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Updated: Mar 30, 2026
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Prevalence of Developmental Delay and Contributing Factors Among Children With Sickle Cell Disease
Catherine Hoyt Drazen1, Regina Abel1, Mohamed Gabir1
1Program in Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri.
Insights
Young children with sickle cell disease (SCD) often experience developmental delays. Positive home environments correlate with better cognitive development in these children, suggesting a target for interventions.
Area of Science:
- Pediatric Health
- Developmental Psychology
- Hematology
Background:
- Children with sickle cell disease (SCD) face higher risks of cognitive deficits.
- Previous studies focused on disease severity, neglecting the home environment's role.
- Early development in young children with SCD requires further investigation, considering environmental influences.
Purpose of the Study:
- To investigate the developmental trajectories of young children with SCD.
- To assess the impact of environmental and family factors on development in children with SCD.
- To identify potential intervention targets for improving developmental outcomes.
Main Methods:
- A prospective, single-center cohort study evaluated children with SCD aged 1-42 months.
- Developmental assessments used the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).
- Home environment was assessed using the Home Observation for Measurement of the Environment (HOME) during home visits.
Main Results:
- Over 50% of children with SCD showed below-average scores in cognition and expressive language.
- No association was found between SCD severity and BSID-III developmental scores.
- Positive correlations were observed between socioeconomic status and the home environment, and between the HOME score and cognitive development.
Conclusions:
- Developmental delays are prevalent in young children with SCD, necessitating identification of modifiable factors.
- The home environment presents a promising target for interventions to enhance developmental progress.
- Further research is needed to establish the efficacy of home-based interventions for this population.
Background:
Children with sickle cell disease (SCD) are at higher risk for deficits in cognition compared to the general population, even at young ages. Disease severity has been co-assessed in earlier studies, but the home environment has not. The purpose of the current study was to investigate the development of young children with SCD and secondarily, the impact of environmental and family factors.
Methods:
The current study is a baseline cross-sectional evaluation of a prospective, single-center cohort. Children with SCD between the ages of 1 and 42 months and their primary caregiver were included. Participants lived within 30 miles of the site and spoke English. Children underwent developmental evaluation using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III). Home visits were completed and screened using the Home Observation for Measurement of the Environment (HOME).
Results:
Over 3 years, 43 caregiver-child dyads consented and participated. Over 50% of children scored significantly below average on cognition and expressive language subscales. SCD severity was not associated with BSID-III scores. Socioeconomic status (SES) determined by the Diez-Roux method positively correlated (r = 0.401, P < 0.01) with the home environment. The HOME correlated (r = 0.360, P < 0.05) with the cognitive subscale on the BSID-III.
Conclusions:
Given the high prevalence of developmental delay in this population, identifying modifiable factors to maximize developmental progress is essential. The home environment would be a targeted method for intervention. Future research is needed to identify the benefits of home-based intervention for this population.
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