Peripheral Arterial Tonometry and Neurocognition in Sickle Cell Disease
NCT Number: NCT06477289
Status: RECRUITING
Study Type: OBSERVATIONAL
Conditions: Sickle Cell Disease
Sponsor: St. Jude Children's Research Hospital
Sex: ALL
Age: CHILD, ADULT
Enrollment: 65
Study Design: Observational Model: |Time Perspective: p
Start Date: 2024-09
Completion Date: 2028-06
Locations: St. Jude Children's Research Hospital, Memphis, Tennessee, 38105, United States

Summary:
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.

Primary Outcome Measures: 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., A linear regression model will be used to assess the relationship between SpO2 and neurocognitive functioning after adjusting for age, genotype, and social vulnerability., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Secondary Outcome Measures: Assess differences in white matter integrity among children (ages 12-18) 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 children with and without sleep disordered breathing., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|Assess differences in silent cerebral infarcts among children (ages 12-18) 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 children with and without sleep disordered breathing., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|Assess differences in neuroinflammation, among children (ages 12-18) 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 children with and without sleep disordered breathing., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|Assess differences in functional connectivity among children (ages 12-18) 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 children with and without sleep disordered breathing., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|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., 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., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.
Other Outcome Measures: 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, A linear regression model will be used to assess the relationship between SpO2 and neurocognitive functioning after adjusting for age, genotype, and social vulnerability, Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|Assess the feasibility of an ultraportable ring oximeter (BodimetricsCircul+ Ring) in children (ages 12-18) diagnosed with sickle cell disease., Feasibility of the Circul+Ring equipment will be defined as more than 50% of participants wearing Circul+Ring for at least one night's sleep. There is no power calculation needed for this feasibility objective. However, 55 participants give 92% power to detect a 20% difference at an assumed 70% of participants who will wear the ring for at least one night's sleep, with a significance level of 0.05 based on a one-sided test. The number of hours worn for the WatchPAT and Circul+Ring will be compared using Wilcoxon rank sum test. Descriptive statistics will be used to summarize the two-question survey., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.|Assess the concordance between the Circul+Ring with the WatchPAT in children (ages 12-18) diagnosed with sickle cell disease., To evaluate concordance, the nocturnal SpO2 obtained using the WatchPAT and a Circul+ Ring will be plotted against each other, and Pearson/Spearman correlations will be used to examine their relationship. The Bland-Altman method will be used to investigate the variability between the two measurements using the WatchPAT and Circul+ Ring. Lin's concordance correlation coefficient will be determined to demonstrate the level of agreement., Baseline remote sleep assessment over 3 days followed by in-clinic assessment.