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

William G. Flight, Jonathan Shaw, Susan Johnson, A. Kevin Webb, Andrew M. Jones, Andrew M. Bentley, Rowland J. Bright-Thomas

Research output: Contribution to journalArticlepeer-review


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 16months (range 2-90). Mean FEV 1 fell by 212ml over the year before NIV but increased by 18ml 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.

Original languageEnglish
Pages (from-to)187-192
Number of pages6
JournalJournal of Cystic Fibrosis
Issue number3
Publication statusPublished - 1 May 2012


  • Cystic fibrosis
  • Lung transplantation
  • Non-invasive ventilation
  • Respiratory failure


Dive into the research topics of 'Long-term non-invasive ventilation in cystic fibrosis - Experience over two decades'. Together they form a unique fingerprint.

Cite this