Highly effective modulator therapies (HEMT) have transformed care for children with cystic fibrosis (CF) by improving lung function, reducing infections and supporting healthy growth. However, a recent study shows that obstructive sleep apnea (OSA) is still common – and may be more likely in children receiving HEMT.
In this retrospective study – Aarti Shakkottai, M.D., Pediatric Pulmonologist and Sleep Medicine Physician at Children’s Medical Center Dallas, part of Children’s HealthSM, and Assistant Professor at UT Southwestern – analyzed 12 years of sleep study data in children with CF. The findings challenge the idea that better breathing always means better sleep and highlight the need for routine sleep evaluation.
In this Q&A, Dr. Shakkottai discusses her findings and what they mean for health care providers who care for children with CF.
What led you to study sleep apnea in children with cystic fibrosis?
Many children with CF report sleep problems such as snoring, restless sleep and nighttime breathing issues. However, they are not routinely screened for sleep disorders. Limited data suggest OSA is more common in children with CF than in those without. However, how HEMT affects this risk remains unknown. We wanted to understand how common OSA is in children with CF and whether being on HEMT changes that risk.
What did your study find?
We reviewed sleep study data from 49 children with CF and found that 57% had a diagnosis of OSA. Tonsillar hypertrophy was a strong, independent risk factor for OSA.
We were surprised to find that children on HEMT were four times more likely to have OSA compared to those not on HEMT. These children had normal lung function and BMI, yet they were still at higher risk. In short, children who appear “well” by CF standards can still have significant sleep-disordered breathing.
Why might children on modulator therapy be more likely to have disturbed sleep?
That’s still unclear, but we have some theories. HEMT has led to major improvements in lung function and nutrition in children with CF. While lung function and BMI weren’t directly linked to increased OSA risk, subtle changes in body composition – such as fat deposits in the upper airway – may play a role.
We’ve also seen more cases of low iron stores in children with CF. Iron deficiency is associated with restless sleep, periodic limb movement disorder and sleep-disordered breathing. More research is needed to explore these possibilities.
When should providers consider referring a child with CF for a sleep study?
Ask about sleep issues during routine visits with children and teens who have CF – even those doing well on HEMT. Validated tools like the Pediatric Sleep Questionnaire can help screen for sleep disorders.
If there are concerns about sleep apnea during the clinical interview or physical exam, refer the child for a sleep study. Providers can also refer patients directly to the Children’s Health Sleep Disorders Clinic for further evaluation.
What are the risks of untreated sleep apnea in children who have cystic fibrosis?
Untreated sleep apnea is linked to problems with learning, memory and school performance. It also increases the risk for ADHD, anxiety, depression and poor cardiometabolic health. Children with CF are already at higher risk for many of these conditions, and comorbid sleep apnea may worsen these issues. Children with CF may also be more sensitive to the effects of poor sleep than those without CF.
How can providers help improve sleep for children with cystic fibrosis?
Providers should ensure children with CF get the recommended amount of sleep. Families should be encouraged to maintain a consistent sleep schedule on weekdays and weekends, and limit screen time at least one hour before bed.
Screening for iron deficiency and prescribing iron supplements may also help. OSA management in children with CF is similar to those without and may include referral to otolaryngology for possible adenotonsillectomy, or to the Sleep Disorders Clinic to begin positive airway pressure (PAP) therapy. Non-surgical options like intranasal steroids or montelukast may also be discussed.
What’s on the horizon for your research?
We are working to understand why children with CF on HEMT are at greater risk for sleep apnea and poor sleep. We’re exploring whether factors like age, genetics, therapy duration, weight gain or fat distribution play a role. Not every child on a modulator has sleep apnea, so we want to identify what sets those children apart.
What makes Children’s Health Pulmonology and the Sleep Disorders Program unique?
Our Sleep Disorders Program at Children’s Health and UT Southwestern is accredited by the American Academy of Sleep Medicine. As the largest pediatric sleep center in North Texas, our team includes experts in pulmonology, sleep medicine and psychology. Our 24-bed Sleep Studies Lab is staffed by technologists with deep expertise in respiratory therapy and neurophysiology.
We coordinate closely with referring providers, including those at the Claude Prestidge Cystic Fibrosis Center at Children’s Health, to identify sleep issues in children with CF and improve their health.
Learn more about our Sleep Disorders Program or refer a patient for a sleep study.
