ABSTRACT

Sickle cell disease is a haemoglobin disorder in which the sickle beta globin gene (β s) is inherited. This is most commonly manifested as homozygous sickle cell anaemia (haemoglobin SS), sickle cell trait or β thalassaemia. Sickle cell disease is commonly found in patients with an ethnic background from Africa, West Indies, India, the Mediterranean and Middle East. The frequency of sickle cell carriers is 1 in 4 in West Africans and 1 in 10 in Afro-Caribbeans. It is estimated that the number of patients with sickle cell disease in the UK is around 10000.