Cause

Sickle cell disease is an inherited disorder. More specifically, sickle cell disease is an autosomal recessive disease. This means that to have the disease, you must inherit a gene for the disease from both parents.

• Normally, a person inherits two genes that tell the body to produce normal hemoglobin A. One gene comes from each parent.
• People who inherit one defective hemoglobin S gene and one normal hemoglobin A gene have sickle cell trait. These people don't have symptoms of sickle cell disease nor do their bodies make sickled blood cells. But they can pass the defective hemoglobin S gene to their children.
• Sickle cell disease occurs when a person inherits one defective hemoglobin S gene from each parent.
• Similar sickle cell disorders occur when a person inherits a hemoglobin S gene from one parent and another type of defective hemoglobin gene from the other parent. Sickle cell disease is a sickle cell disorder.

Symptoms

Painful events (crises) in the hands or feet, abdomen, back, or chest are the most common symptom of sickle cell disease. This pain may last from hours to days. Most people with sickle cell disease experience anemia. Symptoms of anemia include feeling weak and tired. People with sickle cell disease can appear pale or washed out. Or they have a yellowish look to their skin and the whites of their eyes (jaundice).

When a child is born with sickle cell disease, it isn't possible to predict which symptoms will appear, when they will start, or how bad they will be. Most symptoms of sickle cell disease are related to either long-term (chronic) anemia or blood vessels blocked by sickled cells.

Symptoms related to chronic anemia

Most people who have sickle cell disease have at least mild symptoms of chronic anemia, which may include:

• Weakness.
• Tiredness (fatigue).
• Pale appearance.
• Yellowing of the skin and the whites of the eyes (jaundice).
• Shortness of breath, especially when they are active.

Severe anemia may raise the chance of a person with sickle cell disease getting high blood pressure in the lungs (pulmonary hypertension), and this can be deadly.

Symptoms caused by blocked blood vessels

Pain symptoms caused by blocked blood vessels in bones, organs, and other tissues include hours to days of extreme pain. These painful events can occur rarely to often. Sometimes home treatment can help the pain. And sometimes a hospital stay is needed.

Children ages 6 months to 4 years may have episodes of extreme pain in the hands, the feet, or both (hand-foot syndrome).