Sickle cell disease status and outcomes of African-American men presenting with priapism

Introduction. Priapism is associated with sickle cell disease (SSD); however, few men receive education about this condition, which contributes to attenuated return of functional erections. Aim. To define the demographics, SSD status, and treatment outcomes of African-American men presenting with priapism. Main Outcome Measures. Demographics, medical history, self-report of sickle cell status, and outcome assessment using International Inventory of Erectile Function (IIEF) of men with priapism were retrospectively reviewed. Methods. A review of 39 cases of venocclusive priapism in African-American men was conducted. Charts were reviewed for demographics, medical history including SSD status by patient self-report, serum hemoglobin electrophoresis results, and priapism treatment and outcome. Results. Mean duration of presenting priapism episode was 22 +/- 12 hours (6-70 hours). Eight percent of men had priapism for < 12 hours, 59% 12-24 hours, 22% 24-36 hours, and 11% > 36 hours. All patients with priapism events of > 12 hours complained of reduction in erectile rigidity. No patients with priapism > 36 hours duration had return of spontaneous functional erections, but 44% (24-36 hours), 78% (12-24 hours) and 100% (< 12 hours) were able to generate functional erections with or without the use of sildenafil. Follow-up IIEF erectile function domain scores paralleled incidence of functional erections. Penile shunt surgery was required in 28%. Only 5% of men recalled learning that priapism was a complication of SSD. Six men denied a history of SSD; however, hemoglobin electrophoresis revealed abnormal hemoglobin S and elevated hemoglobin F levels in four of these men. Conclusions. The association of SSD and venocclusive priapism is well known in the medical community, yet few patients ever receive education regarding the emergency nature of the condition. The majority of men presents in a delayed fashion, and a significant proportion requires shunt surgery leading to long-term erectile dysfunction. Of those who denied having SSD, two-thirds had SSD by hemoglobin electrophoresis.