Although sickle cell disease is not bound to a specific geographical region, it occurs mainly in Africa and countries with large populations of people with African ancestry, especially from areas that have traditionally been malaria-affected regions. Half of the children in the Netherlands who have SCD are of African origin and a third originate from Suriname, Curaçao, or the Netherlands Antilles. From January 2007, SCD has been included in the neonatal screening program (the heel prick test). The total number of SCD patients in the Netherlands is estimated at 1,000-1,500, of which two thirds are children. The number of new cases per year lies between 40 and 60. An estimated 10% to 15% of people (about one in seven) with African, Surinamese, Curaçaoan, or Netherlands Antillean ancestry carry the disease.

Sickle cell disease and malaria

Occurrence of sickle cell disease in a region is related to the presence of malaria in that same region. This is because malaria parasites cannot thrive or multiply in the red blood cells of sickle cell disease carriers to the same extent as they can in normal red blood cells. As a result, sickle cell carriers have, throughout evolution, turned out to survive better in malaria-endemic areas. Still, if you actually have sickle cell disease, this does not mean you have built-in full malaria protection. In fact, if you do happen to contract malaria, the course may be more severe. Good prophylaxis against malaria is therefore always important, also when you have SCD.