title: Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival.
abstract: BACKGROUND
  Home tracheotomy mechanical ventilation (HTMV) can prolong survival in patients with amyotrophic lateral sclerosis (ALS) when non-invasive ventilation (NIV) fails, but knowledge about HTMV is scarce. The aim of this study was to determine the causes of tracheotomy and the main issues of 1-year HTMV in a cohort of patients with ALS.
  
  
  METHODS
  A prospective study of all patients needing HTMV was performed in a referral respiratory care unit (RCU) from April 2001 to January 2010. Patients' informed decisions about HTMV were fully respected. Caregivers were trained and could telephone the RCU. Hospital staff made home visits.
  
  
  RESULTS
  All patients (n=116) agreed to participate and a tracheotomy was needed for 76, mainly due to bulbar dysfunction. Of the 38 who had a tracheotomy, in 21 it was performed in an acute setting and in 17 as a non-emergency procedure. In 19 patients the tracheotomy was related to the inadequacy of mechanically assisted coughing (MAC) to maintain normal oxygen saturation. During HTMV, 19 patients required hospitalisation, 12 with respiratory problems. The 1-year survival rate was 78.9%, with a mean survival of 10.39 months (95% CI 9.36 to 11.43). Sudden death was the main cause of death (n=9) and only one patient died from respiratory causes. No predictive factors for survival were found.
  
  
  CONCLUSIONS
  Besides NIV inadequacy, the ineffectiveness of mechanically assisted coughing appears to be a relevant cause of tracheotomy for patients with ALS with severe bulbar dysfunction. Patients choosing HTMV provided by a referral RCU could have a good 1-year survival rate, respiratory problems being the main cause of hospitalisation but not of death.
authors.affiliation: Respiratory Care Unit, Respiratory Medicine Department, Hospital Clínico Universitario, Avd Blasco Ibañez 17.46010 Valencia, Spain. cchinesta@eresmas.com
authors.forename: Jesús
authors.initials: J
authors.lastname: Sancho
authors.forename: Emilio
authors.initials: E
authors.lastname: Servera
authors.forename: José Luis
authors.initials: JL
authors.lastname: Díaz
authors.forename: Pilar
authors.initials: P
authors.lastname: Bañuls
authors.forename: Julio
authors.initials: J
authors.lastname: Marín
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000401:mortality / Q000628:therapy*; D002423:Cause of Death; D004812:Epidemiologic Methods; D005260:Female; D018575:Home Care Services, Hospital-Based; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011379:Prognosis; D011446:Prospective Studies; D012120:Respiration Disorders / Q000209:etiology; D012121:Respiration, Artificial / Q000009:adverse effects / Q000379:methods*; D012124:Respiratory Care Units; D013030:Spain / Q000453:epidemiology; D016019:Survival Analysis; D014140:Tracheotomy / Q000009:adverse effects / Q000379:methods*