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- Non-invasive positive pressure ventilation
- cystic fibrosis
- children
- non invasive ventilation
- paediatric lung disease
Non-invasive positive pressure ventilation (NIPPV) for respiratory failure in cystic fibrosis (CF) is frequently used in adults and has been shown to be of benefit to patients with advanced disease in terms of stabilisation of lung function,1 reduction in symptoms and increased exercise capacity.2 When used as an adjunct to physiotherapy, NIPPV increases oxygen saturations (Spo2), tidal volume, maximum expiratory muscle strength and ease of sputum clearance.3 4 There are no guidelines for the assessment of gas exchange or timing and mode of NIPPV initiation in patients with CF. The British Thoracic Society guideline states ‘there is insufficient evidence to recommend its routine use in patients with CF’.5 The aim of this study was to establish current …