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Sickle Cell Anemia

More than 70,000 Americans have sickle cell anemia. And about 2 million Americans ( including 1 in 12 African Americans) have the sickle cell trait, which means they carry a single gene for the disease and can pass this gene along to their children, but do not have the disease itself.

Sickle cell anemia occurs because an abnormal form of hemoglobin (HbS) is produced. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape.

RBCs containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy.


People with sickle cell anemia can also experience complications from impaired blood circulation and infection-fighting problems. These include a higher risk of certain infections and stroke as well as a condition called acute chest syndrome, which is caused by inflammation, infection, or occlusions (blockages) of blood vessels in the lungs by sickled cells.

Because people with sickle cell trait don't have the disease, they may never discover that they carry the gene. That's why it's recommended that teens who are unsure of their sickle cell status ask their doctors about testing.

The National Institutes of Health (NIH) recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in almost every state. This helps infants with sickle cell anemia get the care and treatment they need right away.

People with sickle cell anemia also may have bouts of severe pain in the chest, stomach, arms, legs, or other parts of the body. This is caused by sickle cells blocking blood flow through the small blood vessels in those areas. Feeling tired and having trouble fighting infections are also common among teens with sickle cell anemia,

Many teens with sickle cell anemia occasionally need to get transfusions of healthy red blood cells to help carry oxygen to the tissues of their bodies more effectively and treat complications. A few may need transfusions regularly.

Scientists are constantly researching ways to help people with sickle cell anemia. Several new treatments, such as the drug hydroxyurea, have helped reduce painful crises and episodes of acute chest syndrome for adults and kids with sickle cell. Bone marrow transplant, a complex and risky procedure, is the only cure for sickle cell anemia.

Source: kidshealth.org