title: Qualify of life and palliation predict survival in patients with chronic alveolar hypoventilation and nocturnal ventilatory support.
abstract: OBJECTIVE
  Non-invasive positive pressure ventilation (NPPV) improves health-related quality of life (HRQL) in patients with chronic alveolar hypoventilation (CAH). We studied the prognostic impact of HRQL on survival in relation to clinical factors.
  
  
  METHODS
  Forty-four patients with CAH due to post-polio (12), scoliosis (11), post-tb (17) or other diagnoses (4) who received nocturnal NPPV were prospectively studied during 6-10 years.
  
  
  METHODS
  Blood gases and HRQL were analysed at baseline and after 9 months and after 8 years. HRQL was evaluated with measures of functioning (SIP), emotional well-being (HADS and MACL), and global QL.
  
  
  RESULTS
  Blood gases and HRQL measures improved during NPPV. The overall 5-year survival rate was 73%. In multivariate survival analysis, a diagnosis of post-polio and low baseline SIP physical index scores, indicating low levels of physical dysfunction, predicted longer survival (P = 0.02, respectively). Similarly, palliation of physical dysfunction and preserved or improved global QL by 9 months were associated with longer overall survival (P = 0.009 and P = 0.001, respectively; multivariate Cox regression).
  
  
  CONCLUSIONS
  Seventy-three percent of patients treated for CAH with NPPV survived more than 5 years. Diagnosis and self-rated physical functioning at pre-treatment were related to survival, as were major improvements in physical functioning and global QL during NPPV.
authors.affiliation: Department of Respiratory Medicine and Allergology, Institute of Medicine, Sahlgrenska University Hospital, The Sahlgrenska Academy at University of Gothenburg, Gothenburg, 413 45, Sweden. jan.olofson@lungall.gu.se
authors.forename: Jan
authors.initials: J
authors.lastname: Olofson
authors.forename: Catharina
authors.initials: C
authors.lastname: Dellborg
authors.forename: Marianne
authors.initials: M
authors.lastname: Sullivan
authors.forename: Bengt
authors.initials: B
authors.lastname: Midgren
authors.forename: Oscar
authors.initials: O
authors.lastname: Caro
authors.forename: Bengt
authors.initials: B
authors.lastname: Bergman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009468:Neuromuscular Diseases; D011175:Positive-Pressure Respiration; D011788:Quality of Life; D012891:Sleep Apnea Syndromes / Q000628:therapy*; D055815:Young Adult