Early Childhood Thalassemia

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Thalassemia is the most common inherited disorder in the world that represents a major public concern. It is characterized by a defect in the genes responsible for production of hemoglobin. Hemoglobin is a protein that consists of alpha and beta chains. If the genetic mutations prevent any formation of beta chains then beta-thalassemia occurs which involves abnormal development of red blood cells and eventually anemia (1, 2). Children born with thalassemia major are normal at birth, but develop severe anemia during the first year of life. Other symptoms can include: Bone deformities in the face, fatigue, growth failure, shortness of breath and yellow skin (jaundice).Severe thalassemia can cause early death (between ages 20 & 30 years)…show more content…
The purpose of transfusion is twofold: to improve the anemia and to suppress the ineffective erythropoiesis. Chronic transfusions prevent most of the serious growth, skeletal and neurological complications of thalassemia major (6). In spite of its vital role in saving lives and enhancing patients' lives, blood transfusion is associated with risks. Making mistakes in blood transfusion and insufficient control of patients who receive blood during the transfusion can lead to death in such patients (7). So, standards of safe blood transfusion must be developed and maintained to ensure a safe and rational approach in the use of blood transfusions in the management of these disorders, also careful consideration must be given to the associated dangers(6). Nurses being responsible for the final bedside check before transfusion, have the final opportunity to prevent a mistransfusion. An understanding and knowledge of the pathophysiology of transfusion reactions, symptoms and treatment is essential to safely administer and monitor transfusions…show more content…
These are extremely challenging diseases that are fatal if left untreated. However, by sharing expert knowledge on their prevention and treatment, these conditions can be effectively managed (13). Nurse's education consists of the theoretical and practical training provided to nurses with the purpose to prepare them for their duties as nursing care professionals. It is very important for nursing staff to engage in plenty of ongoing training as well the needs of patients continue to change and there are new developments in procedure, the education of nurses never stops as they are required to continually master new skills and concepts throughout their career (14).
1.1 Aim of the

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