Sickle cell disease is an inherited disorder that affects hemoglobin. Hemoglobin gives blood its red colour and is responsible for carrying oxygen from the lungs to all parts of the body. The types of sickle cell disease most commonly seen in BC are sickle cell anemia, sickle hemoglobin C disease and sickle beta thalassemia disease. Sometimes sickle cell hemoglobin can be present in combination with other changes in hemoglobin (called hemoglobin C, hemoglobin E and beta-thalassemia). These combinations can also lead to problems similar to those seen in sickle cell disease.
Babies with sickle cell disease make a different kind of hemoglobin. This hemoglobin causes the red blood cells to change their shape (they look like a banana or sickle) and become hard and sticky. The hard, sticky sickle red blood cells have trouble moving through small blood vessels and may block the blood supply and cause pain in that part of the body. They also cause a low blood count that makes the person tired and short of breath. 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. One or two babies are found to have sickle cell disease each year in BC.
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