Children with sickle cell disease (SCD) are particularly prone to severe infections to germs such as peumococcus and salmonella. Pathophysiological basis of this increased sensitivity is not fully understood, although functional hyposplenism is one of the mechanism and probably also abnormalities of the complement system. Early loss of spleen function results from repeated micro-vasoocclusive events in the peculiar spleen vasculature.

In countries where SCD is not detected at birth and adequate antibiotic prophylaxis not provided together with a reinforced immunization regimen, under-five mortality by infection can reach 95% of the children living in remote areas. In countries where such programs are in effect, survival rate at the age of 20 reaches 95%.

Thank you for the clarification.