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Topics:
Pediatric Hematology/Oncology • Hematology • Sickle Cell • Benign Hematology • Pulmonology • Pulmonary Hypertension
How would you manage a patient with HbSS and severe pulmonary hypertension on home oxygen?
No evidence of VTE on multiple scans. On Crizanlizumab but not tolerant of hydroxyurea. The patient is admitted frequently for VOCs with acute on chronic hypoxic respiratory failure. Would you consider heart/lung transplant and/or gene therapy?
Answer from: at Academic Institution
This patient should be referred to a specialist in pulmonary hypertension in sickle cell disease for right heart catheterization and aggressive management of the pulmonary hypertension. As described, the patient is not a good candidate for lung transplant or gene therapy.
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