Behavioral and emotional problems in children with sickle cell disease and healthy siblings: Multiple informants, multiple measures.

Background: Behavioral and emotional problems in children with sickle cell disease (SCD) may be related to disease factors, or to socio-demographic factors. The aim of this study was to investigate the prevalence of behavioral and emotional problems in children with SCD living in a Western European country, compared to healthy siblings (who were comparable in age, gender, ethnicity, and socio-economic status-SES), and to a Dutch norm population.

Methods: The Child Behavior Checklist (CBCL), Teacher Report Form (TRF) and Disruptive Behavior Disorders rating scale (DBD) were distributed among caregivers and teachers of 119 children with SCD aged 6-18 years and among caregivers and teachers of 38 healthy siblings.

Results: Questionnaires were returned by caregivers and/or teachers of 106 children with SCD and 37 healthy siblings. According to caregivers and teachers, children with SCD had more severe internalizing problems than healthy siblings and the norm population. According to teachers, subgroups of both children with SCD and healthy siblings had more severe externalizing problems than the norm population. Children with SCD had more difficulties than healthy siblings in terms of school functioning, showed less competent social behavior and tended to have more attention deficits.

Conclusions: Children with SCD are at increased risk of developing internalizing problems as a result of their disease. Subgroups of children with SCD are at increased risk of developing severe externalizing problems, which may either be related to socio-demographic factors, or to disease factors, such as neurocognitive deficits associated with cerebral infarction.