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Pediatric Sickle Cell Disease (SCD)

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Summary

Children inherit sickle cell disease (SCD) from their parents. SCD is a particular type of blood disease that causes anemia. Children with SCD have abnormal red blood cells, which are the cells that carry oxygen in the blood.

Expanded Overview

Sickle cell disease gets its name from the shape of the abnormal red bloods cells that people with SCD have. Instead of the normal, smooth "donut" shape, SCD red blood cells are crescent, or sickle, shaped.

In the United States, African-Americans are most likely to carry the genes that cause sickle cell disease. It is one of the most common types of inherited blood diseases in the country.

In sickle cell disease, hemoglobin (part of the red blood cells) becomes sticky and stiff. The sticky, stiff hemoglobin eventually deforms the red blood cells. When many of the red blood cells stiffen and deform, the spleen destroys them. The spleen is an organ that monitors the blood for problems, so it sees the deformed blood cells as defective and targets them for elimination.

When the spleen gets overwhelmed with too many tasks, it shuts down. This is called asplenia. When the spleen shuts down, children can develop many types of serious infections.

Sometimes the red blood cells break down and die while they are in the blood vessels or inside the bones. This can cause a lot of pain and it also can cause the bones to become deformed or bent, so children who have SCD usually have growth problems.

Because the blood gets "stickier," children with SCD are also more likely to have serious problems later in life, such as strokes and high blood pressure or tension within the lungs.

Test and Diagnosis

Doctors can tell if a baby will have sickle cell disease before the child is born. All children are tested for SCD at birth, even if they appear healthy. The following test help doctors determine what type of treatment is needed.

Blood transfusions help children who develop severe anemia. SCD is a serious disease in children but the recent use of preventive antibiotics and vaccines have greatly improved children's outcomes. For all SCD patients, continued medical management and care is very important to make sure they have a healthy life.

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