SCDCogO2: Analyzing Oxygen Saturation and Neurocognitive Functioning in Patients with Sickle Cell Disease

Brief Summary

This study evaluates the effectiveness of functional near-infrared spectroscopy (fNIRS) as a tool to measure brain oxygen saturation and cognitive function in patients with sickle cell disease. The predominant cause of morbidity in sickle cell anemia is vaso-occlusion associated with significant injury to organs throughout the body, including the brain. Low baseline hemoglobin and low peripheral oxygen saturation are associated with increased risk of silent cerebral infarcts, white matter damage, and declines in neurocognitive performance. However, there is minimal research assessing cerebral oxygen saturation in sickle cell anemia.

Near-infrared spectroscopy has the potential to be used as a point-of-care test to measure cerebral oxygen saturation and risk for neurocognitive decline. In this study, we aim to measure the ability of near-infrared spectroscopy to detect differences in cerebral oxygen saturation and cortical activation in patients with sickle cell anemia compared to controls.

Patients and siblings will take part in a one-time assessment to measure cognitive skills (e.g., vocabulary, sustained attention, working memory, and processing speed) and functional nearinfrared spectroscopy. Parents will also complete a basic demographic questionnaire.

Primary Objective

Estimate differences in cortical activation (oxygenated hemoglobin response) among patients with sickle cell anemia and healthy controls during a visual stimulation task.

Eligibility Criteria

Inclusion criteria for patients include:

• 8 to 17 years old 
• Primary diagnosis of sickle cell Anemia 
• Enrolled on Sickle Cell Clinical Research Intervention Program (SCCRIP) clinical trial 
• Completed Sickle Cell Assessment of Neuropsychology Skills (SCANS) assessment 
• Being treated with hydroxyurea (maximum tolerated dose) 
• Read and speak English 

Exclusion criteria for patients include:

• Significant impairment in global intellectual functioning 
• Received transfusion treatment within the past 3 months 
• Received treatment with psychostimulant or psychotropic medication within the past 2 weeks 
• History of documented central nervous system injury, including a traumatic brain injury, Moya Moya disease, or overt stroke 
• Treated with voxelotor, mitapivat, or etavopivat within the past 3 months 

Inclusion criteria for siblings include:

• Siblings of St. Jude patients with sickle cell disease 
• Do not have any form of sickle cell disease 
• 8 to 17 years of age 

Exclusion criteria for siblings include:

• Significant impairment in global intellectual functioning 
• History of documented CNS injury or disease 
• History of documented attention deficit hyperactivity disorder (ADHD—diagnosed by MD only) 
• Currently being treated with psychostimulant or psychotropic medication (within the past 2 weeks) 
• Sensory or motor impairment that would preclude valid cognitive testing 
• History of documented central nervous system injury or neurological disorder 

Study Sites

St. Jude Children’s Research Hospital, Memphis, Tennesse

About this study

This study uses functional near-infrared spectroscopy (fNIRS) to measure brain oxygen use and brain activity in patients with sickle cell anemia. Scientists want to find out if fNIRS can find differences in:

• Brain activity 
• How the brain uses oxygen 

Scientists will compare the results from patients and their siblings. This will help us understand how well this technology works.

Patients and siblings will take a test that measures thinking skills such as language, attention and memory. During the tests, they will wear a cap with wires that connect to a laptop computer. After the cap is removed, they will do a final set of tasks on the computer.

Parents will answer questions on a form.

Details that scientists learn from this study may help other patients in the future.

Purpose of this clinical trial

This study aims to find out if fNIRS can find differences in brain oxygen use and brain activity in patients with sickle cell disease. We will compare results from these patients with results from their siblings.

Eligibility overview

This clinical trial is open only to patients at the St. Jude Children’s Research Hospital and their siblings.

St. Jude Patients

• 8 to 17 years old 
• Sickle cell Anemia 
• Treated with hydroxyurea (maximum tolerated dose) 
• Enrolled in the Sickle Cell Clinical Research Intervention Program (SCCRIP) clinical trial 
• Have completed Sickle Cell Assessment of Neuropsychology Skills (SCANS) assessment 
• Read and speak English 

Siblings

• Siblings of St. Jude patients with sickle cell disease 
• Do not have sickle cell disease 
• 8 to 17 years old