Sickle Cell Disease

About

Sickle cell disease is a disorder passed down through families. The red blood cells that are normally shaped like a disk take on a sickle or crescent shape. Red blood cells carry oxygen throughout the body.

Causes

Sickle cell disease is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.

• Hemoglobin S changes the red blood cells. The red blood cells become fragile and shaped like crescents or sickles.
• The abnormal cells deliver less oxygen to the body's tissues.
• They can also easily get stuck in small blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.

Sickle cell disease is inherited from both parents. If you get the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell disease.

Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.

Outlook (Prognosis)

In the past, people with sickle cell disease often died between ages 20 and 40. Thanks to a modern care, people now can live to the age of 50 and beyond.

Causes of death include organ failure and infection.

When to Contact a Medical Professional

Call your health care provider if you have:

• Any symptoms of infection (fever, body aches, headache, fatigue)
• Pain crises
• Painful and long-term erection (in men)

Alternative Names

• Anemia - sickle cell
• Hemoglobin SS disease (Hb SS)
• Sickle cell anemia