Noninvasive ventilation in respiratory failure due to cystic fibrosis

Background. Noninvasive positive-pressure ventilation (NIPPV) is increasingly used as an effective means of avoiding endotracheal intubation and mechanical ventilation in patients with respiratory insufficiency or failure. Methods. We retrospectively reviewed our experience with NIPPV to treat respiratory failure in five patients with cystic fibrosis (CF). Results. Despite chronic lung disease related to CF, none of our cases were end-stage. All patients had recent pulmonary function tests showing a forced expiratory volume in 1 second (FEV₁) of more than 30% predicted for age. All patients had progressive atelectasis, hypoxemia, and impending respiratory failure related to an acute pulmonary exacerbation or upper abdominal surgical procedure (open gastrostomy tube placement). Respiratory rates decreased, oxygen saturation increased, fraction of inspired oxygen (FIO₂) requirement decreased, transcutaneous CO₂ decreased, and atelectasis resolved with NIPPV. Conclusions. Use of NIPPV provides effective respiratory support while avoiding the need for endotracheal intubation. The applications of NIPPV, reports of its use in patients with CF, and the equipment required are reviewed.