Noninvasive ventilation in cystic fibrosis

Abstract

A physiological rationale has been demonstrated for the use of noninvasive positive pressure ventilation (NPPV) in patients with cystic fibrosis. Indeed, as lung disease progresses, respiratory muscle load increases. As a result, patients develop a compensatory mechanism of a rapid shallow breathing pattern in an attempt to reduce this increase in load. Although this breathing strategy may maintain the level of minute ventilation, alveolar hypoventilation, characterized by hypercapnia and hypoxemia, may develop. Physiological studies during wakefulness and sleep have demonstrated that NPPV is associated with a reduction in respiratory muscle-load and work of breathing, an increase in minute ventilation and an improvement in gas exchange. NPPV has been shown to reduce oxygen desaturation during exercise and chest physiotherapy and may facilitate the recovery of a severe respiratory exacerbation. However, long-term data are lacking and are warranted to determine the optimal criteria to propose NPPV in these patients, and to assess the benefits in terms of improved survival and quality of life.