Priapism: Epidemiology, Treatment, and Secondary Prevention

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Priapism, an undesired painful erection of the penis, is a common complication of sickle cell disease. Pilot data in children suggest that up to 90% of males with sickle cell anemia will experience priapism at least once by 20 years of age. If priapism persists 4 hours or more without detumescence the patient is at risk for irreversible ischemic penile injury, which may terminate in fibrosis and impotence. Yet, incidence is unclear, management remains anecdotal, treatment methods are inconsistent, and outcome has not been comprehensively evaluated. This research project includes three specific aims which will be addressed by a series of multi-center research studies. (1) To better understand which, when, and under what circumstances individuals develop priapism, we will administer an age appropriate and culturally sensitive questionnaire to a large cross section of American male adult and pediatric patients with sickle cell anemia. This will allow enumeration of prevalence, development of actuarial risk, and detailing of the common characteristics of priapism in persons with sickle cell disease. Patients participating in this part of the study will receive structured education about priapism. (2) To determine the efficacy and side effects of aspiration of the corpora cavernosa and irrigation with dilute epinephrine to induce immediate detumescence during prolonged episodes of priapism. (3) To determine the effectiveness of two different agents in secondary prevention of priapism. (3a) The ability of oral pseudoephedrine to decrease the frequency and severity of recurrent priapism in patients with two or more episodes of priapism in the preceding six months will be assessed in a double blind, placebo-controlled, crossover study. (3b) For patients with multiply recurrent episodes of priapism which interferes substantially with quality of life, the ability of a six-month course of depot leuprolide to prevent priapism will be assessed. Measurement of bone density as well as blood and serum markers of bone turnover will be used to investigate potential osteopenia from this therapy.