Disease Management Decision-Making Amongst Caregivers of Children with Sickle Cell Anemia

Introduction: Sickle cell anemia (SCA) is the most common form of sickle cell disease and is associated with more severe complications in children. Disease management activities including adherence to medication regimens and health-promoting behaviors improve quality of life and minimize serious complications for children with SCA (e.g., stroke, premature morbidity or mortality). Parents’ and primary caregivers’ involvement in medical decision making is also critical to successful and timely management of pediatric SCA, as parents/caregivers hold primary disease-management responsibility during formative childhood years. Available research documents parental involvement in medical decision-making as integral to cultivating children’s long term self-efficacy in SCA disease management, yet the processes through which caregivers make SCA-related decisions remain unknown. As such, the current study examined caregivers’ decision-making processes and related priorities when managing their child’s SCA.

Methods: Parents and primary caregivers (N=28), hereafter referred to as caregivers, of children with SCA (ages 0-12 years old) were recruited from four outpatient pediatric hematology clinics in New England. The exploratory nature of this research and focus on uncovering processes was best suited to a qualitative approach. As such, participants completed individual semi-structured qualitative interviews exploring links between caregivers’ decision-making and both daily and ongoing disease management practices of their child’s SCA. Data were transcribed verbatim, cleaned, systematically coded, and analyzed using applied thematic analysis. Data collection continued until themes reached saturation.

Results: Participating caregivers were primarily mothers (84%), single (56%), publicly insured (60%) and identified as Black or African American (88%). Decision-making processes were driven by caregivers’ SCA knowledge, their disease management experience, and the child’s disease severity. Caregivers described their decision-making processes across three contexts: acute symptom management (e.g., hospitalizations, at-home management of pain crises), preventative disease management (e.g., adhering to medication regimens, being mindful of extreme temperatures) and treatment initiation or discontinuation (e.g., agreeing to chronic transfusions or starting hydroxyurea). Across all three contexts, caregivers described themselves as the primary decision-maker but noted conferring with their child’s healthcare providers, their partners, and other family members. The well-being of the child with SCA was prioritized in decision-making processes, as caregivers repeatedly reported making decisions based on their child’s SCA severity level (e.g., having recurring severe symptoms) and not wanting to worsen the child’s condition. Consequently, the prioritization of the child with SCA’s well-being often superseded household needs (e.g., prioritizing child’s medication over paying other bills). Caregivers reported relying on their SCA disease-knowledge and trust, or lack thereof, in their child’s SCA providers when making SCA-related decisions. Further, the weight placed on SCA-disease knowledge and trust in providers often depended on the child’s age and the caregiver’s experience to date with managing the child’s SCA needs. Caregivers reported that decision making became easier with more SCA knowledge and/or experience and with time, as did their trust in providers.

Discussion: Understanding how caregivers make SCA-related decisions is critical to identifying specific areas for development of targeted interventions and support. In this study, we found that caregivers’ decision making is often driven by their SCA disease knowledge, disease management experience and overall prioritized desire to protect and improve their child’s quality of life. Findings of this research should inform the development of various interventions (e.g., pamphlets, web-based platforms) to determine best approaches for providers to implement collaborative decision-making supports throughout childhood for children with SCA.