Determinants of retention in care of newborns diagnosed with sickle cell disease in Liberia: Results from a mixed-methods study of caregivers.

High-income nations have established that early diagnosis and preventive treatment reduces early deaths in sickle cell disease (SCD). However, in low-/middle-income countries where SCD is common, attrition from clinical care is common. Reasons for poor retention in care are multi-factorial and poorly understood. The objective of this study was to identify factors that influence caregiver decision-making around chronic health care needs of a child with SCD. We conducted an exploratory sequential mixed methods study of caregivers of children diagnosed with SCD during a newborn screening program in Liberia. Caregivers completed questionnaires and semi-structured interviews designed to identify drivers of health decision-making. Interviews were digitally recorded, transcribed, coded, and analyzed using semi-structured thematic analysis to identify themes. Data integration occurred by using quantitative results to expand and clarify the qualitative themes. Twenty-six caregivers participated in the study. The mean age of the child at the interview was 43.7 months. Five themes influencing health decisions were identified: grief, the importance of support networks, stigma, perceived benefits, and the burden of chronic disease. The five themes crossed multiple domains of a socioecological model and identified complex interactions between family, community, social and cultural norms, and organizational structures. This study highlights the importance of community awareness of SCD and appropriate health communication by healthcare workers. Healthcare decision-making is multifactorial and complex. These results provide a framework for improving retention in care. In a low-resource country such as Liberia, much can be done by leveraging existing resources and cultural practices.

The Consortium on Newborn Screening in Africa for sickle cell disease: study rationale and methodology.

Sickle cell disease (SCD) is a common condition within sub-Saharan Africa and associated with high under-5 mortality (U5M). The American Society of Hematology instituted the Consortium on Newborn Screening in Africa (CONSA) for SCD, a 7-country network of sites to implement standardized newborn hemoglobinopathy screening and early intervention for children with SCD in sub-Saharan Africa. CONSA's overall hypothesis is that early infant SCD screening and entry into standardized, continuous care will reduce U5M compared with historical estimates in the region. Primary trial objectives are to determine the population-based birth incidence of SCD and effectiveness of early standardized care for preventing early mortality consortium-wide at each country's site(s). Secondary objectives are to establish universal screening and early interventions for SCD within clinical networks of CONSA partners and assess trial implementation. Outcomes will be evaluated from data collected using a shared patient registry. Standardized trial procedures will be implemented among designated birth populations in 7 African countries whose programs met eligibility criteria. Treatment protocol includes administering antibacterial and antimalarial prophylaxis and standard childhood vaccinations against infections commonly affecting children with SCD. Infants with a positive screen and confirmation of SCD within the catchment areas defined by each consortium partner will be enrolled in the clinical intervention protocol and followed regularly until age of 5 years. Effectiveness of these early interventions, along with culturally appropriate family education and counseling, will be evaluated by comparing U5M in the enrolled cohort to estimated preprogram data. Here, we describe the methodology planned for this trial.