Date of Award


Document Type

Open Access Dissertation



First Advisor

Jeffrey C. Schatz


Sickle Cell Disease (SCD) is a genetic disorder which affects hemoglobin and is associated with high rates of neurologic and neurocognitive deficits. Recent studies have indicated executive functioning (EF) as a common area of impairment for children diagnosed with SCD; however, there is no consensus about which measures of executive function are best to use in clinical practice or research. The purpose of the present research is to assess the properties of a new executive function measure, the “EXAMINER” to determine its utility with the SCD population. Thirty-two children with SCD and 86 demographically-matched controls completed established cognitive measures known to be sensitive to SCD-related neurocognitive deficits as well as EXAMINER tasks of executive attention, set shifting, working memory, inhibition, planning, and fluency. A statistical analysis compared performance on the attention, set shifting, inhibition, planning, and fluency measures in the EXAMINER relative to established measures (i.e., the verbal comprehension, processing speed, short term memory measures in the Woodcock Johnson Tests of Cognitive Abilities III) to compare reliability and validity. It was expected that the EXAMINER would show comparable reliability and validity to the established measures using traditional definitions of these constructs. In addition, examination of cultural validity was examined due to the high preponderance of SCD among the African-American population. Statistical analysis indicated support for internal consistency, convergent validity, cultural validity, and sensitivity of the EXAMINER, relative to traditional cognitive measures. Taken together, these results suggest that the EXAMINER is a useful neuropsychological test for the pediatric SCD population. Future research should focus on using more sophisticated scale development approaches, including greater ethnic and economic diversity with norming groups, and incorporating children in the statistical models to further establish strong psychometric properties.

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