Stuttering/Recurrent Priapism

Background
Stuttering priapism is the occurrence of multiple self-limited episodes of unwanted, often painful erections lasting <4 hours. Priapism, including stuttering priapism, is common, affecting 35 percent of boys and men with SCD. Stuttering priapism may lead to a major episode of greater than 4 hours duration and may adversely affect quality of life and lead to impotence. Treatment with chronic hormonal therapy, transfusion therapy, and other treatments may reduce or eliminate these episodes; however, there are no data demonstrating improvement in functional outcomes. Therefore, the decision to treat must be balanced against the side effects of interventions, which can include decreased libido. Summary of the Evidence One RCT, 7 observational studies, and 39 case reports described priapism in the setting of SCD. Of these, only two studies evaluated the chronic management of priapism: the RCT and one observational study. Both studies were small, thus making the overall quality of the evidence very low. The RCT noted cessation of bouts of priapism with stilbestrol during a 2-week crossover phase in nine men with SCD. The observational study involved 35 participants and examined the effects of finasteride on recurrences of priapism. It reported a decrease in the number of priapic episodes and increased length of time between episodes. There are no data demonstrating improvement in functional outcomes, so the potential benefits must be balanced against the side effects of interventions, including decreased sexual function. However, even in the absence of RCTs demonstrating long-term benefit, individualized therapy devised in consultation with a urologist may be considered for symptomatic relief. Recommendations In men and boys with SCD and recurrent or stuttering priapism offer evaluation and treatment in consultation with a sickle cell disease specialist and a urologist, especially when episodes increase in severity or frequency. (Weak Recommendation, Low-Quality Evidence)