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Peripheral Arterial Tonometry and Neurocognition in Sickle Cell Disease

This study will examine sleep disordered breathing and sleep quality in participants (ages 12-18) diagnosed with sickle cell disease of any genotype. We will utilize remote peripheral arterial tonometry (PAT) and questionnaires to evaluate difficulties with sleep. PAT assessments will occur remotely in the homes of participants. Neurocognitive, behavioral, and neuroimaging evaluations will occur on the same day as a routine clinic visit. Primary Objective: Evaluate the relationship between nocturnal oxyhemoglobin saturation (SpO2) and neurocognitive functioning (working memory and verbal comprehension) in children (ages 12-18) diagnosed with sickle cell disease controlling for age, genotype, and social vulnerability. Secondary Objective: Assess differences in white matter integrity, silent cerebral infarcts, neuroinflammation, and functional connectivity among children (ages 12-18) diagnosed with sickle cell disease with and without sleep disordered breathing after controlling for age. Assess differences in self- and caregiver-reported mood and pain severity among children (ages 12-18) diagnosed with sickle cell disease with and without sleep disordered breathing after controlling for age. Exploratory Objectives: Explore the relationship between nocturnal oxyhemoglobin saturation (SpO2) and neurocognitive functioning (attention, processing speed, verbal memory, visual memory, motor dexterity) in children (ages 12-18) diagnosed with sickle cell disease controlling for age, genotype, and social vulnerability. Assess the feasibility of an ultraportable ring oximeter (BodimetricsCircul+ Ring) in children (ages 12-18) diagnosed with sickle cell disease. Assess the concordance between the Circul+Ring with the WatchPAT in children (ages 12-18) diagnosed with sickle cell disease.

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