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Sickle cell disease is the most common genetic disease in Guadeloupe. More than twenty years ago, a coordinated charge was initiated, including newborn screening and medical and psychological support. The pathological symptoms of this genetic disease are multiple and appear at different times. They can affect all organs, in particular the brain. Patients are often described as having executive function disorder. The functions affected are needed for learning and allowing adaptation to newness. Our main aim was to determine the consequences of sickle cell disease on intellectual and executive functioning. We measured, in a group of patients aged from 6 to 16 years, intelligence (WISC-V), some executive functions (NEPSY-II), and everyday executive difficulties, as perceived by their parents (BRIEF). We found low scores in each indicator of the WISC-V and the NEPSY-II. Parents mentioned executive difficulties in everyday life in the BRIEF. Identifying intellectual and executive disorders is a real issue. The procedure should allow progression in patient support by proposing appropriate and useful help for their learning and their social and professional integration.