ABSTRACT
Sickle cell disease (SCD) is an inherited haemoglobin disorder that affects how the oxygen is transported within the blood. Individuals with sickle cell trait are generally asymptomatic and may be diagnosed accidently, for example from blood investigations prior to surgery or antenatal care. SCD is characterised by a change in the shape of red blood cells. In SCD when red blood cells are oxygenated, they retain the normal cell shape. However, when they become deoxygenated they polymerise causing them to change shape and become long fibres and ‘stick’ together, causing them to become sickle in shape. During a severe sickle cell crisis and acute deterioration patients may require admission to critical care units. Vaso-occlusive crises (VOC) occur when the sickle-shaped cells occlude blood vessels, preventing oxygen and nutrients reaching tissues; this leads to tissue hypoxia, tissue necrosis and severe pain. The underlying cause of VOC is often infection, therefore the cause should be identified and appropriately treated.
