Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function

Abstract

Objective: To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. Methods: This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. Results: Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. Conclusion: Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.

Epidemiology and treatment of priapism in sickle cell disease

“…1,18 Sexual and erec tile func tion assess ments using an IIEF or Erection Hardness Score should be con sid ered for all patients with priapism due to the asso ci ated high rate of erec tile dys func tion. 1,2 In acute priapism man age ment, exchange blood trans fu sion is some times used, but lim ited evi dence sup ports its effi cacy. We reserve exchange trans fu sion for a recal ci trant priapism epi sode not responding to cor po ral aspi ra tion while pre par ing for shunt uro logic sur gery inter ven tion.…”

“…Sickle cell dis ease (SCD) is asso ci ated with priapism, a per sis tent pain ful erec tion occur ring with or with out sex ual desire. 1,2 Priapism related pain is a car di nal fea ture of ische mia (low fl ow) due to com part ment syn drome. In con trast, nonischemic (high fl ow) priapism results from a trauma related arte rial injury.…”

“…1 Priapism occurs less com monly in chil dren youn ger than 18 years (3.6 % ). 1,2 Genome wide asso ci a tion stud ies have reported an asso ci a tion between priapism and poly mor phism in genes of transform ing growth fac tor -β recep tor ( TGFBR3 ), sub unit of fac tor VIII, integrin α V, aquaporin, and Klotho gene. 5 Distinguishing major priapism (last ing ≥ 4 hours) from stuttering priapism ( < 4 hour epi sode) is crit i cal for risk strat i fi ca tion of time depen dent priapism related erec tile dys func tion and penile fi bro sis.…”

Haptoglobin treatment contributes to regulating nitric oxide signal and reduces oxidative stress in the penis: A preventive treatment for priapism in sickle cell disease

“…Recurrent events of priapism promote fibrosis in the erectile tissue, which results in permanent erectile dysfunction ( Musicki and Burnett, 2020 ). Clinical studies have shown that 30–45% of men with SCD have episodes of ischemic priapism and of these 30% progress to erectile dysfunction ( Mantadakis et al, 1999 ; Adeyoju et al, 2002 ; Alvaia et al, 2020 ). Despite the high incidence of priapism, the main treatments available do not prevent priapism ( Salonia et al, 2014 ).…”