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Updated: Feb 24, 2026

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Developmental Screening in Pediatric Sickle Cell Disease: Disease-Related Risk and Screening Outcomes in 4 Year Olds
Jeffrey Schatz1, Alyssa Schlenz, Laura Reinman
1*Department of Psychology, University of South Carolina, Columbia, SC; †Department of Pediatrics, Medical University of South Carolina, Charleston, SC; ‡Department of Pediatrics, University of South Carolina, Columbia, SC.
Insights
Four-year-olds with sickle cell disease (SCD) and high-risk genotypes showed developmental delays. Early screening using parent reports and behavioral testing can identify children needing support for neurodevelopmental concerns related to SCD.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Hematology
Background:
- Early child development in sickle cell disease (SCD) shows links between disease risks and developmental status.
- These associations become more apparent by early elementary school age.
Purpose of the Study:
- To assess if biomedical risk factors for neurologic disease in 4-year-old children with SCD relate to developmental screening outcomes.
- To identify an intermediate age for screening preschoolers with SCD.
Main Methods:
- Seventy-seven 4-year-old children with SCD underwent developmental screenings.
- Screenings included child testing (Fluharty Preschool Speech and Language Screenings Test, 2nd ed.) and parent-report (Ages and Stages Questionnaire, 2nd ed.).
- Genotype and other biomedical variables were extracted from medical records.
Main Results:
- Children with higher-risk SCD genotypes performed worse on syntactic processing compared to lower-risk genotypes.
- High-risk genotypes were associated with significantly higher rates of positive developmental milestone screenings (52% vs. 12%).
- Screening outcomes correlated with transcranial Doppler ultrasound findings indicating cerebral blood flow.
Conclusions:
- Developmental screening at age 4 is a potential strategy for identifying preschoolers with SCD-related neurodevelopmental concerns.
- Both parent-reported developmental milestones and behavioral testing can aid in screening for children requiring follow-up.
- Early identification allows for timely intervention to address potential neurodevelopmental effects of SCD.
Objective:
Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age.
Methods:
Seventy-seven 4-year-old children with SCD (M = 4.5 yrs, SD = 0.3 yrs) completed developmental screenings at routine hematology visits using child testing (Fluharty Preschool Speech and Language Screenings Test, 2nd edition) and parent-report (Ages and Stages Questionnaire, 2nd edition) procedures. Genotype and other biomedical variables were coded from medical records.
Results:
Children with higher-risk SCD genotypes (n = 52) showed lower performance than children with lower-risk genotypes (n = 25) on a measure related to neurologic disease risk in older children (syntactic processing); genotype risk was also related to rates of positive screenings on parent-reported developmental milestones (52% positive screenings in high-risk genotypes vs 12% in low-risk genotypes). Screening outcomes were also related to transcranial Doppler ultrasound findings assessing cerebral blood flow.
Conclusion:
Developmental screening at age 4 may be a useful target age for identifying preschoolers with sickle cell-related neurodevelopmental concerns. Parent report of developmental milestones and behavioral testing each may have a role in screening for children in need of follow-up services to address potential neurodevelopmental effects from SCD.
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