title: Prevalence of Developmental Delay and Contributing Factors Among Children With Sickle Cell Disease.
abstract: 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.
authors.affiliation: Program in Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Catherine Hoyt
authors.initials: CH
authors.lastname: Drazen
authors.affiliation: Program in Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Regina
authors.initials: R
authors.lastname: Abel
authors.affiliation: Program in Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Gabir
authors.affiliation: Division of Public Health Sciences, Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Grant
authors.initials: G
authors.lastname: Farmer
authors.affiliation: Program in Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri.|Division of Public Health Sciences, Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.|Department of Pediatrics Hematology/Oncology, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Allison A
authors.initials: AA
authors.lastname: King
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology*; D003430:Cross-Sectional Studies; D002658:Developmental Disabilities / Q000209:etiology*; D005190:Family; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies
keywords: cognition; development; home environment; sickle cell disease