Priapism in sickle cell disease

Priapism is, for most patients, an unpredictable, recurrent event associated with devastating consequences for erectile function and quality of life. It occurs in a significant number of males who have all forms of sickle cell disease. Patients should be educated about priapism and currently available treatment options; this education should focus on the need to report recurrent events and to present for care within 4 hours of onset during a prolonged episode. Hematologists, as the physicians invested in the longitudinal care of such patients, must actively oversee the management of episodes, particularly of prolonged priapism, to ensure access to interventions such as aspiration and irrigation when these are required. Given such interest, it should be possible to design and conduct large-scale clinical trials of available intervention strategies to reach consensus about optimum strategies to terminate and prevent severe, frequently recurrent episodes of priapism in patients who have sickle cell disease.