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

BackgroundNon-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.MethodsWe reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010.ResultsOf 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 FEV1 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 FEV1. From 1991 to 2000, 70% underwent lung transplantation; from 2001 to 2010 only 27% were transplanted.ConclusionsNIV may slow or reverse the decline in lung function in advanced CF. NIV is increasingly used beyond a bridge to transplantation at our centre.