Painful swelling of the fingers and toes, called dactylitis, can occur in infants and children younger than age 3. The pain can occur anywhere, but most often occurs in the chest, arms, and legs. These are also called vaso-occlusive crises. This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. Healing and normal growth and development may be delayed because of chronic anemia. The decrease in red blood cells causes anemia. Severe anemia can make the person's ability to carry oxygen to the tissues more difficult, possibly causing them to be pale, dizzy, short of breath, and tired. This sickle shape makes the cells stiff and sticky causing them to become stuck in the vessels, destroyed by the spleen, or simply die because of their abnormal function. In sickle cell disease, red blood cells are produced but then become deformed into the sickle shape, which causes red blood cells to lose their ability to carry oxygen. This is the most common symptom of all the sickle cell anemia. Symptoms and complications may include, but are not limited to, the following: However, each individual may experience symptoms differently. It is recommended that people who carry a hemoglobin trait meet with a genetic counselor to obtain more information.The following is a list of symptoms and complications more commonly associated with sickle cell anemia. The couple also has a 25 percent chance of having a child with regular hemoglobin (AA) and a 50 percent chance of having a child with a hemoglobin trait like the parents. If both parents are carriers of sickle cell trait or another hemoglobin change (like beta thalassemia), there is a 25 percent (or one in four) chance that they will have a child with sickle cell disease. It is important to identify people who are carriers of abnormal hemoglobin so they will be aware of their risk of having children with sickle cell disease. Individuals with sickle cell beta thalassemia disease inherit a hemoglobin S gene from one parent and a beta thalassemia gene from the other parent. Like height and eye color, a person inherits genes that produce hemoglobin from each parent. Why is it Important to Know if You Carry Abnormal Hemoglobin? This results in periodic plugging of blood vessels, thereby preventing delivery of oxygen to tissues and organs. When affected by sickle cell disease, the red blood cells become rigid, sticky, and sickle shaped. Normal red blood cells are soft, smooth, round and can move easily through the body. Hemoglobin is found in all red blood cells and carries oxygen from the lungs to tissues and organs throughout the body. Sickle cell beta thalassemia disease is a lifelong condition and requires lifelong treatment. Individuals with sickle cell beta thalassemia may have medical problems that include anemia (low hemoglobin), crises (painful episodes), organ damage, infections, lung problems, leg ulcers, bone damage and strokes. However, if the beta thalassemia gene produces no normal hemoglobin, b0 thalassemia, the condition is identical to sickle cell disease. If the gene produces a small amount of normal hemoglobin, b+ thalassemia, individuals have milder symptoms. The severity of the disease varies because the beta thalassemia gene may still produce a small amount of normal hemoglobin. However, it is also common in people whose families are from Africa, South or Central America, the Caribbean Islands and the Middle East.Ī person with sickle cell beta thalassemia disease has red blood cells that sickle due to the hemoglobin S gene, and other red blood cells that are small, pale and misshapen due to the beta thalassemia gene. Hemoglobin S beta thalassemia disease is the most common sickle cell syndrome seen in people of Mediterranean descent. The most common types of sickle cell disease are SS, SC and S beta thalassemia. Sickle cell beta thalassemia (Hb S/ß Th) is an inherited form of sickle cell disease that affects red blood cells. Sickle Cell Beta Thalassemia Disease What is Sickle Cell Beta Thalassemia Disease? Newborn Screening Program - Sickle Cell Disease and Other
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