A Review of Treatment Options for the Management of Sickle Cell Disease - Episode 6
Michael R. DeBaun, MD, MPH, provides insight on the screening and monitoring procedure for red blood cell transfusion for the management of sickle cell disease.
Michael R. DeBaun, MD, MPH: According to some of the recent guidelines, particularly the American Society of Hematology guidelines on red blood cell transfusion, ideally you would identify the patient’s minor red blood cell antigen phenotype. This is so that, in the event the patient develops red blood cell antibodies, you can know explicitly what would be or would not be compatible with that patient. I cannot underscore enough the importance of establishing an extended red blood cell phenotype, particularly for our adults, who often receive 1 or 2 units of blood, often inappropriately, for an acute vaso-occlusive pain episode while they’re in the emergency department in a non–sickle cell disease center, with the belief that this will abate the pain episode. But there is limited to no evidence that transfusing someone in the midst of a vaso-occlusive pain episode, for the sole reason of decreasing the acute vaso-occlusive pain episode length of time, is effective. Despite that, many adults will have multiple alloantibodies and become extremely difficult to transfuse, which puts their lives in peril, because often when there’s acute chest syndrome or multiorgan disease, the only antidote is the transfusion.
Some of the complications to red blood cell transfusions that we initiate for primary and secondary stroke prevention, and other indications for prevention of disease in this population, include acute red blood cell transfusion reaction, red blood cell alloimmunization, which I just mentioned, and viral-borne disease, which is now extraordinarily rare in the United States. Once you are transfused routinely, let’s say monthly for at least a year and often up to 2 years, typically you have excessive iron stores in your body. Then, we have to give you a treatment for the iron. So, not only do we have to treat you to prevent a complication of the disease, but then we have to give you another treatment to prevent the complications of the treatment. That’s chelation therapy, and that is quite cumbersome for individuals who are being transfused on a monthly basis. Consequently, that presents another barrier for families, having less than high levels of enthusiasm for monthly transfusions in this patient population.
Transcript Edited for Clarity