Recurrent ischemic priapism is a common complication of sickle cell disease (SCD). We assessed the burden, characteristics, and types of priapism, including sexual dysfunction, in a cohort of men with and those without SCD, to test the hypothesis that sexual dysfunction is more prevalent in men with SCD. In Kano, Nigeria, we conducted a comparative cross-sectional survey that included 500 and 250 men 18 to 40 years of age, with and without SCD, respectively. The survey used the Priapism Questionnaire and the International Index of Erectile Function for sexual function assessment. All eligible participants approached for the study gave informed consent and were enrolled. Stuttering and major priapism were defined based on the average duration of priapism experiences that lasted ≤4 and >4 hours, respectively. The prevalence of priapism was significantly higher in men with SCD than in those without it (32.6% vs 2%; P < .001). Stuttering priapism accounted for 73.6% of the priapism episodes in men with SCD. Nearly 50% of the participants with SCD-related priapism had never sought medical attention for this complication. The majority of the men with SCD-related priapism used exercise as a coping mechanism. Priapism affected the self-image of the men with SCD, causing sadness, embarrassment, and fear. The percentage of the men with SCD who had erectile dysfunction was more than twofold higher than that of those without SCD who had erectile dysfunction (P = .01). The men with SCD had a higher prevalence of priapism and sexual dysfunction than the men without SCD.

Despite priapism being one of the most frequent complications of sickle cell anemia (SCA) in male individuals, little has been reported about the impact of priapism in this population. The authors used a sequential independent mixed-methods design, which used both international multicenter focus group discussions (n = 35) and a quantitative patient-reported outcome measure (n = 131) to determine the impact of priapism on men with SCA in Nigeria and the United States. The authors analyzed data from focus groups using an iterative inductive-deductive approach. Comparison of the Priapism Impact Profile data was done using the Kruskal-Wallis H test. Our result showed that priapism, across cultures, is associated with shame and embarrassment. These emotions interfere with timely clinical and family communication about priapism symptoms and complications. Participants were dissatisfied with the quality of care at emergency facilities. The quality of life and physical wellness of men with SCArelated priapism were significantly different for the 3 groups: (1) priapism condition getting better, (2) priapism condition getting worse, and (3) priapism condition remain the same (P = 0.002 and P = 0.019, respectively). Psychological, sexual, and physical wellbeing are all adversely affected by priapism. Evidence-based methods are necessary for adequate medical, educational, and psychological treatment for recurrent priapism.

We conducted one of the first prospective studies to test the hypothesis that the clinical history of priapism underestimates priapism incidence compared to a priapism pain diary. Eligibility criteria were men with SCA between 18-40 years of age who have had at least 3 episodes of priapism in the past 12 months. Seventy-one men with SCA completed the diary for at least 3 months. Due to participant fatigue, only the first three months of the priapism diary were included in the analysis. A total of 298 priapism episodes were recorded, and 80% (57 of 71) of the participants had at least one priapism event. Priapism severity was reported as in the range of moderate to the worst imaginable pain in 81.5% (263 of 298), and the median pain rating was 6 (IQR: 5-8) on a scale from 1 to 10. The monthly incidence rate of priapism per participant based on history versus a self-report pain diary was 2.0 (95% confidence interval 1.9 - 2.1) and 1.4 (95% confidence interval 1.2 - 1.6), respectively (p<0.001). For participants that had a prior priapism episode, 80% had another episode during the three-month interval follow-up. The median time to that second episode was 27.5 days. Major priapism occurred in 9.9% of episodes and was associated with the sum of future priapism events. Men with SCA and at least 3 priapism episodes in the past 12 months are at significant risk for recurrent priapism in the following 3 months.

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