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Topics:
Pediatric Hematology/Oncology • Medical Oncology • Internal Medicine • Hematology • Sickle Cell • Pediatric Hematology • Benign Hematology
What is the role of hydroxyurea in variant sickle cell genotypes?
Hydroxyurea is demonstrated to reduce complications and improve long-term outcomes in severe genotypes HbSS and sickle beta0 thalassemia. What clinical features help you decide when to start hydroxyurea in other SCD genotypes (HbSC or sickle beta+ thalassemia, etc.)?
Answer from: at Academic Institution
I have had little success using hydroxyurea in my patients with hgb SC disease and at this point in patients with two or more acute visits over 12 months for pain, I am using crizanlizumab as some of the subjects in the phase 2 study on which the drug received FDA approval had SC disease (Ataga et a...
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Answer from: at Academic Institution
My success with hydroxyurea in patients with HbSC disease has been variable. Some have had excellent responses and have become advocates for hydroxyurea with their peers. Others have not responded. So my approach is to offer a 6 months-1 year trial to patients with this genotype. I do counsel them a...