Sickle cell disease (SCD) is a group of inherited single-gene autosomal recessive disorders caused by the ‘sickle’ gene, which affects haemoglobin structure. SCD has its origins in sub-Saharan Africa and the Middle East, hence it is most prevalent in individuals of African descent as well as in the Caribbean, Middle East, parts of India and the Mediterranean, and South and Central America. Owing to population migration, SCD is now of increasing importance worldwide and there are increasing numbers of affected individuals in Europe and the USA.
The term SCD includes sickle cell anaemia (HbSS) and the heterozygous conditions of haemoglobin S and other clinically abnormal haemoglobins. These include combination with haemoglobin C (giving HbSC), combination with beta thalassaemia (giving HbSB thalassaemia) and combination with haemoglobin D, E or O-Arab. All of these genotypes will give a similar clinical phenotype of varying severity. Haemoglobin S combined with normal haemoglobin (A), known as sickle trait (AS), is asymptomatic, except for a possible increased risk of urinary tract infections and microscopic haematuria, and is not considered further in this guideline.
The purpose of this guideline is to describe the management of pregnant women with sickle cell disease (SCD). It will include preconceptual screening and antenatal, intrapartum and postnatal management. It will not cover the management of women with sickle cell trait.