Sickle Cell Anemia
...-occlusive crisis - Painful episode, characterized by distal ischemia and pain. Sequestration Crisis – Pooling of blood in liver and spleen with decreased blood volume and shock. Aplastic Crisis – Diminished RBC production resulting in anemia. Hyperhemolytic Crisis – Accelerated rate of RBC destruction characterized by anemia, jaundice and reticulocytosis. Chest Syndrome – Chest pain, fever, pneumonia-like cough and anemia. CVA – Sudden with no related illness-Blockage of major blood vessels to brain. DIAGNOSTIC EVALUATION Newborn screening for SCA is mandatory in most of the US. If SCA is not diagnosed in early infancy it is likely to manifest symptoms during the toddles and preschool years. SCA is occasionally first diagnosed during a crisis that follows and acute respiratory or G.I. infection. Routine hematologic test are done to evaluate the anemia. The sickle-turbidity test screens for SCA in a finger stick. Hgb electrophoresis is an accurate, rapid and specific test for detecting the homo- and heterozygous form of disease. THERAPEUTIC MANAGEMENT Therapy aims at preventing conditions that enhance sickling phenomena and to treat medical emergencies of sickle cell crisis Medical management is usually directed at supportive and symptomatic treatment. The main objectives are to provide (1) bed rest to minimize energy expenditure and oxygen use; (2) hydration through oral and IV therapy; (3) electrolyte replacement, since hypoxia results in metabolic acidosis, which promotes sickling; (4) analgesics for severe pain from vaso-occlusion; (5) blood replacement to treat anemia and to reduce the viscosity of the sickled blood; and (6) antibiotics to treat existing infection. The vaccines for pneumococcal and meningococcal are recommended. Hepatitis B vaccine is recommended for those receiving transfusion. Oral penicillin prophylaxis is recommended by 2 months of age. Exchange transfusion reduces the number of circulating sickle cells and slows down the vicious circle of hypoxia, thrombosis, tissue ischemia and injury. NURSING CONSIDERATIONS Educate family and chil...