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Developmental Screening in Pediatric Sickle Cell Disease: Disease-Related Risk and Screening Outcomes in 4 Year Olds

Schatz, Jeffrey PhD*; Schlenz, Alyssa PhD*,†; Reinman, Laura MA*; Smith, Kelsey MA, MEd*; Roberts, Carla W. MD

Journal of Developmental & Behavioral Pediatrics: October 2017 - Volume 38 - Issue 8 - p 654–662
doi: 10.1097/DBP.0000000000000486
Original Articles

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.

*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.

Address for reprints: Jeffrey Schatz, PhD, Department of Psychology, University of South Carolina, Columbia, SC 29208; e-mail:

Disclosure: Portions of this work were supported by awards to J. Schatz from the Palmetto Health Foundation and to A. Schlenz by the National Heart, Lung, and Blood Institute (F31 HL108582). The remaining authors declare no conflict of interest.

Received November , 2016

Accepted June , 2017

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