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The Relationship Between Executive Functioning And Adaptive Functioning In Pediatric Sickle Cell Disease

Principal Investigator:
Andrew
Zabel

Little is known about the relationship between executive functioning in children and adolescents with sickle cell disease (SCD). Similar to other chronic illnesses, children and adolescents with SCD engage in a greater number of daily adaptive tasks, particularly medical self-care tasks, compared to typically-developing children and adolescents. Further, those with SCD are at increased risk for attention and executive functioning difficulties because of the neurological implications of the disease (e.g., stroke, chronic deoxygenation to the brain). Individuals with difficulties with attention and executive functioning are also likely to have difficulties with the independent completion of daily tasks. Individuals with SCD are at particular risk because not only do they have executive functioning difficulties but they also have greater adaptive demands, secondary to their illness. However, the relationship between executive functioning and adaptive functioning in this unique population has not been studied. Because adolescents with SCD present with difficulties in transitioning to adult care, it is important to understand how impairments in executive functioning along with increased self-care demands can impact this transition.

This study aims to better describe the relationship between executive functioning and adaptive functioning in a sample of children and adolescents with SCD. More specifically, can neurocognitive measure of executive functioning predict one’s general adaptive functioning skills, above and beyond other variables (e.g., illness severity, general intellectual functioning, other cognitive abilities)? Second, is a similar pattern observed when attempting to predict independence in completing medical self-care skills? Hierarchical linear regression will be utilized to address these questions.The study from which these data were obtained was conducted at Drexel University College of Medicine/St. Christopher’s Hospital for Children (DUCOM/SCHC) as part of one of the study member’s (Dr. Kelly Jones) doctoral research. The study is now in the phase of data analysis and manuscript preparation, and Dr. Jones will continue to collaborate with the principle investigator at DUCOM/SCHC, Dr. Reem Tarazi. In order to assist with data analysis and manuscript preparation, Dr. Tarazi will provide Dr. Jones with a database. All information in the database will be deidentified and Dr. Jones will not have access to any files with identifying information. A copy of the original IRB approved by DUCOM/SCHC is included in this submission for review.

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