Sickle cell disease in India: a scoping review from a health systems perspective to identify an agenda for research and action

Abstract

IntroductionSickle cell disease (SCD) disproportionately impacts Adivasi (tribal) communities in India. Current research has focused on epidemiological and biomedical aspects but there has been scarce research on social determinants and health systems aspects. Given its fragmented distribution, resources and programmes have emerged in west and central India. This scoping review seeks to identify geographical and evidence gaps for action on SCD from a health systems lens.MethodsWe followed a scoping review protocol, using Google Scholar and PubMed for published literature. Keywords used included sickle cell anaemia/disease, health systems, tribal and India. We used Google search for grey literature. We compiled a list of 55 records (of which 35 were retained), with about half pertaining directly to India and others relevant to similar settings. Results were organised and analysed using the WHO health systems framework to identify geographical and evidence gaps.ResultsWe found substantial literature on biomedical and clinical aspects of SCD but little on the design and implementation of programmes in community and Adivasi-specific contexts as well as on social determinants of SCD. There were regional gaps in knowledge in southern and northeast India. Wherever community-based programmes exist, they have originated in civil society initiatives and relatively limited state-led primary healthcare-based efforts pointing to weak agenda setting.ConclusionBoth research and action on SCD especially among tribal populations need immediate attention. While geospatial epidemiology has been well understood, gaps remain in context-specific knowledge for action in several parts, as well as evidence gaps across several health system building blocks, including governance and financing of care. Despite publication of a draft policy, delayed adoption and lapses in implementation have limited the response largely to local communities and non-governmental organisations.