In patients with SCA, a genetic defect causes normal hemoglobin A to be partially or completely replaced by hemoglobin S, depending on the severity of the disease.
Unlike normal RBCs that are shaped like biconcave discs, RBCs are sickle or crescent-shaped in patients with SCA. Due to their abnormal shape, sickle-shaped RBCs tend to stick together, blocking blood flow within blood vessels throughout the body.
Jaundice is common in patients with SCA due to the rapid breakdown (hemolysis) of abnormal red blood cells. Rapid hemolysis can lead to a build-up of bilirubin, causing noticeable discoloration of the skin and sclera.
Obstruction of blood vessels by sickled RBCs can block blood flow causing ischemia and pain. In patients who experience chronic vaso occlusive episodes, leg ulcers may develop. Symptoms of a vaso-occlusive crisis can include severe pain in the hands, feet, joints, and abdomen, stroke, changes in vision, yellowing of the skin and eyes, blood in the urine, and priapism (persistent painful erection).
Patients may present with a fever during a vaso-occlusive crisis.
Sickled RBCs may prevent blood from leaving the penis during a vaso-occlusive crisis, causing priapism or a prolonged, painful erection.
Patients typically experience mild to excruciating pain during a vaso-occlusive crisis, due to tissue hypoxia. Pain commonly occurs in areas such as the abdomen, hands, and feet.
Sickle cell anemia is common among African Americans. Although it can occur in other ethnicities, the incidence among these populations is lower.
If SCA is not diagnosed during infancy, patients with this disease may remain asymptomatic until they experience a crisis due to lack of adequate oxygenation, or dehydration.
Individuals with SCA are at an increased risk for infection due to splenic damage. Autoinfarction of the spleen typically occurs after the age of ten and renders the spleen unable to destroy bacteria and other foreign substances in the body.
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