Cross Sectional Survey of Priapism and Sexual Dysfunction in 353 Men with Sickle Cell Disease
Authors: Idris Ibrahim Musa , Galadanci Jamil Aliyu , Abba Akib , Mashi Sharfuddeen Abbas , Uzoma Anele , Bonnet Kemberlee R , Schlundt David G , Hussain Nafiu , Burnett Arthur , DeBaun Michael R. 
Published: November 2019

Introduction:
In both high and low income countries, life span in sickle cell disease (SCD) is increasing and quality of life is improving. Recent research activities have focused on decreasing SCD-related morbidities. However, limited research has been done on clinical epidemiology of priapism, sexual dysfunction and libido in men with SCD. As part of our ongoing Priapism in Nigeria (PIN) cohort to assess knowledge gap in clinical epidemiology of priapism and sexual dysfunction in men with SCD, we tested the hypothesis that sexual dysfunction is high in men with SCD compared to age/race matched men without SCD.
Methods:
We utilized a mixed method study design (cross-sectional survey and focus group discussions) in men, aged 18-40 years, with confirmed SCD and men without SCD as comparators for the survey. Participants were recruited from the adult sickle cell clinic and general outpatients department of Aminu Kano Teaching Hospital (AKTH) and Murtala Mohammed Specialists Hospital (MMSH) in Kano, Nigeria from February to July 2019. Priapism is defined as a purposeless painful erection, unrelated to sexual desire; and mostly occurs in the stuttering or recurrent ischemic form, which lasts less than 4 hours. We used the validated International Index of Erectile Function (IIEF) questionnaire to assess erectile dysfunction in this population. Additionally, we conducted 6 focus group discussions in Nigeria (3 sessions) and United States (3 sessions), respectively; in which we asked open ended questions about symptoms, experiences, beliefs and life impacts of priapism.
Data were presented as means ± standard deviation or proportions with 95% Confidence Intervals. The t-test and Chi square test were used to compare demographic data. Similarly, domain-specific scores, which included: 1) erectile function, 2) sexual desire, 3) orgasmic function, 4) overall satisfaction with sex life, and 5) intercourse satisfaction- were compared between the two groups using t-test; where higher scores indicate better sexual function. Erectile domain was further sub-classified into normal (26-30), mild ED (22-25), mild-moderate ED (17-21), moderate ED (11-16) and severe ED (0-10). We considered alpha level of significance to be <0.05. The focus group data was analyzed using an iterative inductive/deductive approach.
Results:
A total of 353 men with SCD and 250 men without SCD were evaluated; for the demographic features the only significant difference was in monthly income (p 0.007) with SCD being higher, table 1. The prevalence of any priapism episode (major or stuttering) in men with and without SCD was 31.72% (112 of 353) and 2% (5 of 250). Among men with SCD, 25.9% (29 of 112) and 74.1% (83 of 112) had major and stuttering priapism episodes, respectively. Based on the IIEF, the men with SCD when compared to men without SCD had significantly lower total mean scores (24.9 vs 29.6, p 0.0002), erectile function (9.9 vs 11.5, p 0.005), sexual desire (5.8 vs 7.1, p<0.0001), and overall satisfaction with sex life (2.3 vs 3.5, p<0.0001). No significant difference in orgasmic function (p=0.29) and intercourse satisfaction (p=0.12) was observed. Among the married men with and without SCD, 55% (21 of 38) and 84% (22 of 26) had normal erectile function, and 26.3% (10 of 38) and 11.5% (3 of 26) had severe erectile dysfunction, respectively.
Based on the data from our focus groups with a total of 28 and 7 participants (n=35), in Nigeria and United States respectively, we were guided by biopsychosocial and socio-ecological models to develop a conceptual framework (not shown). We identified themes on cultural context, priapism (triggers, schema, episodes and coping strategies), emotional and sexual function impacts, with respective quotes (results not shown).
Conclusions:
In the largest cross-sectional and qualitative study of priapism in men with SCD to date, we demonstrated that priapism and sexual dysfunction are significant cause of morbidity when compared to men without SCD. Married participants with SCD have 2-fold greater proportion of severe erectile dysfunction than those without SCD. Our qualitative results revealed tremendous impact of priapism and sexual dysfunction in men with SCD. Participants with priapism experience shame, anxiety and depression, and declined sexual function. There was diversity in causal attribution and coping strategies of priapism.

Disclosures
Idris: Fogarty International Center: Research Funding.