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.