Sickle cell disease (SCD) is an inherited disorder that affects hemoglobin. Hemoglobin is a protein in the red blood cell that is responsible for carrying oxygen from the lungs to all parts of the body. Babies with sickle cell disease make a different kind of hemoglobin called hemoglobin S which is not as efficient at transporting oxygen. Some people with sickle cell disease can have hemoglobin S in combination with other hemoglobin variants called hemoglobin C, hemoglobin E and beta-thalassemia. These combinations can also lead to problems similar to those seen in sickle cell disease.
In SCD, the hemoglobin S causes the red blood cells to change their shape to look like a sickle or crescent moon shape. These cells are hard, sticky, and have trouble moving through small blood vessels. Babies with SCD usually have no symptoms at birth but if untreated are at risk of certain infections, pain crises and other health problems. Screening means that babies with sickle cell disease can receive early treatment, including immunizations and antibiotics, which will help prevent serious illness and allow the child to live a healthier life.
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