuron disease: current UK practice. Amyotroph Lateral Scler Other Motor Neuron Disord. 2002;3(3):145–9.
  14. O’Neill CL, Williams TL, Peel ET, McDermott CJ, Shaw PJ, Gibson GJ, et al. Non-invasive ventilation in motor neuron disease: an update of current UK practice. J Neurol Neurosurg Psychiatry. 2012;83(4):371–6.
  15. Kohler A, Janssens JP, Haenggeli CA, Rossi JM, Sztajzel R. Assisted ventilation in chronic neuromuscular diseases. The Vincre Group. Rev Med Suisse Romande. 1997;117(6):479–84.
  16. Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) – revised report of an EFNS task force. Eur J Neurol. 2012;19(3):360–75.
  17. HAS. Care of patients with amyotrophic lateral sclerosis (ALS). Consensus conference guidelines.Haute Autorité de Santé, 2006.
  18. Farrero E, Prats E, Povedano M, Martinez-Matos JA, Manresa F, Escarrabill J. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement. Chest. 2005;127(6):2132–8.
  19. Radunovic A, Annane D, Jewitt K, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2009(4):CD004427.
  20. Neudert C, Oliver D, Wasner M, Borasio GD. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol. 2001;248(7):612–6.
  21. Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family, and physician attitudes. Neurology. 1993;43(2):438–43.
  22. Gonzalez-Bermejo J. Indications and equipment needs for ventilatory support in amyotrophic lateral sclerosis. Rev Neurol (Paris). 2006;162 Spec No 2:4S320–4S2.
  23. Gonzalez-Bermejo J, Hurbault A, Coupé C, Meininger V, Similowski T. Soins palliatifs et ventilation mécanique dans la sclérose latérale amyotrophique (Ventilatory support and palliative care in amyotrophic lateral sclerosis). Medecine Palliative. 2010;9:309–17.
  24. Chio A, Calvo A, Ghiglione P, Mazzini L, Mutani R, Mora G. Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry. 2010;81(10):1141–3.
  25. Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roos RP, Stocking CB, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Advance care planning and outcomes. Chest. 1996;110(1):249–55.
  26. Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. J Neurol Sci. 1996;139(Suppl):123–8.
  27. Narayanaswami P, Bertorini TE, Pourmand R, Horner LH. Long-term tracheostomy ventilation in neuromuscular diseases: patient acceptance and quality of life. Neurorehabil Neural Repair. 2000;14(2):135–9.
  28. Sancho J, Servera E, Diaz JL, Banuls P, Marin J. Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival. Thorax. 2011;66(11):948–52.
  29. Sancho J, Servera E, Banuls P, Marin J. Prolonging survival in amyotrophic lateral sclerosis: efficacy of noninvasive ventilation and uncuffed tracheostomy tubes. Am J Phys Med Rehabil. 2010;89(5):407–11.
  30. Kawata A, Mizoguchi K, Hayashi H. A nationwide survey of ALS patients on trachoestomy positive pressure ventilation (TPPV) who developed a totally locked-in state (TLS) in Japan. Rinsho Shinkeigaku. 2008;48(7):476–80.
  31. Hirano YM, Yamazaki Y, Shimizu J, Togari T, Bryce TJ. Ventilator dependence and expressions of need: a study of patients with amyotrophic lateral sclerosis in Japan. Soc Sci Med. 2006;62(6):1403–13.
  32. Vianello A, Arcaro G, Palmieri A, Ermani M, Braccioni F, Gallan F, et al. Survival and quality of life after tracheostomy for acute respiratory failure in patients with amyotrophic lateral sclerosis. J Crit Care. 2011;26(3):329 e7–14.
  33. Kaub-Wittemer D, Steinbuchel N, Wasner M, Laier-Groeneveld G, Borasio GD. Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manage. 2003;26(4):890–6.
  34. Albert SM, Whitaker A, Rabkin JG, del Bene M, Tider T, O'Sullivan I, et al. Medical and supportive care among people with ALS in the months before death or tracheostomy. J Pain Symptom Manage. 2009;38(4):546–53.
  35. Rabkin JG, Albert SM, Rowland LP, Mitsumoto H. How common is depression among ALS caregivers? A longitudinal study. Amyotroph Lateral Scler. 2009;10(5-6):448–55.
  36. Junod Perron N, Morabia A, de Torrente A. Quality of life of Do-Not-Resuscitate (DNR) patients: how good are physicians in assessing DNR patients’ quality of life? Swiss Med Wkly. 2002;132(39-40):562–5.
  37. Uhlmann RF, Pearlman RA. Perceived quality of life and preferences for life-sustaining treatment in older adults. Arch Intern Med. 1991;151(3):495–7.
