A Pilot Study of Parent Education Intervention Improves Early Childhood Development Among Toddlers with Sickle Cell Disease

Background: Young children with sickle cell disease (SCD) are at high risk of cognitive delay. In a previous study, >50% of the infants and toddlers in our SCD clinic were significantly delayed (scaled scores 7 or below) in cognitive and language skills. These developmental delays were related to the home environment, but not to biologic risk factors associated with SCD. We hypothesized that a parent education program to encourage positive parent-child interactions would improve developmental outcomes.

Methods: Children with SCD, ages 1 - 36 months, living in stable housing within 30 miles of the hospital and with caregivers who spoke fluent English were enrolled over a three-year period in a pilot study of a single-arm behavioral intervention. Caregivers consented to participate in an accredited Parents as Teachers (PAT) Born to Learn curriculum, and to complete Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III) and the Home Observation for Measurement of the Environment (HOME) assessments. An occupational therapist, certified as a PAT provider, completed all services and assessments. Home visits were scheduled monthly, but participants could cancel or reschedule at their discretion. Participation continued until the child was older than 3.5 years, or the parent chose to exit, or at the end of the three-year pilot period. SCD measures were extracted from medical records upon enrollment in the study. Analyses were conducted using IBM SPSS Statistics (Version 22, Chicago, IL). Continuous data were analyzed using the Wilcoxon signed ranks test for comparisons over time.

Results: Thirty-five participants (20 male; 16 HbSS, 15 HbSC, 1 HbS beta thalassemia⁰, 1 HbS beta thalassemia⁺, 2 HbS with hereditary persistence of fetal hemoglobin) had at least 2 PAT visits with BSID-III assessments. The mean ages of children was 7.9 + 6 months (range, 1-34 months) and of primary caregivers was 25.2 + 5 years (range, 15-35). At baseline, the children's hematocrit was 28.0 + 3.5% (range, 22-39) and their pulse oximetry was 99 + 1.3% (range, 96-100). The median number of home visits was 12 (range, 2-41) over a median of 18 months (range, 2-32). None of the biological variables significantly correlated with the BSID-III domains, but the HOME scores correlated with cognition (r=.410, p=.014). While there was no significant difference in the pre/post HOME scores over time (pre: 29.2, post: 31.3, n=14; p=.161), statistically significant improvements occurred in the cognitive (p=.016) and expressive language domains (p=.002). Table I provides longitudinal BSID-III results.

Conclusion: Young children with SCD are at high risk for poor developmental outcomes. This is the first parenting intervention to specifically target parents of young children with SCD, and significant improvements occurred in the children's cognitive and expressive language domains. Further investigation is warranted to test the effect of implementation of this evidence-based intervention in a larger cohort of this vulnerable population.