Peripheral Arterial Tonometry and Neurocognition in Sickle Cell Disease

Peripheral Arterial Tonometry and Neurocognition in Sickle Cell Disease

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.

Inclusion Criteria:

• Diagnosed with sickle cell disease of any genotype
• Participant in the Sickle Cell Clinical Research and Intervention Program
• Between 12-25 years of age at the time of enrollment
• English is the primary language
• Access to an electronic device with WiFi

Exclusion Criteria:

• History of an intellectual disability
• History of a traumatic brain injury or seizure disorder
• History of a stroke
• Undergoing potential curative treatment for SCD (stem cell transplant or gene therapy)
• Currently prescribed an intervention for a sleep disorder
• Inability or unwillingness of research participant or legal guardian/representative to give written informed consent.