Introduction
Thalassemia is a disorder of hemoglobin production, one of the most common monogenic disorder in the world. Thalassemia is caused by mutations in the alpha or beta globin genes which lead to decreased synthesis of respective alpha and beta globin changes ultimately causing anemia.1 Children and adolescents with severe mutations require regular packed red blood cell (PRBC) transfusions every 3-5weeks to maintain a hemoglobin that allows for appropriate growth and development. Indications for transfusion include inability to maintain a hemoglobin baseline of >7g/dL or those who have complications of ineffective erythropoiesis, such as splenomegaly and frontal bossing.2
Care for a child with chronic illness such as thalassemia places a burden on both the child and family. Monthly appointments for PRBC transfusion therapy and clinical visits create a financial burden with co-payments, time constraints, missed work, childcare for siblings, parking and food etc. The culmination of these stressors impact the parents’ employment and quality of life of both the patient and their family.3 As children reach young adulthood this burden carries over to their own employment and family life. Within the health care system, a longer length of stay for transfusion therapy incurs more health care resources and costs.
For the child with transfusion dependent thalassemia, PRBC transfusions administered in an infusion center by the medical team are part of their health maintenance treatment; children receiving them are healthy and without acute symptoms. The clinical procedure for a red cell transfusion is based on guidelines from national and international professional hematologic organizations. The American Association of Blood Banks (AABB) states that patients should be closely monitored for transfusion reaction during the first 15 minutes of each unit of a PRBC transfusion.4 The rate of transfusion can be as fast as the patient can tolerate but each unit of PRBCs must be completed within 4 hours.4 Lal and colleagues2report that the traditional transfusion rate used in children with thalassemia is 5mL/kg/hr transfusion rate for children with adults receiving a unit of PRBCs in one hour. At our institution, PRBC transfusions for children with thalassemia who are otherwise healthy, are administered at a rate of up to 10mL/kg/hr of red cells.
When pediatric patients reach young adulthood and are transitioned to an adult hematology clinic, or patients are transfused at facilities closer to home, they have reported that each PRBC transfusions take longer. For young children, many institutions will titrate up to a maximum rate of 5mL/kg/hr, resulting in infusions taking more than 3 hours. For adolescent and young adults, each unit of PRBCs may be transfused at rates between 2 to 4 hours. Thus for an adult patient receiving 3 units of PRBCs, overall transfusion time can increase up to 6 to 12 hours. These prolonged infusions negatively impact the quality of life of the patient and family.
To our knowledge, there are no publications that summarize patient outcomes when red cells are infused at a faster rate in an outpatient setting. The purpose of this study is to examine the safety of the 10mL/kg/hr transfusion rate through summarizing the outcomes of children with thalassemia undergoing PRBC transfusions.