Sickle Cell Disease Phenotypes and Obstructive Sleep Apnea; Are They Related?

Area of Science:

• Pediatric Sleep Medicine
• Hematology
• Genetics

Background:

• Sickle cell disease (SCD) is a genetic blood disorder with varying clinical manifestations across different ethnic groups.
• Obstructive sleep apnea (OSA) is a common comorbidity in children with SCD, impacting their health and quality of life.
• Phenotypic differences in SCD, such as Arab-Indian and Benin types, may influence the prevalence and severity of associated complications like OSA.

Purpose of the Study:

• To compare polysomnographic features between children with sickle cell disease (SCD) of the Arab-Indian and Benin phenotypes.
• To investigate potential differences in the prevalence and severity of obstructive sleep apnea (OSA) between these SCD phenotypes.

Main Methods:

• A prospective cross-sectional study was conducted at a children's hospital.
• Children diagnosed with SCD were recruited from pediatric hematology and sleep medicine clinics.
• Polysomnography was used to assess sleep features and diagnose OSA.

Main Results:

• Eighty-four children with SCD were included (median age 9 years).
• Obstructive sleep apnea (OSA) was more prevalent in the Benin phenotype (66.7%) compared to the Arab-Indian phenotype (35.2%) (p=0.006).
• Moderate to severe OSA was significantly higher in the Benin phenotype (56.7%) versus the Arab-Indian phenotype (18%) (p=0.0003).
• The odds of having OSA were 4.68 times higher in the Benin phenotype compared to the Arab-Indian phenotype.

Conclusions:

• The Benin phenotype of SCD is associated with a higher risk and greater severity of obstructive sleep apnea (OSA) compared to the Arab-Indian phenotype.
• These findings suggest that factors beyond sickle cell disease itself may contribute to the increased prevalence of OSA in the Benin phenotype.
• Further research is warranted to identify these additional risk factors and develop targeted interventions.