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Current developmental screening practices in young children with sickle cell disease.
Chibuzo J Aguwa1,2, Alicia D Cannon3, James F Casella4
1Neurodevelopmental Medicine, Kennedy Krieger Institute, Johns Hopkins University, Baltimore, Maryland, USA.
British Journal of Haematology
|December 1, 2022
Summary
Children with sickle cell disease (SCD) often miss crucial developmental screenings. This study highlights significant gaps in timely and appropriate neurodevelopmental disorder (NDD) surveillance for these vulnerable children.
Area of Science:
- Pediatric Hematology
- Neurodevelopmental Pediatrics
- Public Health
Background:
- Neurodevelopmental disorders (NDDs) can impact children with sickle cell disease (SCD).
- Current guidelines recommend regular developmental screening for children with SCD.
- Observed rates of NDDs in children with SCD appear lower than anticipated, suggesting potential underdiagnosis.
Purpose of the Study:
- To evaluate the adherence to developmental screening guidelines in children with sickle cell disease.
- To assess the frequency and appropriateness of developmental and autism-specific screenings in this population.
- To identify disparities in screening practices for children with SCD.
Main Methods:
- Retrospective chart review of 276 children with sickle cell disease.
- Analysis of the availability and completion rates of developmental and autism-specific screenings.
- Examination of the tools used and the age appropriateness of screenings conducted.
Main Results:
- Developmental screening was performed in 70% of charts, while autism-specific screening occurred in only 19%.
- Validated tools were utilized in 32% of general developmental screenings but 92% of autism-specific screenings.
- A significant proportion of children (57%) were screened outside the recommended age windows.
Conclusions:
- Children with sickle cell disease are not consistently receiving recommended developmental screening and surveillance.
- There are significant gaps in the systematic application of validated screening tools and adherence to age guidelines.
- Healthcare providers need to improve the implementation of developmental monitoring for children with SCD to ensure early detection of NDDs.

