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The burden of sleep disordered breathing in children with sickle cell disease

Ilaria Liguoro1, Michele Arigliani2, Hui-Leng Tan3

  • 1Department of Medicine, Division of Paediatrics, University Hospital of Udine, Udine, Italy.

Pediatric Pulmonology
|August 25, 2021
PubMed

Insights

Children with sickle cell disease (SCD) face higher risks of sleep disordered breathing (SDB). While SDB is linked to neurological and cardiac issues, its impact on pain crises and chest syndrome requires further study.

Area of Science:

  • Pediatric Hematology
  • Sleep Medicine
  • Pulmonology

Background:

  • Children with sickle cell disease (SCD) exhibit a higher prevalence of sleep disordered breathing (SDB) than the general pediatric population.
  • Research on SDB in pediatric SCD is expanding, but knowledge gaps persist regarding risk factors and clinical consequences.

Purpose of the Study:

  • To systematically review and synthesize current evidence on the epidemiology, clinical presentation, complications, and management of SDB in children with SCD.
  • To provide a comprehensive overview of SDB in this vulnerable pediatric population.

Main Methods:

  • A systematic electronic literature search was performed across PubMed/Medline, Scopus, and Cochrane databases for studies published between January 2000 and December 2020.
  • Inclusion criteria encompassed studies on SDB in children (0-20 years) with SCD, published in English. A quantitative synthesis and meta-analysis of treatment outcomes were conducted.

Main Results:

  • Sixty-two studies were included in the analysis. Sleep disordered breathing (SDB) in children with SCD is associated with adverse neurological, neurocognitive, and cardiological outcomes.
  • The association between SDB and the frequency or severity of vaso-occlusive pain events and acute chest syndrome remains unclear.
  • Interventions such as adenotonsillectomy or oxygen supplementation may improve nocturnal oxygen saturation, but their clinical effectiveness requires further investigation.

Conclusions:

  • Sleep disordered breathing is a significant concern in children with sickle cell disease, impacting neurological and cardiac health.
  • Further research is needed to clarify the relationship between SDB and specific SCD complications like pain crises and acute chest syndrome.
  • While some treatments show potential for improving oxygenation, their definitive clinical benefits in pediatric SCD patients with SDB warrant additional study.

Keywords:
childrennocturnal oxygen desaturationsickle cell diseasesleep apneasleep disordered breathing

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