Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts Healthy Cell, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells Sickle Cell become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia.
What makes the red cell sickle? There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape Sickle Cell instead of the round shape Healthy Cell.
How do you get sickle cell disease or trait? You inherit the altered hemoglobin from your parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You can not catch it. You are born with the sickle cell hemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease.
Is Sickle Cell only in African Americans? Sickle cell is in many nationalities including African Americans, Arabs, Greeks, Italians, Latin Americans, and those from India. You can be Caucasian and have sickle cell disease or trait. All races should be screened for this hemoglobin at birth.
How can I be tested? A simple blood test called the hemoglobin electrophoresis can be done by your doctor or local sickle cell foundation. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.
Newborn Screening Most States now perform the sickle cell test babies are born. The simple blood test will detect sickle cell disease or sickle cell trait . Other types of traits that may be discovered include:
- Hemoglobin C trait
- Hemoglobin E trait
- Hemoglobin Barts - which indicates an alpha thalassemia trait
- Beta thalassemia trait
Are there different types of sickle cell disease? There are three common types of sickle cell disease in the United States.
- Hemoglobin SS or sickle cell anemia
- Hemoglobin SC disease
- Hemoglobin sickle beta-thalassemia Each of these can cause sickle pain episodes and complications, but some are more common than others.
All of these may also have an increase in fetal hemoglobin which can protect the red cell from sickling and help prevent complications. The medication hydroxyurea also increases fetal hemoglobin.
