Prevalence of Developmental Delay and Contributing Factors Among Children With Sickle Cell Disease

Catherine Hoyt Drazen, Regina Abel, Mohamed Gabir, Grant Farmer, Allison A. King

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


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.

Original languageEnglish
Pages (from-to)504-510
Number of pages7
JournalPediatric Blood and Cancer
Issue number3
StatePublished - Mar 1 2016


  • Cognition
  • Development
  • Home environment
  • Sickle cell disease


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