Morbidity pattern of sickle cell disease in India: A single centre perspective

inherited blood condition worldwide resulting from single DNA mutation within the beta globin gene1. Due to recent population movements, there is gene flow from high allele frequency areas of sub-Saharan Africa, the Middle East, and India to Europe and parts of America2. This has led to sizeable sickle cell populations emerging in previously unaffected areas of the world. Each year approximately 300,000 children are born with sickle cell anaemia or one of its variants and nearly 80 per cent of these births occur in poor socio-economic countries3,4. The incidence of most clinical complications varies markedly both with time in the same individual and between different individuals5. The same variability and unpredictability are seen with most other serious complications, including cerebrovascular disease6, acute chest syndrome7, renal failure8 and premature death9 . Despite the complexity and multifactorial pathophysiology of vaso-occlusion, relatively straightforward interventions have been found to greatly improve outcomes for children with SCD. These include, (i) early identification by neonatal screening programme; (ii) education of parents and patients of medical complications and early recognition; (iii) preventive measures with prophylactic penicillin and pneumococcal immunizations; (iv) screening programmes for early signs of organ damage especially transcranial Doppler (TCD) examination; and (v) therapeutic intervention with transfusions, hydroxyurea, or stem cell transplantation. Although outcomes have improved significantly over the last 40 years in many countries, clinical management remains primitive particularly so in countries where the disease is most common, including Africa and India.