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Thalassemia and learning: Neurocognitive functioning in children
1Department of Pediatrics, Mailman Center for Child Development, University of Miami Miller School of Medicine, Miami, Florida 33101, USA. darmstrong@miami.edu
Insights
Thalassemia can lead to silent brain infarcts and neurocognitive issues in children and adults. Early screening and intervention are recommended for children with thalassemia to address potential impairments.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Thalassemia management and cures are improving, shifting focus to long-term complications.
- Neurologic and neurocognitive effects are increasingly recognized in thalassemia patients.
Purpose of the Study:
- To review existing literature on neurologic and neurocognitive effects in thalassemia.
- To compare findings in thalassemia with those in sickle cell anemia.
- To recommend screening and intervention for affected children.
Main Methods:
- Literature review of neurologic and neurocognitive outcomes in thalassemia.
- Comparison of limited thalassemia data with large sickle cell anemia studies.
Main Results:
- Adults with beta-thalassemia major/intermedia and children with sickle beta-thalassemia face a significant risk of silent brain infarcts.
- These infarcts may correlate with neurocognitive impairment, similar to sickle cell anemia in children.
Conclusions:
- Children with thalassemia should be screened for neuropsychological impairments.
- Early intervention and special education access are crucial for children with thalassemia, guided by IDEA and Section 504 regulations.
Abstract:
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 beta-thalassemia major and intermedia, and for children with sickle beta-thalassemia (Sbeta-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.
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