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Sickle Cell Disorder

Definition

Sickle cell disorder is a genetic disease where red blood cells assume an abnormal, rigid, sickle shape. These misshapen cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

Analogy

Imagine if instead of soft, flexible marshmallows (normal red blood cells) flowing through a straw (blood vessel), you have rigid, pointy ice cubes (sickle-shaped cells). The ice cubes would get stuck and block the flow through the straw.

Related terms

Hemoglobin: This is the protein molecule in red blood cells that carries oxygen from the lungs to the body's tissues and returns carbon dioxide from the tissues back to the lungs.

Anemia: A condition marked by a deficiency of red blood cells or of hemoglobin in the blood, resulting in pallor and weariness. In sickle cell disorder, anemia occurs because sickle-shaped red blood cells die off faster than new ones can be produced.

Genetic Mutation: A permanent alteration in the DNA sequence that makes up a gene. Sickle cell disorder is caused by a mutation in one of the genes that instructs your body how to make hemoglobin.