Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure

•NIV is a treatment option for CF patients with type II respiratory failure.•This study examined the impact and outcomes of NIV use in adult CF patients studied over a 9 year period.•NIV use in CF adults with respiratory failure was associated with long term improvements in lung function and BMI and a reduction in carbon dioxide levels.•25% of patients received lung transplantation and modelling suggests that 50% of patients commenced on NIV will survive for approximately 5 years.