Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades, the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF may also benefit from NIV for other diagnosis beyond advanced lung disease. NIV can improve gas exchange, quality of sleep, exercise tolerance, and augment airway clearance in CF. CF providers can readily become comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation, and discuss how to order and monitor patients on NIV. We will discuss aspects unique to people with CF and the use of NIV, as well as suggestions on how to reduce risks such as infection. We hope that this serves as a resource for CF providers, in particular those who do not have dedicated training in sleep medicine as we all continue to care for the CF patient population.

Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they may benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF are now subject to the same co-morbidities that may benefit from NIV as their age matched cohorts. NIV can improve gas exchange, quality of sleep, exercise tolerance and augment airway clearance in CF, and it is important that CF providers are comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation of NIV, and postulate a practical approach for CF clinicians to take fin monitoring patients on NIV. We will discuss aspects unique to people with CF and the use of NIV. We hope that this serves as a resource for CF providers, especially those of us who do not have dedicated training in sleep medicine, as we continue to care for our patient population.