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Expert Opinion Hydroxyurea Could Prevent Strokes for People with Sickle Cell Disease in Lieu of Transfusions During COVID-19 Blood Supply Shortage

(WASHINGTON, April 13, 2020) — The COVID-19 pandemic threatens to cause a blood supply shortage in the United States, and as a result, clinicians may be compelled to ration or conserve blood products. For people living with sickle cell disease (SCD), blood transfusions are often necessary to decrease the frequency of severe symptoms. In a new Blood paper, Michael R. DeBaun, MD, MPH, of Vanderbilt University Medical Center, Vanderbilt-Meharry Sickle Cell Disease Center of Excellence, suggests that while monthly blood transfusions are the standard method of stroke prevention for children with SCD and at high risk of strokes, initiation of hydroxyurea therapy may be an effective alternative, if the monthly blood transfusions are interrupted. The paper presents the advantages and challenges of stroke prevention treatment without regular blood transfusion availability and suggests that low-dose hydroxyurea, with consideration of increasing the dose later, should be started immediately to offer clinical benefits and pose minimal risks to children living with SCD at risk for strokes.

“During the COVID-19 pandemic, we are in an uncharted period of time for health care delivery, particularly for our most vulnerable population, children with a chronic disease, SCD, who require monthly visits to the hospital for blood transfusion therapy for preventing strokes,” said Dr. DeBaun. “Fortunately, we have the benefit of clinical research conducted in the U.S., Nigeria, and Jamaica, where strong evidence indicates hydroxyurea is a reasonable alternative to blood transfusion therapy for preventing strokes when compared to no therapy at all. We are taking every precaution to continue regular blood transfusions to prevent strokes, but if we cannot, we have a viable option to mitigate the risk of strokes in children with SCD. We have offered this strategy to all of our children at Monroe Carell Jr. Children’s Hospital at Vanderbilt receiving regular blood transfusion therapy to prevent strokes, and approximately 90% of the families have started low-dose hydroxyurea while receiving ongoing blood transfusions.”

Blood editor-in-chief Nancy Berliner, MD, said, “the COVID-19 pandemic represents a threat to blood availability for sickle cell patients who depend on chronic transfusion for stroke prevention. The common-sense recommendation to immediately add low-dose hydroxyurea to their treatment regimen may provide a ‘safety net’ for sickle cell patients who have to forego exchange transfusion in the coming weeks and months.”

Blood (www.bloodjournal.org), the most cited peer-reviewed publication in the field of hematology, is available weekly in print and online. Blood is a journal of the American Society of Hematology (ASH) (www.hematology.org).

Leah Enser, American Society of Hematology
[email protected]; 202-552-4927