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

Cerebral Hemodynamics and Neurocognitive Functioning in Sickle Cell Disease

Category:

Psychology

Diseases Treated:

Non-therapeutic

Eligibility Overview:

This is a non-therapeutic clinical trial that is only open to St. Jude patients.

St. Jude Patients

  • 8 to 17 years old 
  • Sickle cell anemia 
  • Enrolled on the 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

Siblings

  • Siblings of St. Jude patients with sickle cell disease 
  • Do not have sickle cell disease 
  • 8 to 17 years old 
  1. 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

  2. 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
  3. SCDCogO2
    Sponsors: St. Jude Children’s Research Hospital
    ClinicalTrials.gov identifier  
    Trial Start Date: April 2022
    Estimated Enrollment: 20
    Study Type: Observational
    Ages: 8 to 17 years old
    Principal investigator: Andrew Heitzer, PhD, ABPP-CN
    Study Sites: St. Jude Children’s Research Hospital
    For a consultation or to discuss SCDCogO2: St. Jude Physician/Patient Referral Office
    1-888-226-4343
    referralinfo@stjude.org

St. Jude Children’s Research Hospital
262 Danny Thomas Place
Memphis, TN 38105  USA
Voice: 1-888-226-4343 or 901-595-4055
24-Hour Emergency Access Pager: 1-800-349-4334
Email: referralinfo@stjude.org

The above information is intended to provide only a basic description about a research protocol that may be currently active at St. Jude. The details made available here may not be the most up-to-date information on protocols used by St. Jude. To receive full details about a protocol and its status and or use at St. Jude, a physician must contact St. Jude directly.

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