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Psychosocial and Neurocognitive Aspects of Sickle Cell Disease
Sarah Helps1, Peter Fuggle2, Orlee Udwin1
1Mary Sheridan Centre for Child Health, 5 Dugard Way, Renfrew Road, London SE11 4TH, UK.
Insights
Sickle cell disease (SCD) affects children globally. While emotional issues are not more common, severe SCD increases risks for neurocognitive difficulties and cerebrovascular events, requiring psychological support.
Area of Science:
- Hematology
- Genetics
- Pediatrics
Background:
- Sickle cell disease (SCD) is the most common inherited blood disorder worldwide.
- It primarily affects individuals of African and African-Caribbean descent, presenting variable severity.
- Increased life expectancy in SCD patients necessitates focus on psychosocial and neurocognitive outcomes.
Purpose of the Study:
- To review literature on psychological and neurocognitive functioning in children with SCD.
- To highlight the increased risk of cerebrovascular events and neurocognitive issues in severe SCD.
- To outline the role of psychological interventions in improving healthcare for children with SCD.
Main Methods:
- Literature review of psychological and neurocognitive functioning in pediatric SCD.
- Analysis of research on emotional and behavioral disorders in children with chronic conditions.
- Examination of evidence linking severe SCD to cerebrovascular events and neurocognitive deficits.
Main Results:
- Recent research does not indicate increased rates of emotional/behavioral disorders in children with SCD.
- Children with severe SCD face significantly higher risks of cerebrovascular events.
- Neurocognitive difficulties are also a significant concern for children with severe SCD.
Conclusions:
- Psychological and neurocognitive functioning are critical aspects of care for children with SCD.
- Severe SCD poses specific risks that warrant targeted monitoring and intervention.
- Psychological input can substantially enhance healthcare delivery and outcomes for affected children and families.
Abstract:
Sickle cell disease (SCD) comprises a group of recessively inherited blood disorders and is the most common genetic disorder in the world (Embury et al., 1994). It is a chronic condition of variable severity that mainly affects people of African and African-Caribbean heritage. Over the last 20 years life expectancy has increased significantly, particularly for patients receiving western healthcare so that increasing attention has been directed to the psychosocial adaptation and neurocognitive profile of children and adolescents with SCD. Previously, research suggested that, like children with other chronic health conditions, children and adolescents with SCD were at increased risk for emotional and behavioural disorders. More recent research has not demonstrated increased rates of such difficulties. Increasing evidence suggests that children with severe disease are, however, at significantly increased risk for cerebro-vascular events and neurocognitive difficulties. This paper reviews the literature regarding the psychological and neurocognitive functioning of children with SCD and outlines a number of ways that psychological input may significantly contribute to more effective health care for these children and their families.
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