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Department of Pediatrics, Mailman Center for Child Development, University of Miami Miller School of Medicine, Miami, Florida 33101, USA
Daniel Armstrong, Ph.D., Department of Pediatrics (D-820), University of Miami School of Medicine, P.O. Box 106280, Miami, FL 33101. Voice: 305-243-6801; fax: 305-243-5978. darmstrong{at}miami.edu
As more effective management and even cure of thalassemia become possible, attention is beginning to be directed to the potential neurologic and resulting neurocognitive effects of this illness on adults and children. Recent studies indicate that for adults with ß-thalassemia major and intermedia, and for children with sickle ß-thalassemia (Sß-thalassemia), there is a substantial risk for silent brain infarcts that may be associated with neurocognitive impairment similar to that reported for children with sickle cell anemia. Here the available literature in this area is reviewed and the limited outcomes are compared with those available from large, multicenter longitudinal studies of sickle cell anemia. On the basis of these comparisons, it is recommend that children with thalassemia be screened for specific neuropsychological impairments and that they be provided early intervention and special education access as available under the Individuals with Disabilities Education Act (IDEA) or the 504 Regulations of the Rehabilitation Act of 1973.
Key Words: thalassemia • neurocognitive • silent infarct • chronic anemia • educational intervention • neurodevelopment
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