Initial outcomes of a comprehensive care model for sickle cell disease among a tribal population in rural western India

Abstract

Background: Sickle cell disease (SCD) is the most prevalent inherited blood disorder worldwide, and is increasingly recognized as a neglected chronic disease. Several cost-effective interventions have dramatically reduced morbidity and mortality from SCD in developed countries. However, access to care for SCD in India is limited in most rural tribal regions with the highest SCD prevalence . Methods: In 2014, we implemented a comprehensive care model in rural Gujarat, India, with the aim of providing high-quality screening and treatment to increase coverage of proven interventions for sickle cell disease. Components of the intervention include: new-born, antenatal and family screening; outpatient sickle cell clinic; inpatient protocols; chronic disease registry; and health education. We followed a cohort of patients enrolled between February 2014 and October 2014 and compared the severity of disease and coverage of proven interventions at baseline and after one year of follow-up. Results: Of 164 patients diagnosed with sickle cell disease who enrolled in the comprehensive care program, 72 patients (43.9%) had SCD pain crises, 59 (35.9%) were hospitalized, 43 (26.2%) received blood transfusions, and 3 (1.8%) died during follow-up. Compared to baseline, coverage of pneumococcal vaccine (0.6% to 78.5%, p<0.001) and hydroxyurea (4.5% to 50.0%, p=0.002) significantly improved. There was a reduction in the proportion of patients with ≥3 pain crises per year (35.4% to 9.8%, p<0.001) and the proportion of patients with ≥1 hospitalization per year (56.7 go 36.0%, p<0.001) with no significant change in patients with ≥1 blood transfusion in last year (32.9% to 26.2%, p=0.127) . Conclusions: This study demonstrates that implementing a continuity care model for sickle cell disease in rural India is feasible and can improve coverage of evidence-based interventions.