title: Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades.
abstract: BACKGROUND
  Non-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting.
  
  
  METHODS
  We reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010.
  
  
  RESULTS
  Of 47 patients studied, 36% underwent lung transplantation, 28% died without transplantation and 30% remain alive on NIV. Median duration of NIV was 16 months (range 2-90). Mean FEV(1) fell by 212 ml over the year before NIV but increased by 18 ml in the following year (p<0.01). Individual response to NIV was associated with lower baseline and more rapid decline in FEV(1). From 1991 to 2000, 70% underwent lung transplantation; from 2001 to 2010 only 27% were transplanted.
  
  
  CONCLUSIONS
  NIV may slow or reverse the decline in lung function in advanced CF. NIV is increasingly used beyond a bridge to transplantation at our centre.
authors.affiliation: Manchester Adult Cystic Fibrosis Centre, University Hospital of South Manchester, Southmoor Road, Manchester, M23 9LT, United Kingdom. william.flight@uhsm.nhs.uk
authors.forename: William G
authors.initials: WG
authors.lastname: Flight
authors.forename: Jonathan
authors.initials: J
authors.lastname: Shaw
authors.forename: Susan
authors.initials: S
authors.lastname: Johnson
authors.forename: A Kevin
authors.initials: AK
authors.lastname: Webb
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Jones
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Bentley
authors.forename: Rowland J
authors.initials: RJ
authors.lastname: Bright-Thomas
mesh_terms: D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011175:Positive-Pressure Respiration / Q000379:methods*; D011300:Preoperative Care / Q000379:methods*; D011379:Prognosis; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult