what causes sickle cell anaemia

Sickle cell anemia is an inherited disease. People who have the disease inherit two copies of the sickle cell gene—one from each parent.

The sickle cell gene causes the body to make abnormal hemoglobin. Hemoglobin is the iron-rich protein that gives blood its red color and carries oxygen from the lungs to the rest of the body.

In sickle cell anemia, the hemoglobin sticks together when it delivers oxygen to the body’s tissues. These clumps of hemoglobin are like liquid fibers. They cause the red blood cells to become stiff and shaped like a sickle, or “C.” The sickled red blood cells tend to stick together and get caught in the blood vessels. (Other cells also may play a role in this process.)

Two copies of the sickle cell gene are needed for the body to make the abnormal hemoglobin found in sickle cell anemia.

Sickle Cell Trait

If you inherit only one copy of the sickle cell gene (from one parent), you will not have sickle cell anemia. Instead, you will have sickle cell trait.

People who have sickle cell trait usually have no symptoms and lead normal lives. However, they can pass the sickle cell gene to their children.

The following image shows how two parents who have sickle cell trait can pass the sickle cell gene to their children.

The image shows how sickle cell genes are inherited. A person inherits two copies of the hemoglobin gene—one from each parent. A normal gene will make normal hemoglobin (A). An abnormal (sickle cell) gene will make abnormal hemoglobin (S).

When each parent has a normal gene and an abnormal gene, each child has: a 25 percent chance of inheriting two normal genes; a 50 percent chance of inheriting one normal gene and one abnormal gene; and a 25 percent chance of inheriting two abnormal genes.

what is sickle cell anaemia

What Is Sickle Cell Anemia?

Sickle cell anemia (uh-NEE-me-uh) is a serious disease in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a "C."

Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through your blood vessels. Red blood cells contain the protein hemoglobin (HEE-muh-glow-bin). This iron-rich protein gives blood its red color and carries oxygen from the lungs to the rest of the body.

Sickle cells contain abnormal hemoglobin that causes the cells to have a sickle shape. Sickle-shaped cells don’t move easily through your blood vessels. They’re stiff and sticky and tend to form clumps and get stuck in the blood vessels. (Other cells also may play a role in this clumping process.)

The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage.

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