  38. Gilbert DT, Wilson TD. Why the brain talks to itself: sources of error in emotional prediction. Philos Trans R Soc Lond B Biol Sci. 2009;364(1521):1335–41.
  39. Kahneman D, Krueger AB, Schkade D, Schwarz N, Stone AA. Would you be happier if you were richer? A focusing illusion. Science. 2006;312(5782):1908–10.
  40. Albert SM, Murphy PL, Del Bene ML, Rowland LP. A prospective study of preferences and actual treatment choices in ALS. Neurology. 1999;53(2):278–83.
  41. Emanuel EJ, Emanuel LL. Four models of the physician-patient relationship. JAMA. 1992;267(16):2221–6.
  42. Ferrer ML, Sellares J, Valencia M, Carrillo A, Gonzalez G, Badia J, et al. Non-invasive ventilation after extubation in hypercapnic patients with chronic respiratory disorders: randomised controlled trial. Lancet. 2009;374:1082–8.
  43. Girault C, Bubenheim M, Abroug F, Diehl JL, Elatrous S, Beuret P, et al. Noninvasive ventilation and weaning in patients with chronic hypercapnic respiratory failure: a randomized multicenter trial. Am J Respir Crit Care Med. 2011;184(6):672–9.
  44. Ornico SR, Lobo SM, Sanches HS, Deberaldini M, Tofoli LT, Vidal AM, et al. Noninvasive ventilation immediately after extubation improves weaning outcome after acute respiratory failure: a randomized controlled trial. Crit Care. 2013;17(2):R39.
  45. Bach JR, Goncalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest. 2010;137(5):1033–9.
  46. Schuster DP. Everything that should be done – not everything that can be done. Am Rev Respir Dis. 1992;145(3):508–9.
  47. Maessen M, Veldink JH, van den Berg LH, Schouten HJ, van der Wal G, Onwuteaka-Philipsen BD. Requests for euthanasia: origin of suffering in ALS, heart failure, and cancer patients. J Neurol. 2010;257(7):1192–8.
  48. Goldstein LH, Abrahams S. Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol. 2013;12(4):368–80.
  49. Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83(1):102–8.
  50. Rippon GA, Scarmeas N, Gordon PH, Murphy PL, Albert SM, Mitsumoto H, et al. An observational study of cognitive impairment in amyotrophic lateral sclerosis. Arch Neurol. 2006;63(3):345–52.
  51. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998;51(6):1546–54.
  52. Rabkin JG, Albert SM, Tider T, Del Bene ML, O’Sullivan I, Rowland LP, et al. Predictors and course of elective long-term mechanical ventilation: A prospective study of ALS patients. Amyotroph Lateral Scler. 2006;7(2):86–95.
  53. Young JM, Marshall CL, Anderson EJ. Amyotrophic lateral sclerosis patients’ perspectives on use of mechanical ventilation. Health Soc Work. 1994;19(4):253–60.
  54. Lyall R, Gelinas D. Control of symptoms: dyspnoea and respiratory symptoms. In: Oliver DB, GD; Walsh, D, editor. Palliative care in amyotrophic lateral sclerosis From diagnosis to bereavement. Oxford: Oxford University Press; 2006. p. 63–93.
  55. Bach JR. Continuous noninvasive ventilation for patients with neuromuscular disease and spinal cord injury. Semin Respir Crit Care Med. 2002;23(3):283–92.
  56. Servera E, Gomez-Merino E, Perez D, Marin J. Home mechanical ventilation in amyotrophic lateral sclerosis patients is not always a problem. Chest. 2000;117(3):924.
  57. Bach JR, Martinez D. Duchenne muscular dystrophy: continuous noninvasive ventilatory support prolongs survival. Respir Care. 2011;56(6):744–50.
  58. Bach JR. Noninvasive ventilation is more than mask ventilation. Chest. 2003;123(6):2156–7; author reply 7.
  59. Bach JR, Goncalves MR, Hon A, Ishikawa Y, De Vito EL, Prado F, et al. Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus. Am J Phys Med Rehabil. 2013;92(3):267–77.
  60. Mockford C, Jenkinson C, Fitzpatrick R. A Review: carers, MND and service provision. Amyotroph Lateral Scler. 2006;7(3):132–41.
  61. Lloyd-Owen SJ, Donaldson GC, Ambrosino N, Escarabill J, Farre R, Fauroux B, et al. Patterns of home mechanical ventilation use in Europe: results from the Eurovent survey. Eur Respir J. 2005;25(6):1025–31.
  62. Bruno M, Bernheim JL, Schnakers C, Laureys S. Locked-in: don’t judge a book by its cover. J Neurol Neurosurg Psychiatry. 2008;79(1):2.

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