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Priapism and erectile dysfunction in sickle cell disease

Joshua J Field, MD
Vijaya M Vemulakonda, MD, JD
Michael R DeBaun, MD, MPH
Arthur L Burnett, MD, MBA, FACS
Section Editors
Stanley L Schrier, MD
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD


Priapism (penile erection in the absence of sexual activity or desire) is a common complication of sickle cell disease (SCD) in men. The vast majority of cases are ischemic, in which increased pressure compromises the vascular circulation (ie, a type of compartment syndrome). Over time, repeated episodes cause permanent damage and erectile dysfunction. Thus, priapism is considered a medical emergency in which timely diagnosis and appropriate management are vital to preserving normal function. This is a challenging management area because there are few experts dedicated to managing priapism in SCD and few large trials on which to base practice, and management often involves multiple specialties including urology, emergency medicine, pediatrics, and hematology.

This topic review discusses our approach to the evaluation and treatment of priapism and erectile dysfunction in boys and men with SCD.

Related subjects, including the use of hydroxyurea and regular blood transfusion, as well as overviews of the other clinical findings and routine management of SCD, are presented separately:

Hydroxyurea – (See "Hydroxyurea use in sickle cell disease".)

Transfusion – (See "Red blood cell transfusion in sickle cell disease".)

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Literature review current through: Nov 2017. | This topic last updated: Nov 15, 2017.
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