Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades

J Cyst Fibros. 2012 May;11(3):187-92. doi: 10.1016/j.jcf.2011.11.006. Epub 2011 Dec 16.


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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / mortality
  • Cystic Fibrosis / therapy*
  • Female
  • Follow-Up Studies
  • Forced Expiratory Volume
  • Humans
  • Lung Transplantation
  • Male
  • Middle Aged
  • Positive-Pressure Respiration / methods*
  • Preoperative Care / methods*
  • Prognosis
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy*
  • Retrospective Studies
  • Survival Rate
  • Time Factors
  • United Kingdom / epidemiology
  • Young Adult