Sickle Cell Disease
Explanation
Sickle cell disease is a group of inherited disorders in which abnormal haemoglobin changes red blood cells, causing anaemia and episodes of blocked blood flow.
What happens in sickle cell disease?
Haemoglobin is the protein in red blood cells that carries oxygen. In sickle cell disease, inherited changes produce abnormal haemoglobin. Red blood cells can become rigid and unusually shaped.
How can the changed cells cause illness?
The cells can break down earlier than normal, leading to anaemia, which means too little haemoglobin to carry oxygen well. They can also block small blood vessels, causing painful episodes and injury to tissues or organs.
Is sickle cell trait the same as the disease?
No. A person with sickle cell trait carries one changed gene and generally does not have sickle cell disease, but can pass the gene to a child. Sickle cell disease involves a different inherited combination and can vary in type and severity.
How is it identified?
Blood tests can identify abnormal haemoglobin and help distinguish sickle cell disease from carrier status. Haemoglobin electrophoresis is one test used for this purpose, while the disease name describes the inherited condition itself.
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