title: Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis.
abstract: BACKGROUND
  Tracheostomy mechanical ventilation (TMV) is performed in amyotrophic lateral sclerosis (ALS) patients with a respiratory failure or when the non-invasive ventilation (NIV) is no longer effective. We evaluated the clinical characteristics and survival of a cohort of tracheostomized ALS patients, followed in a single ALS Clinical Center.
  
  
  METHODS
  Between 2001 and 2010, 87 out of 279 ALS patients were submitted to TMV. Onset was spinal in 62 and bulbar in 25. After tracheostomy, most patients were followed up through telephone interviews to caregivers. A complete survival analysis could be performed in fifty-two TMV patients.
  
  
  RESULTS
  31.3% ALS patients underwent tracheostomy, with a male prevalence (M/F=1.69) and a median age of 61 years (interquartile range=47-66). After tracheostomy, nearly all patients were under home care. TMV ALS patients were more likely than non-tracheostomized (NT) patients to be implanted with a PEG device, although the bulbar-/spinal-onset ratio did not differ between the two groups. Kaplan-Meyer analysis showed that tracheostomy increases median survival (TMV, 47 months vs NT, 31 months, p=0.008), with the greatest effect in patients younger than 60 at onset (TMV ≤ 60 years, 57.5 months vs NT ≤ 60 years, 38.5 months, p=0.002).
  
  
  CONCLUSIONS
  TMV is increasingly performed in ALS patients. Nearly all TMV patients live at home and most of them are fed through a PEG device. Survival after tracheostomy is generally increased, with the stronger effect in patients younger than 60. This survival advantage is apparently lost when TMV is performed in patients older than 60. The results of this study might be useful for the decision-making process of patients and their families about this advanced palliative care.
authors.affiliation: ALS Clinical Research Center, Bio.Ne.C, University of Palermo, 90129 Palermo, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Spataro
authors.forename: Valeria
authors.initials: V
authors.lastname: Bono
authors.forename: Santino
authors.initials: S
authors.lastname: Marchese
authors.forename: Vincenzo
authors.initials: V
authors.lastname: La Bella
mesh_terms: D017668:Age of Onset; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000401:mortality; D015331:Cohort Studies; D018450:Disease Progression; D004750:Enteral Nutrition / Q000706:statistics & numerical data; D005260:Female; D005500:Follow-Up Studies; D006699:Home Care Services / Q000706:statistics & numerical data; D006801:Humans; D007326:Institutionalization / Q000706:statistics & numerical data; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010166:Palliative Care; D011446:Prospective Studies; D012121:Respiration, Artificial / Q000379:methods* / Q000706:statistics & numerical data; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy*; D014139:Tracheostomy / Q000706:statistics & numerical data