• Anne-Chantal Heritier Barras, Dan Adler, Ruxandra Iancu Ferfoglia, Bara Ricou, Yvan Gasche, Igor Leuchter, Samia Hurst, Monica Escher, Pierre Pollak, Jean-Paul Janssens, and CeSLA group.
• Department of Clinical Neurosciences, Division of Neurology, Geneva University Hospitals, Switzerland. Anne-Chantal.HeritierBarras@hcuge.ch
• Swiss Med Wkly. 2013 Jan 1;143:w13830.

Question Under StudyAmyotrophic 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.MethodsThe 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.ResultsImproved 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.ConclusionsCurrent 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.

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