Obstructive sleep apnea in children with cystic fibrosis on highly effective modulator therapy

Aarti Shakkottai1, Stephany Kim2, Ron B Mitchell3

  • 1Department of Pediatrics, Division of Pediatric Pulmonology and Sleep Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

PubMed

Insights

Obstructive sleep apnea (OSA) is common in children with cystic fibrosis (CF), even after highly effective modulator therapies (HEMT). Children on HEMT were older and more likely to have OSA, suggesting a continued need for screening.

Area of Science:

  • Pediatric Pulmonology
  • Sleep Medicine
  • Cystic Fibrosis Research

Background:

  • Obstructive sleep apnea (OSA) is a known complication in pediatric cystic fibrosis (CF) patients.
  • Highly effective modulator therapies (HEMT) have improved CF outcomes, but their impact on OSA frequency is unclear.

Purpose of the Study:

  • To determine the frequency of OSA in children with CF in the post-HEMT era.
  • To assess the association between HEMT use and OSA in this population.

Main Methods:

  • Retrospective review of polysomnography (PSG) data from 49 children (aged 0-18 years) with CF between 2012-2023.
  • Patients were categorized based on HEMT status.
  • Logistic regression analysis was employed to evaluate the association between HEMT and OSA.

Main Results:

  • OSA was diagnosed in 57% of the children studied.
  • Children on HEMT were older and had a significantly higher odds of OSA (OR=4.3, p=0.02).
  • Tonsillar hypertrophy was also independently associated with increased odds of OSA (OR=6.6, p=0.03).

Conclusions:

  • OSA remains a frequent diagnosis in children with CF, even after the introduction of HEMT.
  • While HEMT use was associated with older age and higher OSA likelihood, key health metrics like lung function and nutritional status were similar between groups.
  • Further prospective research is warranted to fully elucidate the relationship between HEMT and OSA in pediatric CF patients.
Abstract

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