Q: 32 year old male with sickle cell disease presented to ER with severe episode of priapism. What should be considered?


Answer: For priapism in sickle cell disease, early exchange transfusion is indicated, meanwhile pain can be controlled with epidural analgesia. Exchange transfusions may be required to increase hemoglobin concentration to higher than 10% and decrease hemoglobin S to less than 30%. Before any intervention urology consult should be obtiained for possible use of terbutaline and pseudoephedrine. Some studies suggest that the use of terbutaline orally, at a dose of 5-10 mg, followed by another 5-10 mg 15 minutes later, if required, produces resolution in about one third of patients. Oral pseudoephedrine, 60-120 mg orally has also been suggested as a potential therapy due to its alpha-agonist effect. The exact efficacy of this medication orally is unknown.