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  Sickle cell anemia

Sickle cell anemia is a subtype of sickle-cell disease. This is a genetic, non-infectious disorder that occurs when both parents are carriers of the genetic marker. Patients are mainly of African American descent, but the disease is also present in other ethnic groups - such as Mediterranean and Middle Eastern. Anyone can get sickle cell anemia.

Sickle cell anemia Blood disorder
Sickle cell anemia


In sickle cell anemia the red blood cells change shape and fluidity. The red blood cells become rigid and form the shape of a farm tool called sickle. The sickle shape is similar to a crescent. One in twelve African Americans (roughly two million Americans) are carriers for sickle cell anemia, having one gene for the disease and yet, do not have the disease.

Typical hemoglobin, a protein in red blood cells, helps these blood cells carry oxygen through the blood stream to all parts of the body. Sickle cells are produced when an abnormal form of hemoglobin is created referred to as HbS. HbA or normal hemoglobin in red blood cells provides normal functioning within the body. HbS tends to make red blood cells group together in a sticky clump. Red blood cells with the HbS protein will repeatedly change shape between the normal shape and the sickle shape. Eventually a red blood cell with HbS will stay in the sickle shape.

Symptoms:

Normal red blood cells last in the body for about four months. Sickle cells are extremely fragile. These cells break down or die out when they are between ten to twenty days. This will cause the patient to suffer from severe anemia. This type of anemia is caused by a dramatically decreased level of red blood cells in the blood stream. The resulting symptoms are exhaustion and weakness.

Because of their abnormal shape, sickle cells can block arteries and veins, depriving the body of nutrients and oxygen, which are needed for good health.

In sickle cell anemia - white blood cells and platelet levels can be higher than normal. This can contribute to vasso-occlusion and a vaso-occlusion crisis. Vaso-occlusion occurs when capilaqrries are blocked by sickle cells. The restricted blood flow will cause tissue damage, etreme pain and organ damage. The spleen, an organ in the abdomen that produces blood cells, is a affected adversely by sickle-cell anemia. It usually is removed before adult hood in sickle-cell anemia patients. The removal of the spleen increases the risk of infection and prophylactic antibodies are often prescribed.

Bones of the leg are particularly affected by vaso-occlusive damage. The femur bone may start to die (avascular necrosis) or bone deterioration may occur. The pain commonly experienced by sickle-cell patients is also from bone ischemia. Bone ischemia is the lack of blood supply to the bones.

Acute chest syndrome is a condition whose symptoms are fever, chest pain and difficulty breathing. It is caused by infection and when sickle cells become lodged within the lungs.

Complications can develop from sickle-cell anemia, such as post splenectomy infection, stroke, jaundice, gallstones due to increase bilirubin production, cerebral infarction in children, cerebral bleeding in adults, aseptic bone necrosis (death), priapism, bacterial bone infection and decreased immune reactions.

Diagnosis:

Diagnosis for sickle-cell anemia is made with a blood test called a hemoglobin electrophoresis. This test will determine if HbS is present in the patient’s red blood cells.

Treatment:

Unfortunately, there is no cure for sickle-cell anemia. Treatment can be used to prevent complications from developing. Teens often take regimens of folic acid to help red blood cell production and penicillin to help fight and prevent infection. Pain medication is often prescribed to control pain.

A sickle-cell anemia crisis can be treated and managed at home with rest, increased fluid and pain medication. If a sickle-cell anemia crisis is extreme, a hospital admission is required - where the patient can receive IV fluids, oxygen therapy and strong pain medication.

Teenagers who have sickle-cell anemia will often receive blood transfusions to help blood to deliver oxygen to the entire body.

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