Prophylaxis crucial in sickle cell disease

NMUH Staff Publications

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<span style="font-size: 10pt;">Sickle cell disease comprises a heterogeneous family of disorders characterised by the tendency of erythrocytes to 'sickle' when deoxygenated. It is an autosomal recessive condition that occurs when sickle haemoglobin (HbS), encoded by the gene Betas, combines with another abnormal haemoglobin gene. The most common form is homozygous sickle cell disease (SS), commonly known as sickle cell anaemia. The Betas gene can also interact with a thalassaemia genes and other structurally abnormal haemoglobins, such as HbC, found in parts of Ghana, and HbDPunjab, found in parts of India, to produce other forms of sickle cell disease - SC and SD respectively. The sickle gene is widely distributed throughout Central and Southern Africa, the Eastern Mediterranean and the Indian subcontinent.</span>