COOLEY ANEMIA

Course overview

THALASSEMIA MAJOR OR COOLEY ANEMIA IS ONE OF THE COMMON MONOGENIC HEREDITARY HEMOGLOBIN DISORDERS. IT RESULTS FROM THE ABSENCE OF A BETA-GLOBIN CHAIN IN THE PATHWAY OF HEMOGLOBIN PRODUCTION. IT IS NOW A GLOBAL PUBLIC HEALTH CONCERN DUE TO CHANGING DEMOGRAPHICS. THE PRIMARY UNDERLYING PATHOPHYSIOLOGY IS INEFFECTIVE ERYTHROPOIESIS. AS A RESULT, PATIENTS WITH THALASSEMIA BECOME LIFE-LONG BLOOD TRANSFUSION DEPENDENT. TO AVOID THE HIGH MORBIDITY AND MORTALITY ASSOCIATED WITH THIS CONDITION, IT MUST BE PROMPTLY DIAGNOSED AND TREATED. THIS ACTIVITY REVIEWS THE PROPER EVALUATION AND TREATMENT OPTIONS OF THALASSEMIA MAJOR AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM AND PATIENTS IN THE MANAGEMENT OF ITS COMPLICATIONS.