A new era dawns on sickle cell disease in India

Abstract

The severity of sickle cell disease (SCD) is frightening as 50-90 per cent of children do not live to celebrate their 5^th birthday in Africa and India, while millions continue to suffer life-threatening comorbidities^1-3. The first inception of molecular medicine is marked by the description of sickle haemoglobin in SCD by Linus Pauling in 1949⁴. In three quarters of a century since Pauling’s discovery, this scientific milestone is yet to be translated into clinical success; the estimated life expectancy of SCD patients remains only about 54 yr, almost two decades lower than non-SCD cohorts in the United States⁵. Although the characteristic sickling of red blood cells (RBCs) is well described, the mechanisms underlying disease-related morbidity and mortality are complex and remain poorly understood - thus precluding advances in disease-modifying and disease-mitigating therapies⁶