Preventive and curative treatment of priapism in sickle cell disease.

Priapism is a frequent and serious cause of morbidity in males with sickle cell disease. Acute priapism (AP) is preceeded in two-thirds of the cases by repeated minor events called stuttering priapism (SP). Since 1994, we have used a specific approach to prevent the commonly devasting effects of AP, using the alpha adrenergic agent etilefrine. Treatment of AP has been simplified (drainage without aspiration followed by one or two intracavernous injections (ICI) of 10 mg of etilefrine, until detumescence). For SP lasting more than one hour or causing pain, we use oral etilefrine and/or self ICI. This strategy was effective in five patients seen having AP, 21 patients with SP; it is simple, cheap, and avoids surgical procedure and transfusion. Morever, erectile dysfunction, present in three patients, has been treated safely by ICI of protaglandins.