Abstract
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.