Sickle cell disease is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body.

In someone who has sickle cell disease, hemoglobin is abnormal, causing red blood cells to become hard and sticky and look like a C-shaped farm tool called a sickle.

The sickle cells die early, which causes a constant shortage of red blood cells, and when they travel through small blood vessels, sickle cells get stuck and clog the blood flow. This can cause pain and other serious complications (health problems) such as infection, acute chest syndrome, and stroke.

Cause

Sickle cell disease is a genetic condition that is present at birth. It is inherited when a child receives two genes (one from each parent) that code for abnormal hemoglobin.

Screening for sickle cell is done using a simple blood test either after birth or while the baby is in the womb. Because children with sickle cell disease are at an increased risk of infection and other health problems, early diagnosis and treatment are important.

Symptoms

People with sickle cell disease may start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications are different for each person and can range from mild to severe.

Treatment

Management of sickle cell disease is focused on preventing and treating pain episodes and complications. Prevention strategies include lifestyle behaviors such as maintaining adequate fluid intake and avoiding extreme temperatures and medical screenings. Your health care provider can determine what treatment and management strategies are best for you or your child.

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