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.