Sleep and Breathing in Cystic Fibrosis

Sleep hypoxemia, hypoventilation, and cough occur in patients with CF, especially as pulmonary disease worsens. Sleep disturbance is common, with a significant number of patients reporting their sleep quality to be poor. Changes in neurocognitive function and daytime activation may be related to sleep loss. Improvements in gas exchange have been achieved during acute physiological and short-term interventional studies with oxygen breathing, while nocturnal noninvasive ventilation has been shown to maintain ventilation in REM sleep and reduce work of breathing. However, high-quality long-term trials examining the impact of these therapies on disease progression and daytime function are lacking.