Haemoglobinopathies in Pregnancy

Haemoglobinopathies is a collective term used for inherited disorders of haemoglobin synthesis. Individuals with sickle cell disease can be homozygote SS (HbSS), heterozygotes SC (HbSC) and heterozygotes S-beta thalassaemia (HbS beta thalassaemia). The homozygous state (HbSS) is referred to as sickle cell anaemia (SCA). Worldwide, SCD is one of the most common inherited single gene autosomal recessive genetic disorders. Approximately, 150,000 Nigerian babies with HbSS are born yearly, one of the highest in the world. Pregnancy in SCD is a high-risk pregnancy especially in homozygous HbSS which may be associated with adverse maternal and foetal outcome. This chapter describes the pathophysiology of SCD. Additionally, it describes the complications and management of SCD in pregnancy as well as preventive measures. Management of sickle cell disease in pregnancy in low and middle resource countries like Nigeria is saddled with a lot of challenges, as a result of which it is associated with high morbidity and mortality for both the mother and the foetus. Preconception care, quality antenatal, intrapartum, postpartum and family planning services, using a multidisciplinary approach, are all essential in ensuring improved outcomes for both mother and baby.