Sickle

Sickle Cell Disease

Sickle Cell Disease (pronounced: /ˈsɪkəl sɛl dɪˈziːz/) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia (SCA).

Etymology

The term "sickle" is derived from the Old English term "sicol" which means a short-handled farming tool with a semicircular blade. The disease is named so because the red blood cells assume a sickle shape due to the mutation.

Symptoms

Symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.

Causes

Sickle cell disease is caused by a mutation in the HBB gene. This gene provides instructions for making a protein called beta-globin, which is a component of hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen to the body's tissues.

Treatment

Treatment of sickle cell disease is usually aimed at avoiding crises, relieving symptoms, and preventing complications. Treatments might include medications, blood transfusions, and rarely a bone marrow transplant.

Related Terms

• Anemia
• Hemoglobin
• HBB gene
• Bone marrow transplant
• Blood transfusion

External links

• Medical encyclopedia article on Sickle
• Wikipedia's article - Sickle

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