Future Directions
The sleep community is aware of the long-term consequences of untreated sleep-disordered breathing. Although few articles specifically look at untreated sleep apnea in the CF population, it is reasonable to extrapolate that the CF population is at risk for these consequences as well. Gastroesophageal reflux is associated with sleep-disordered breathing68, and this has well known effects on failure to thrive in the pediatric CF population. Cardiovascular complications such as hypertension, coronary artery disease, and stroke have been described in adults22 2324. Sleep-disordered breathing in other chronic lung disease patients has been associated with the development of sequelae such as pulmonary hypertension, hyperlipidemia, insulin resistance, increased susceptibility to certain infections, and increased inflammatory markers.69-75Autonomic dysfunction in sleep apnea is associated with impaired glucose tolerance25, and the implications of the development of diabetes is well known to the CF provider community. All of these sequelae are known to affect the quality of life in CF patients76.
More research in CF specific sleep-disordered breathing is necessary to understand the natural course of sleep across the lifespan. This information could assist the CF community to develop best practices in screening, diagnosis, and treatment for sleep disordered breathing. The timing in disease course and lifespan to best implement screening tools for sleep is unknown. The impact of modulator therapy is expected to be beneficial for pulmonary outcomes, which should reduce rates of sleep-disordered breathing. However, modulators may also increase rates of obesity, and then paradoxically increase rates of sleep-disordered breathing. The long term sequelae of undiagnosed and therefore untreated sleep-disordered breathing may not be available to us at this time, but studies looking at the impact of improved screening and effective treatment evaluating outcome measures such as quality of life, improved lung function, CF exacerbations and hospitalizations, and development of chronic medical conditions would be highly informative in guiding management of these patients. Large studies such as this will inform future CF care, potentially leading to screening guidelines for sleep-disordered breathing.