How Parents Navigate Hydroxyurea Adherence for Their Young Children with Sickle Cell Disease
Abigail S Robbertz, Aimee K Hildenbrand, Rogelle TA Hackworth, Charles T Quinn, Constance Mara, Alexis A Thompson, Sherif Badawy, Venee Tubman, Jean Raphael, Kim Smith-Whitley, Allison King, Cecelia Calhoun, Susan Creary, Patricia E Houston, Cynthia D Gipson, Lisa M Shook-Chiles, Lori CrosbyAbstract
Background
Despite the benefits, hydroxyurea adherence in pediatric patients with sickle cell disease (SCD) remains low (Creary et al., 2019, Reeves et al., 2023), which may indicate adherence barriers at the patient, family, and healthcare systems levels (Treadwell et al., 2022). Some adherence barriers have been frequently assessed (e.g., forgetting, worries about side effects), while others (e.g., who is responsible for medication administration and organization) have not been examined. As part of the Engaging Parents of Children with Sickle Cell Anemia and their Providers in Shared-Decision Making for Hydroxyurea (ENGAGE-HU) trial (NCT03442114), the study’s community advisory board indicated that it was important to better understand adherence in the context of shared decision-making. This insight prompted the development of an exploratory aim to examine contextual factors influencing hydroxyurea adherence for young children with SCD.
Methods
A subset of caregivers (n = 21) of children between the ages of 0-5 years old with SCD who were enrolled in the larger trial opted-in to complete the medication adherence subscale of the medical adherence measure (MAM) at their follow-up study visit. This subscale consists of 9-items that ask about medication taking behaviors, and barriers and supports for adherence. Descriptive statistics were used to contextualize the sample and describe medication adherence.
Results
Caregivers (90.5% mothers, 4.8% grandmother, and 4.8% father) completed the MAM based on their child’s (52.4% male, 47.6% female) hydroxyurea prescription. Most children were between the ages of 0-2 years old (90.5%) and all were Black with severe SCD (genotype HbSS). On average, caregivers reported that their children missed a hydroxyurea dose once per week and take it late twice per week. Between 0 (hardly take/usually misses) and 10 (always takes/rarely misses), caregivers reported an average score of 7.8 (SD = 3.4). The most common reason for missing was running out of the medication/did not refill it (28.6%) followed by forgetting (23.8%). Most (95%) reported the child’s mother was responsible for ensuring there was enough medication and ordering more; however, fathers (28.6%), siblings (4.8%), grandparents (9.5%), and aunts/uncles (n = 2, 9.5%) were also noted by participants as responsible. Caregivers also reported varied methods hydroxyurea was stored or organized, including in the refrigerator (42.9%), on a special shelf/cabinet (33.3%), in a plastic bag (28.6%), in my room (19.0%), in a pill box (9.5%), or having no system (9.5%). On average, people had one storage/organization method (M = 1.4, SD = .93) with some up to five methods.
Conclusions
Results suggest that there are significant variations in how caregivers and families store and organize as well as allocate responsibility for refilling hydroxyurea that could impact adherence. For instance, having multiple caregivers responsible for hydroxyurea management could be an indicator of family support (strength) or diffusion of responsibility that contributes to adherence challenges. These preliminary findings underscore the importance of assessing contextual adherence barriers and facilitators. A personalized adherence plan that includes how to organize and allocate responsibility within a family may have potential to improve adherence and should be explored in future studies using multimodal methods of adherence assessment (e.g., electronic monitors, refill records, to self-report).