Sickle cell disease

Abstract

The haemoglobinopathies are the most common monogenetic diseases in the world. They include the thalassaemias and sickle cell syndromes. The sickle cell syndromes encompass several abnormal haemoglobin variants, of which homozygosity for the sickle cell gene – that is, sickle cell anaemia is the most common and most severe. Originally characteristic of the tropics and subtropics, recent mobility and migratory trends have meant that the prevalence of sickle cell disease (SCD) has significantly increased in the UK. It is important that GPs have an understanding of this disease, in order to help their patients deal with complications of every-day life. This article will address three main aspects of SCD: diagnosis, health maintenance, and some acute and chronic complications of SCD.