Children at preschool
Pre school children wearing protective face masks indoors at kindergarten.

Preschool children with sickle cell disease (SCD) have poorer working memory and school readiness compared with healthy children, according to a study published in the British Journal of Haematology. 

SCD predisposes children to cerebral infarcts, nutritional deficits, and growth delays. Combined, these can lead to neurobehavioral problems that disrupt their cognitive and academic skills. 

Studies show that school-aged children with this condition demonstrate cognitive decline that increases with age. This manifests as higher grade retention rates and poorer performance in standardized tests. 

“To identify these cognitive and academic difficulties early in life and facilitate treatment, it is important for hematologists to recognise neurobehavioural problems in SCD and facilitate care through a multidisciplinary approach,” the authors wrote. 

Read more about SCD epidemiology 

The authors aimed to characterize the working memory and school readiness skills in preschool children with SCD. They recruited patients seen at St. Jude Children’s Research Hospital who were diagnosed with SCD between 48 and 68 months of age (n=84). For every patient diagnosed with SCD, the research team prospectively matched 2 control participants (n=168). Control participants did not have SCD or any major chronic medical conditions.  

The researchers used the Stanford-Binet Intelligence Scales-Fifth Edition to measure working memory, which covers verbal and visual working memory. School readiness was measured using the Bracken Basic Concepts Scale-Third Edition: Receptive and the School Readiness Composite, which tests fundamental preacademic skills such as counting, the recognition of colors and letters, and size comparisons. 

The results of the study indicate that preschool children with SCD scored more poorly than their healthy counterparts in both measures. In addition, they discovered that working memory was associated with school readiness. The results of this study demonstrate how early neurocognitive dysfunction in young children with SCD puts them at an academic disadvantage compared with their peers. 

“There is a significant need to test and implement interventions incorporating early executive aspects of school readiness and parent education to prevent future academic deficits in young children diagnosed with SCD,” the authors concluded. 


Heitzer AM, Schreiber JE, Yuan X, et al. Working memory and school readiness in preschool children with sickle cell disease compared to demographically matched controlsBr J Haematol. 2022;10.1111/bjh.18507. doi:10.1111/bjh.18507