This study will examine sleep disordered breathing and sleep quality in participants (ages 12-25) 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 individuals (ages 12-25) 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 individuals (ages 12-25) 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 individuals (ages 12-25) 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 individuals (ages 12-25) diagnosed with sickle cell disease controlling for age, genotype, and social vulnerability. Assess the feasibility of an optical imaging tool (Speckle Contrast Optical Spectroscopy - Open-Motion 3.0) to measure cerebral blood flow and blood volume in patients diagnosed with sickle cell disease (ages 12-25). Assess the concordance between measurement of cerebral blood flow and volume using speckle contrast optical spectroscopy and arterial spin labeling brain MRI.
Interested participants will be consented in-person or virtually. Consented participants will be mailed the equipment (WatchPAT) to conduct the remote assessment. Additional questionnaires will also be sent via mail or email for the participant to complete prior to collecting data on sleep behaviors. After receiving the questionnaires and equipment, a virtual session will be conducted with the participant to provide education on how to use the equipment and complete the included questionnaires. Participants will wear the WatchPAT overnight for three days and data will be captured remotely. After wearing the devices for three consecutive nights, the participant will attend a research visit within approximately the next two weeks where they will complete performance based neurocognitive assessments, behavioral rating forms, and neuroimaging.
Study Type
OBSERVATIONAL
Enrollment
65
St. Jude Children's Research Hospital
Memphis, Tennessee, United States
RECRUITINGEvaluate the relationship between nocturnal oxyhemoglobin saturation (SpO2) and neurocognitive functioning (working memory and verbal comprehension) in individuals (ages 12-25) diagnosed with sickle cell disease.
A linear regression model will be used to assess the relationship between SpO2 and neurocognitive functioning after adjusting for age, genotype, and social vulnerability.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Assess differences in white matter integrity among individuals (ages 12-25) diagnosed with sickle cell disease with and without sleep disordered breathing.
This study will measure resting-state BOLD signal in SCD patients after assessing nocturnal oxyhemoglobin saturation. The primary endpoint is the whole brain BOLD response and functional connectivity among brain regions within the default mode, fronto-parietal, executive control, salience, sensorimotor, and other resting state networks. Then we will compare differences in white matter integrity based on the structural connectivity connectome for the working memory network, silent cerebral infarcts, neuroinflammation, and functional connectivity among individuals with and without sleep disordered breathing.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Assess differences in silent cerebral infarcts among individuals (ages 12-25) diagnosed with sickle cell disease with and without sleep disordered breathing.
This study will measure resting-state BOLD signal in SCD patients after assessing nocturnal oxyhemoglobin saturation. The primary endpoint is the whole brain BOLD response and functional connectivity among brain regions within the default mode, fronto-parietal, executive control, salience, sensorimotor, and other resting state networks. Then we will compare differences in white matter integrity based on the structural connectivity connectome for the working memory network, silent cerebral infarcts, neuroinflammation, and functional connectivity among individuals with and without sleep disordered breathing.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Assess differences in neuroinflammation, among individuals (ages 12-25) diagnosed with sickle cell disease with and without sleep disordered breathing.
This study will measure resting-state BOLD signal in SCD patients after assessing nocturnal oxyhemoglobin saturation. The primary endpoint is the whole brain BOLD response and functional connectivity among brain regions within the default mode, fronto-parietal, executive control, salience, sensorimotor, and other resting state networks. Then we will compare differences in white matter integrity based on the structural connectivity connectome for the working memory network, silent cerebral infarcts, neuroinflammation, and functional connectivity among individuals with and without sleep disordered breathing.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Assess differences in functional connectivity among individuals (ages 12-25) diagnosed with sickle cell disease with and without sleep disordered breathing after controlling for age.
This study will measure resting-state BOLD signal in SCD patients after assessing nocturnal oxyhemoglobin saturation. The primary endpoint is the whole brain BOLD response and functional connectivity among brain regions within the default mode, fronto-parietal, executive control, salience, sensorimotor, and other resting state networks. Then we will compare differences in white matter integrity based on the structural connectivity connectome for the working memory network, silent cerebral infarcts, neuroinflammation, and functional connectivity among individuals with and without sleep disordered breathing.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Assess differences in self- and caregiver-reported mood and pain severity among individuals (ages 12-25) diagnosed with sickle cell disease with and without sleep disordered breathing after controlling for age.
The general linear regression model is then used to assess the relationship of functional connectivity and to assess the relationship of self- and caregiver-reported mood and pain severity with sleep disordered breathing with controlling for age in the model. As a secondary analysis, we will also associate continuous obstructive and central AHI with functional connectivity and self- and caregiver-reported mood and pain severity using linear regression model with adjusting for age.
Time frame: Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
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