Utilization of Early Intervention Among Children with Sickle Cell Disease

Background:

The most prevalent monogenic disorder among children is sickle cell disease (SCD). SCD is the most common cause of pediatric stroke and is primarily inherited by people that identify as Black or of African descent.^1,2 Children with SCD have an elevated risk for delayed development and poor academic outcomes.² Our previous findings identified that 50% of children with SCD have a developmental delay, however, <5% were receiving therapy services.^3-5 Home-based programs have ameliorated cognitive and language delays in children with SCD.⁶ In the United States, home-based therapy services (e.g., occupational, physical, and speech therapy) are provided through a federally mandated program called Early Intervention for free or reduced cost for children with developmental delay <3 years of age.⁷ Unfortunately, this program is underutilized, especially in underserved and minoritized communities, with as few as 2% of eligible children receiving services.^8,9

Hematologists, who are tasked with providing ongoing medical care for young children with SCD, often lack the resources/tools needed to incorporate developmental screening into standard care. Thus, early indicators of developmental delay remain undetected and untreated, costing children and families precious time to intervene early.^6,10,11 This study aimed to assess the use of early intervention and determinants affecting participation in developmental screening and intervention among caregivers of children 0-4 years of age with SCD.

Methods: We recruited caregivers with children under 4 years of age with SCD from clinics in St. Louis, Missouri and Memphis, Tennessee. Semi-structured interviews based on the Reach-Effectiveness-Adoption-Implementation-Maintenance (RE-AIM) framework were used to describe determinants for referral success and describe the mechanisms (barriers and facilitators) that influence early intervention program utilization.¹² Interviews included open-ended to address topics related to reach, perceived effectiveness, and likelihood of adoption of early intervention if recommended. Additional questions assessed caregiver knowledge of developmental delays in SCD and utilization of screening and intervention.

Results: Among 127 caregivers identified as eligible, 47 consented and 20 completed an interview between September 2022 and February 2023. Similar to prior studies, over half of caregivers reported living in a low-income household, over half of children had hemoglobin SS and all participants identified as Black or African American. Interviews lasted between 15-75 minutes (mean = 34). Three themes emerged from the data including 1) Caregivers have not been informed about elevated risk for developmental delay, 2) Caregivers prioritize connecting with their provider and 3) Caregivers had not been adequately informed about early intervention services (Table 1).

Conclusions: This study highlights the low utilization of early intervention services for young children with SCD. Caregivers expressed frustration with the lack of awareness regarding the elevated risk for developmental delays and prioritize that information coming from their specialty providers (e.g., sickle cell clinic). Additionally, inadequate information about available services contributes to underutilization. As healthcare providers, we are tasked with increasing awareness about the associated risks of SCD, integrating developmental screening into standard care, and providing caregivers with information that could improve developmental outcomes for children with SCD.