September 9, 2014

What is sickle-cell disease?

Sickle-Cell Disease (SCD) is a serious disorder in which the body makes sickle-shaped red blood cells that are shaped like a crescent.

Normal red blood cells are disc-shaped and look like doughnuts without holes in the centre. They move easily through your blood vessels. Red blood cells contain an iron-rich protein called hemoglobin. This protein carries oxygen from the lungs to the rest of the body.{{more}}

Sickle cells contain abnormal haemoglobin called sickle haemoglobin or haemoglobin S. Sickle haemoglobin causes the cells to develop a sickle, or crescent shape. Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain and organ damage. It can also raise the risk for infection.

SCD is an inherited, lifelong disease – people who have the disease are born with it. They inherit two genes for sickle hemoglobin – one from each parent.

SCD varies from person to person. Some people who have the disease have chronic (long-term) pain or fatigue. However, with proper care and treatment, many people who have the disease can have improved quality of life and reasonable health much of the time.

It has no widely available cure. However, treatments to improve the anemia and lower complications can help with the symptoms and complications of the disease in both children and adults.

People who inherit a sickle haemoglobin gene from one parent and a normal gene from the other parent have a condition called sickle cell trait.

Sickle-cell trait is different than SCD. People who have sickle cell trait don’t have the disease. However, like people who have SCD, people who have sickle-cell trait can pass the sickle haemoglobin gene to their children.

[Extract from the National heart Lung and Blood Institute website]