Part 1: Sickle Cell Disease

Vivien A. Sheehan, MD, PhD

Assistant Professor of Pediatrics, Baylor College of Medicine/Texas Children’s Hematology Center, Houston, TX

Question 1

A 23-year-old African American woman with homozygous hemoglobin SS (HbSS) disease presents to initiate care. She has been on monthly simple transfusion therapy since she was found to have a transcranial Doppler (TCD) velocity of 220 cm/sec in her right middle cerebral artery at age 7 years. She has not had an overt stroke, and her last magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA), at age 16, was negative for moya-moya or significant stenosis. There were mild ischemic changes in the frontal lobe consistent with silent infarcts. She has no allo- or autoantibodies, and is on an iron chelator, with a ferritin level under 1000 ng/mL. She wants to know when she can discontinue transfusion therapy.

How should the physician respond to this patient's question regarding stopping transfusion therapy?

  •   She can stop now, as she is an adult and no longer at risk for stroke
  •   She can safely switch to hydroxyurea at any time, as it has been shown
    conclusively to prevent stroke with efficacy equal to that of chronic transfusions
  •   She can undergo dural inversion therapy and discontinue transfusions
  •   She should continue transfusion therapy indefinitely

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