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Published on: November 5, 2019
Polysomnographic characteristics of a referred sample of children with sickle cell disease
Valerie E Rogers1, Daniel S Lewin, Glenna B Winnie
1Center for Sleep and Respiratory Neurobiology, Division of Biobehavioral Health Sciences, University of Pennsylvania School of Nursing, Philadelphia, PA, USA. vrog@nursing.upenn.edu
Insights
Obstructive sleep apnea (OSA) and periodic limb movements in sleep (PLMs) are common in children with sickle cell disease (SCD). Hb SS genotype is linked to more severe nocturnal oxygen desaturation compared to Hb SC.
Area of Science:
- Pediatric Hematology
- Sleep Medicine
- Pulmonology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with potential systemic complications.
- Sleep disordered breathing (SDB) is increasingly recognized as a comorbidity in children with SCD.
- Understanding polysomnographic findings in this population is crucial for clinical management.
Purpose of the Study:
- To characterize polysomnographic parameters in children with SCD.
- To identify clinical correlates of SDB in this cohort.
- To compare sleep findings based on SCD genotype, age, and adenotonsillectomy status.
Main Methods:
- Retrospective review of 55 children (aged 2-18 years) with SCD (Hb SS and Hb SC genotypes).
- Analysis of polysomnography data for sleep disordered breathing evaluation.
- Statistical comparison of polysomnographic values across SCD genotypes, age groups, and adenotonsillectomy status.
Main Results:
- Obstructive sleep apnea (OSA) was diagnosed in 69% of children.
- Children with Hb SS genotype showed significantly lower SpO2 and higher T90 compared to Hb SC.
- Increasing age correlated with decreased SpO2, OAHI, and arousal indices.
- Periodic limb movements in sleep (PLMs) were observed in a subset of children, including those without OSA.
- Post-adenotonsillectomy, most children had OSA but with reduced severity of respiratory events and oxygen desaturation.
Conclusions:
- OSA and PLMs are prevalent in children with SCD.
- Hb SS genotype is associated with more significant nocturnal hypoxemia.
- Adenotonsillectomy may improve SDB parameters in children with SCD, though OSA can persist.
Study Objectives:
To describe polysomnographic parameters and their clinical correlates in a referred sample of children with sickle cell disease (SCD).
Methods:
This was a retrospective medical record review of 55 consecutive children aged 2-18 years with SCD (hemoglobin [Hb] SS and Hb SC genotypes) undergoing polysomnography for evaluation of sleep disordered breathing. Polysomnography values were compared between SCD genotypes, 4 age groups, and adenotonsillectomy status using descriptive and nonparametric statistics.
Results:
Obstructive sleep apnea (OSA) was diagnosed in 38/55 (69%) children. Polysomnographic parameters differed significantly between Hb SS and Hb SC genotypes only on arterial oxyhemoglobin saturation (SpO2; 95.2 +/- 3.8 vs. 98.0 +/- 0.8, respectively, p < 0.01) and percent of sleep time below SpO2 90% (T90; 8.0 +/- 22.0 vs. 0.01 +/- 0.02, respectively, p < 0.05). Increasing age was associated with decreasing SpO2 (rho = -0.282, p < 0.05), obstructive apnea-hypopnea index (OAHI; rho = -0.364, p < 0.01), total arousal index (rho -0.272, p < 0.05) and respiratory arousal index (rho = -0.349, p < 0.01). Periodic limb movements in sleep (PLM) averaged 4.7 +/- 8.8/h, with a PLM index > 5/h in 5/17 children without OSA. Post- adenotonsillectomy, 8/10 children had OSA, but compared to untreated OSA-positive children they had a lower mean OAHI (4.4 +/- 5.5 vs. 8.9 +/- 12.5) and a lower T90 (1.6 +/- 4.2 vs. 9.2 +/- 24.9).
Conclusions:
Both OSA and PLMs were common in children with SCD. Children with Hb SS experienced more severe nocturnal oxygen desaturation than did those with Hb SC. Post-adenotonsillectomy, most children had OSA, although they experienced fewer obstructive respiratory events and less severe nocturnal oxygen desaturation than did untreated OSA-positive children.
