The SCCSL was created in by the physicians at Tulane University in order to improve the care and quality of life of patients with sickle cell disease. The SCCSL is composed of many individuals including specialized physicians, nurses, psychiatrists, social workers, and administrators dedicated to helping patients of all ages with sickle cell disease. The SCCSL offers comprehensive medical care including exchange transfusion therapy, Hydroxyurea therapy, individualized pain programs, and psychosocial support. The SCCSL cares for the patient from birth into adulthood.
Sickle Cell Disease
General Information Sickle cell disease (SCD) is an inherited blood disorder that affects the red blood cells. It is inherited from both parents and can affect both males and females. Among African Americans in the United States, 1 in 10 persons are carriers of the sickle gene and 1 in 400 has sickle cell disease. Because the symptoms of SCD vary from person to person, no two children with SCD will necessarily have the same problems or the same intensity of a problem.
The science behind the disease: Red blood cells carry oxygen from the lungs to all parts of the body. Red blood cells travel in arteries and veins (blood vessels). Our bodies need oxygen for every function: eating, walking, even sleeping requires oxygen. Normal red blood cells are soft and round and can squeeze through blood vessels. Normal red blood cells live for about 120 days before new ones are made by the body to replace them.
People with sickle cell disease have red blood cells that are crescent shaped (sickle-shaped) and are stickier. These sickle red blood cells get stuck in the small blood vessels in the body. In addition, these sickle red blood cells do not live as long as normal red blood cells (only about 16 days). As a result, people with sickle cell disease are anemic meaning they have less blood in their bodies than people without SCD.
Hemoglobin is the main substance of the red blood cell. It is the protein that carries oxygen around in the red blood cells. Normal red blood cells contain hemoglobin A. People with sickle cell disease have abnormal hemoglobin called hemoglobin S and/or hemoglobin C. It is this abnormal hemoglobin S or C that causes the red blood cells to have the sickle shape and get stuck in the blood vessels. When sickle-shaped red blood cells get stuck in blood vessels, less blood reaches that part of the body. The part of the body that does not receive normal blood flow can have pain or be damaged. This decrease in blood flow is what causes the complications of sickle cell disease.
New research has a lot of options for people with sickle cell disease to help them live healthier, happier, lives. It is very important for people with sickle cell disease to be seen by a trained hematologist (blood doctor) in a comprehensive setting. People with sickle cell disease need to be followed very closely for complications in all of their organs. They should have heart exams, kidney exams, eye exams, and transcranial doppler studies (that measure blood flow in the brain) regularly to prevent complications from sickle cell disease.
Currently, Hydroxyurea is the only medication available to persons with sickle cell disease that can improve overall outcomes and prolong life. The only available cure for sickle cell disease is bone marrow transplant. However, not all persons with sickle cell disease are able to undergo bone marrow transplant. You should contact your physician to see if you are eligible for this procedure.
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