Wednesday, 15 June 2016

World Sickle Cell Day

Roger Watson, Editor-in-Chief

According to the US National Heart, Lung and Blood Institute: 'The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal haemoglobin, called haemoglobin S or sickle haemoglobin, in their red blood cells.' Sickle cell disorder leads to crises whereby the small blood vessels in the body become occluded leading to extreme pain in several areas of the body. Such crises require hospitalisation. While understanding of the condition and treatment has improved, sickle cell disorder is life-limiting and in some parts of the world is an economic and family burden.

The condition is unequally distributed geographically with populations of sub-Saharan and African origins being affected more commonly. In some communities, due to the recessive nature of the gene for sickle cell disorder, consanguineous (mainly cousin) marriage is responsible for the prevalence of the disorder.

World Sickle Cell Day is on 19 June and JAN is marking this with a Special Section on sickle cell disorders. These six articles were published in the June issue and covered in an editorial by Diana Wilkie, University of Florida USA.

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