The Current Practice of Noninvasive Ventilation in Patients With Cystic Fibrosis

In patients with cystic fibrosis (CF), despite the availability of many different pharmacologic agents, lung function deteriorates and lung disease progresses and leads to hypercapnic respiratory failure in some patients. In such cases, noninvasive ventilation (NIV) seems to be a promising technique that can be used on demand. This review summarizes the current applications of NIV in clinical settings as well as findings of the clinical trials that involved the delivery of NIV on variable occasions, such as an adjunct to physiotherapy, in nocturnal hypoventilation, and acute and chronic respiratory failure. NIV has been used in patients with CF and with advanced lung disease who are not considered candidates for lung transplantation. It can stabilize lung function, although its effect on hypercapnia is not always evident. Nocturnal NIV has been used in patients with CF and with hypoventilation during sleep but without clear benefits on daytime P-CO2. NIV as an adjunct to chest physiotherapy may be helpful when desaturation is observed during physiotherapy and when there are signs of respiratory muscle fatigue. NIV use in CF has been increasing, mainly in adult CF centers, and offers patients an opportunity to reach lung transplantation or to overcome acute hypercapnic respiratory failure.