The use of continuous positive airway pressure or non-invasive ventilation as forms of respiratory support in children with cystic fibrosis.

Non-invasive ventilation is used in patients with cystic fibrosis to treat respiratory failure, as a bridge to transplantation and as an adjunct to airway clearance techniques, despite a limited evidence base, particularly amongst the paediatric population. Where early CF lung disease is complicated by tracheobronchomalacia, we believe CPAP has good theoretical reasons for benefit and we describe a case which serves to illustrate these benefits in clinical practice. As time and technology move on we may begin to see more children with cystic fibrosis being treated with respiratory support.