Advancing sickle cell disease care in India from prevention to management: Challenges and way forward

Sickle cell disease (SCD) is a major Public Health concern in India with highest prevalence amongst the tribal ethnic groups. It is an autosomal recessive disorder which results in haemoglobin polymerisation leading to sickle shaped red blood cells. It can manifest as SCD (Hemoglobin SS [HbSS]), Sickle Cell Trait (HbSS) or Sickle β-Thalassemia (HbS). HbSS is the most severe form, presenting as vaso-occlusion crisis, chronic hemolysis, eventually resulting in progressive multiorgan damage. Given the diversity of clinical presentation, SCD poses a considerable burden on health care system. National Sickle Cell Anemia Elimination Mission (NSCEM) launched by Government of India in 2023 proposes comprehensive framework for SCD screening, diagnosis and management. This article provides an overview of recent updates on SCD, existing programmatic challenges and future directions for advancing sickle cell care in India. Although geospatial epidemiology has been well understood, the reach at the grassroots level has been impacted by lapses in implementation leading to difficulty in health care access in remote tribal regions. The review highlights the need of identifying context-specific gaps and developing an evidence-based optimal model for SCD screening, diagnosis, treatment, and care. Further, newborn screening clubbed with timely follow up ensuring continuum of care can be programmatically measured as a composite care continuum index for every diseased child tracking from diagnosis till treatment initiation. While innovations in point of care testing for SCD diagnosis and liquid oral formulation of hydroxyurea for treatment are promising, there is imperative need of efforts towards improving overall quality of life of SCD individuals, spanning beyond medical management. Integrating Social and Behavioural Change Communication and Advocacy, Communication and Social Mobilization may culminate in the socio-cultural transformation for long-term success. With a robust political commitment, microplanning strategies, intersectoral collaboration, and community participation, NSCEM, India can serve as a global model for sickle cell control in low resource settings.