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Sickle cell disease as a neurodevelopmental disorder.
Jeffrey Schatz1, Catherine B McClellan
1Department of Psychology, University of South Carolina, Columbia, South Carolina 29208, USA. schatz@sc.edu
Mental Retardation and Developmental Disabilities Research Reviews
|October 25, 2006
Summary
Sickle cell disease (SCD) affects the central nervous system (CNS), causing neurocognitive dysfunction in children. Understanding SCD as a neurodevelopmental disorder is key to improving outcomes and quality of life.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with significant central nervous system (CNS) implications.
- CNS involvement in SCD can lead to early-onset neurocognitive dysfunction and academic challenges in children.
- A substantial portion of children with SCD experience CNS effects, impacting cognitive domains.
Purpose of the Study:
- To characterize sickle cell disease (SCD) as a neurodevelopmental disorder.
- To review the mechanisms, timing, and manifestations of neurological morbidity in SCD.
- To explore the interplay between disease processes and social-environmental factors in SCD.
Main Methods:
- Review of existing literature on neurological complications in sickle cell disease.
- Analysis of mechanisms underlying CNS involvement in SCD.
- Examination of cognitive and behavioral outcomes in pediatric SCD patients.
Main Results:
- Neurological complications in SCD can manifest early in life, leading to neurocognitive deficits.
- Approximately 25-33% of children with SCD exhibit CNS effects impacting cognition and academics.
- Social-environmental factors, including stigma and socioeconomic status, exacerbate stress in families affected by SCD.
Conclusions:
- Sickle cell disease should be viewed as a neurodevelopmental disorder due to its profound effects on the CNS.
- Addressing the neurocognitive aspects of SCD is crucial for effective treatment and improved quality of life.
- Increased public awareness of SCD's neurocognitive impact is essential for better patient outcomes and support.
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