Noninvasive ventilation in respiratory failure due to cystic fibrosis

Background. Noninvasive positive-pressure ventilation (NIPPV) is increasing ly used as an effective means of avoiding endotracheal intubation and mecha nical ventilation in patients with respiratory insufficiency or failure. Methods. We retrospectively reviewed our experience with NIPPV to treat res piratory failure in five patients with cystic fibrosis (CF). Results. Despite chronic lung disease related to CF, none of our cases were end-stage. AU patients had recent pulmonary function tests showing a force d expiratory volume in 1 second (FEV1) of more than 30% predicted for age. All patients had progressive atelectasis, hypoxemia, and impending respirat ory failure related to an acute pulmonary exacerbation or upper abdominal s urgical procedure (open gastrostomy tube placement). Respiratory rates decr eased, oxygen saturation increased, fraction of inspired oxygen (FIO2) requ irement decreased, transcutaneous COP decreased, and atelectasis resolved w ith NIPPV. Conclusions. Use of NIPPV provides effective respiratory support while avoi ding the need for endotracheal intubation. The applications-of NIPPV, repor ts of its use in patients with CF, and the equipment required are reviewed.