Cost-effectiveness of interventions for screening and management of sickle cell disease during pregnancy: A systematic review.

Background: Sickle cell disease (SCD) is a widespread inherited condition that has increased in global prevalence by 41% since 2000. Despite advancements in understanding the pathophysiology and clinical management of SCD, limited evidence exists regarding the economic implications of interventions for managing the disease during pregnancy. Aim: The World Health Organization is developing its first global clinical practice guidelines for the management of SCD during pregnancy. This systematic review aims to evaluate the cost-effectiveness of interventions for the screening and management of SCD during pregnancy to inform guideline development. Methods: We searched MEDLINE, Embase, CINAHL, PsycINFO, Global Index Medicus, NHS Economic Evaluation Database, and EconLit for articles published from inception to December 4, 2024, using key terms related to pregnancy, SCD, and economic evaluation. We included full economic evaluations on any interventions for the detection, treatment, and/or management of SCD during pregnancy. Data on the study characteristics, intervention, comparator, and key economic and health outcomes were extracted. Results were reported narratively, with conversions of costs to 2024 US dollars where applicable. The methodological quality of the included studies was assessed using the Consensus on Health Economic Criteria (CHEC) extended checklist. Results: Three studies published between 1999 and 2010 were included, all of which focused on screening strategies as interventions. No evaluations of other SCD management strategies were identified. There was considerable variability and limitations in economic evaluation methodologies, hindering direct comparisons of cost-effectiveness. Studies that assessed universal compared to selective screening strategies found that selective screening was more cost-effective; however, universal screening may be a viable option in high-prevalence contexts. One study that examined primary and secondary screening strategies reported that the cost per pregnancy for offering sickle cell trait screening was highest for primary care with parallel testing ($41) (i.e., test offered to mothers and fathers at the same time), followed by primary care with sequential testing ($37) (i.e., test offered to mothers, followed by father if mother identified as a carrier), and secondary care with sequential testing ($29). Conclusions: There is a significant gap in the economic evidence for interventions for SCD during pregnancy, particularly in low-resource settings. This gap underscores the urgent need for more high-quality economic evaluation studies to inform equitable, evidence-based global guidelines for SCD screening and management during pregnancy.