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.
Abstract

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