Abstract
STUDY OBJECTIVES: Neurocognitive deficits are present in youth with sickle cell disease (SCD) without notable neurological complications. Sleep quality and disturbances can negatively impact their neurocognition and quality of life. This study aimed to assess the relationship between sleep and neurocognitive outcomes in youth with SCD, considering disease-related risks, protective factors, socioeconomic status, and the impact of hydroxyurea (HU). METHODS: Participants were selected from a retrospective clinical database of youth with SCD. All youth completed either a polysomnography (PSG) or sleep questionnaire and a neuropsychological evaluation to assess cognitive skills, including verbal comprehension, working memory, cognitive flexibility (CF), attention, and processing speed (PS). RESULTS: The sample included individuals with sleep questionnaire ratings (n = 42) and individuals with PSG (n = 33). The primary aim indicated that CF decreased (B = -4.269, p < .001) and caregiver-rated executive dysfunction increased (B = 8.565, p = .020) for individuals with higher sleep-related impairments. In the exploratory aim, PSG data revealed that CF (B = -3.082, p = .005) and PS (B = -2.171, p = .008) decrease for each 1% increase in time spent under 90% oxygen saturation (pTST SpO2 < 90%). HU was not a significant moderator in the relationship between sleep and neurocognitive performance. CONCLUSIONS: The study's primary findings highlight that sleep-related impairments are significantly related to neurocognitive challenges in youth with SCD. Exploratory PSG analysis supports these findings, linking pTST SpO2 < 90% and cognition. Future research on sleep interventions is essential for improving neurocognitive functioning in this population. Statement of Significance This study highlights the significant impact of sleep-related impairments on neurocognitive outcomes in youth living with sickle cell disease (SCD) despite the absence of major neurological complications. An assessment of sleep quality and nocturnal oxygen saturation has a bidirectional impact on the disease course and neurocognitive outcomes of youth living with SCD. Neurocognitive challenges can have lasting effects on academic performance, transition readiness, and quality of life. Most research focuses on neurocognitive and disease-related effects of severe complications. This study gives insight into the impact and needs of youth living with SCD without major complications. Thus, addressing the risk of sleep-related impairments through targeted interventions may improve neurocognitive outcomes and reduce the impact of other health complications associated with SCD.