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THALASSEMIA (SIGNS & SYMPTOMS : Depending…

- - - - PURPOSE
        
        To improve anemia
        
        To suppress ineffective erythropoeisis
        
        To reduce splenomegaly and hypersplenism
        
        To decrease absorption of dietary iron
      - WHEN TO START?
        
        Based on inability to compensate for the low haemoglobin
        
        Signs of increased cardiac effort
        
        Tachycardia
        
        Sweating
        
        Poor feeding
        
        Poor growth
        
        Increasing symptoms of ineffective erythropoiesis
        
        Bone changes
        
        Massive splenomegaly
      - FACTORS TO CONSIDER FOR CHRONIC TRANSFUSION
        
        Anemia linked with significant impairment in quality of life or associated morbidities
        
        Poor growth
        
        Inability to maintain daily routine and activities
        
        Evidence of organ dysfunction
        
        Evidence of cardiac disease
        
        Pulmonary hypertension
        
        Dysmorphic bone changes
        
        Hemoglobin drops under 7 g/dL on two occasions, two weeks apart
      - ADMINISTRATION
        
        The amount of blood received on transfusion day is determined by pre-transfusion hemoglobin levels. The target is to maintain the pre-transfusion hemoglobin level between 9 and 10 g/dL. Attempts to maintain pre-transfusion hemoglobin at above 10 g/dL increase transfusion requirements and the rate of iron loading.
        
        Transfusions should generally be given at an interval of three to four weeks. (With aging patients, a transfusion every two weeks may be necessary.)
        
        Product of choice is packed red blood cells depleted of leucocytes and matched with the patient’s red antigen phenotype for at least D, C, c, E, e, and Kell
        
        Blood should be transfused at 5 mL/kg per hour, and the post-transfusion hemoglobin should not exceed 14 g/dL.
        In patients with severe anemia (hemoglobin less than 5 g/dL) or cardiac compromise, the rate of transfusion should be reduced to 2 mL/kg per hour to avoid fluid overload. Diuretics such as furosemide (1 to 2 mg/kg) may be necessary for some patients.
      - MONITORING
        
        Assessment and investigations such as HBsAg, Anti HCV and Anti HIV should be done every 6 months.
        
        Blood glucose should be monitored hourly if glucose infusion was interrupted during transfusion, since hypoglycaemia may occur suddenly
        
        Infusion should be slowed or stopped if any of the following develops during the transfusion
        
        Apnea or tachypnea
        
        Tachycardia, bradycardia or arrhytmia
        
        Cyanosis
        
        Significant change in systolic BP
        
        Significant change in body temperature
        
        Hemoglobinuria
        
        Patients should be assessed for hemolytic reactions if any adverse event is noted during a transfusion. Febrile and allergic reactions may respond to acetaminophen and diphenhydramine before future transfusions.