Sickle cell Anemia

-431800-311150Flashcard Template: Sickle Cell AnemiaDisease Process:Sickle cell anemia is a hereditary blood disease resulting from an abnormal hemoglobin type known as HbS or sickle hemoglobin. This condition arises from a mutant gene on chromosome 11, called HBB, which encodes the beta-globin subunit of hemoglobin. At low oxygen tension, HbS precipitates and forms polymers that alter the shape of the RBCs to become sickle-like. Sickle-shaped cells may block blood vessels, resulting in pain, organ complications, and elevated threat of infection (Rees et al., 2010).Pathophysiology:In a healthy state, red blood cells are biconcave and possess a discoidal structure, which is well suited for blood voyage within the microcirculatory system. However, in sickle cell anemia, these sickle-shaped cells are primarily weaker with a shorter life span, about 10 to 20 days, unlike the normal RBCs, whose lifespan is 120 days. This results in hemolytic anemia, in which the body fails to manufacture enough RBCs to replace destroyed ones (“National Heart, Lung, and Blood Institute [NHLBI],” 2019). All of which leads to increased bilirubin levels, jaundice, and chances of impaired gall bladder function and gall stones.The sickle-shaped cells stick to the blood vessels’ inner linings causing vaso-occlusive crises. These crises lead to severe pain and may cause ischemia of various organs, including the spleen, liver, and lungs (Ballas & Lusardi, 2005).Risk Factors and Why It is a Risk:Genetic inheritance: Sickle cell anemia can be inherited as an autosomal recessive trait. People who carry one sickle cell gene experience no ill effects but can transmit the mutated gene to their offspring. If both the parents are sickle cell gene carriers, the chances of the children developing sickle cell anemia are ˜ 25%. This genetic predisposition identifies the risk for those – who have the covet ethnic background – of African, Mediterranean, Middle Eastern, and Indian origin (Rees et al., 2010).Environmental conditions: Elements such as altitude, barometric pressures, low oxygen levels, and low humidity worsen the disease manifestations. These factors cause vasoconstriction, increased RBC rigidity, and reduced oxygen delivery, which leads to an increased amount of oxygen changing to form more sickle RBCs, causing vaso-occlusive crises (Ballas & Lusardi, 2005).Infections: Patients with sickle cell anemia have greatly reduced spleen function and are, therefore, more prone to infections. The spleen removes bacteria from the blood, but repeated episodes of vaso-occlusion can lead to splenic sequestration and functional asplenia, which puts the patients at risk of developing pneumococcal pneumonia (NHLBI, 2019).Major Signs and Symptoms and How They OccurChronic pain crises: Sickle cell anemia is characterized by pain episodes or vaso-occlusive crises, which are the prototypic symptoms of the disease. These episodes arise when sickle-shaped cells obstruct blood circulation, leading to occlusion and acute pain (Rees et al., 2010).A...