Epidemiology of Thalassemia and Physiologic Pattern in Sickle-cell Anemia Treatment

WEEK4 DQ 2
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WEEK4 DQ 2
Discuss the epidemiology of thalassemia and share evidence-based practice guidelines necessary for chronic management.
Thalassemia is a heterogeneous group of hypochromic anemias characterized by pale red blood cells due to low hemoglobin levels. Thalassemia is caused by decreased or absent production of at least one globin polypeptide chain (beta, alpha, gamma, delta) and leads to abnormal red blood cells prone to hemolysis and anemia. The most common types of thalassemia are α-thalassemia (alpha), β-thalassemia (beta), and βδ-thalassemia (beta-delta). Underlying genetic defects consist of deletions, and deletion is the total or partial loss of the total or partial genetic material) of the globin alpha chains, which is a protein that is part of hemoglobin (Hb). The disorder is most common in areas with high malaria rates.
Thalassemia is a hereditary disease, and treatment focuses on regular blood transfusions, folic acid supplements, chelation therapy to remove excess iron, splenectomy if splenomegaly is present, and stem cell transplant in children. Thalassemia medications to get rid of extra iron include deferasirox, deferiprone, and deferoxamine, and the medications are also used in sickle cell dis...