Sleep, Fatigue and Neurodevelopmental Outcomes in Pediatric Sickle Cell Disease

Background: Children with sickle cell disease (SCD) experience neurodevelopmental decline over time. They also tend to have short duration, poor quality sleep and elevated fatigue levels. This study aimed to describe sleep and fatigue in children and adolescents with SCD and their association with neurodevelopmental measures. Methods: Participants included 19 children and adolescents with SCD, aged 7-18 years, recruited from a tertiary care SCD clinic. The majority were referred for neurodevelopmental testing due to academic or behavioral difficulties. Parents completed the Behavior Rating Inventory of Executive Function (BRIEF). Participants completed the Wide Range Achievement Test (WRAT) and wore an actigraph for one week. Both completed the PedsQL Multidimensional Fatigue scale. Results: Short duration or poor quality sleep were identified in every participant. Fatigue was common. Eighteen participants had at least one abnormal result of the six neurodevelopmental subscales administered. No sleep measure was associated with any neurodevelopmental measure. Greater parent-proxy reported cognitive fatigue was associated with lower WRAT reading (p=0.041), spelling (p=0.002) and math (p=0.010) scores, and with poorer scores on two BRIEF subscales. Greater reported parent-proxy general fatigue was associated with elevated scores on all three BRIEF subscales. Conclusion: Fatigue more importantly than sleep appears to be related to deficits in academic skills and executive function in a clinical sample at risk for neurodevelopmental problems. Larger studies are needed to better define the effects of sleep and fatigue on specific aspects of cognition and behavior in children and adolescents with SCD across early development.