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Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades
William G Flight1, Jonathan Shaw, Susan Johnson
1Manchester Adult Cystic Fibrosis Centre, University Hospital of South Manchester, Southmoor Road, Manchester, M23 9LT, United Kingdom. william.flight@uhsm.nhs.uk
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.
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