title: Predictors of non-invasive ventilation tolerance in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  The most frequent cause of death in patients with Amyotrophic Lateral Sclerosis (ALS) is respiratory failure. Recently, it has been shown that non-invasive ventilation improves survival and quality of life in ALS patients with respiratory failure, but little is known about predictors of non-invasive ventilation adaptation and tolerance. In this study we evaluated the effect of a comprehensive information about non-invasive ventilation use and a prolonged and intensive monitoring on tolerance to this palliative care.
  
  
  METHODS
  We prospectively monitored all consecutive ALS patients with chronic respiratory failure and indication to non-invasive ventilation between January 2005 and December 2007. Non-invasive ventilation adaptation was always performed in a hospital setting.
  
  
  RESULTS
  Forty-four patients were considered eligible: six declined the non-invasive ventilation proposal and one was excluded due to severe fronto-temporal dementia. Non-invasive ventilation was offered to thirty-seven inpatients in our ALS Centre, thirty-two of whom presented with severe (n=9) or mild-moderate (n=23) bulbar impairment at non-invasive ventilation initiation. The mean time interval for adaptation to ventilation was 5±2 days, but patients remained in hospital for an average extended period of one week. Thirty-five of the 37 patients who started non-invasive ventilation, including those with severe bulbar impairment, remained tolerant at twelve months follow-up.
  
  
  CONCLUSIONS
  Our study shows that an intensive educational training and adaptation on non-invasive ventilation, when performed in a hospital multidisciplinary setting, increases compliance and tolerance over time, even in those patients with severe bulbar impairment. However, the design of our study, mainly based on a continuous monitoring and educational training of the patients, might not make it fully applicable to an outpatients setting.
authors.affiliation: Neurorehabilitation Unit, Fondazione Salvatore Maugeri, Mistretta, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Volanti
authors.forename: Fabio
authors.initials: F
authors.lastname: Cibella
authors.forename: Maria
authors.initials: M
authors.lastname: Sarvà
authors.forename: Domenico
authors.initials: D
authors.lastname: De Cicco
authors.forename: Antonio
authors.initials: A
authors.lastname: Spanevello
authors.forename: Gabriele
authors.initials: G
authors.lastname: Mora
authors.forename: Vincenzo
authors.initials: V
authors.lastname: La Bella
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000503:physiopathology*; D001784:Blood Gas Analysis; D015992:Body Mass Index; D017028:Caregivers; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010092:Oximetry; D010361:Patients; D011446:Prospective Studies; D012121:Respiration, Artificial / Q000009:adverse effects*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D012798:Sialorrhea / Q000150:complications; D013147:Spirometry; D012122:Ventilators, Mechanical