A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study

Abstract

Priapism is defined as a prolonged, persistent, and purposeless penile erection. It is a common (35%) but frequently understated complication in young men and adults with sickle cell disease. We had previously demonstrated an association between stuttering attacks (<4 hours) and an acute catastrophic event with its consequent problems of erectile dysfunction and impotence. We describe a randomized, placebo-controlled, clinical study looking at medical prophylaxis with 2 oral α-adrenergic agonists, etilefrine and ephedrine, in preventing stuttering attacks of priapism. One hundred thirty-one patients were registered into a 2-phase (observational and intervention phase) study, and 86 patients (66%) completed Phase A diary charts. Forty-six patients (59%) completed a 6-month treatment phase (Phase B), and the remaining patients were lost to follow-up despite persistent efforts to contact them. Various reasons are postulated for the high attrition rates. The drugs were well tolerated, and no serious adverse events were reported. There was no significant difference among the 4 treatment groups in the weekly total number of attacks in Phase B (analysis of covariance P = .99) nor among the average pain score per attack after adjusting for attack rates and pain scores in Phase A (analysis of covariance P = .33). None of the patients who completed the study required penile aspiration at study sites while on medical prophylaxis. Young men with sickle cell disease are not comfortable engaging with health care providers about issues relating to their sexual health. The full impact of an improved awareness campaign and early presentation to hospital merits further standardized study. Priapism still contributes seriously to the comorbidity experienced by this previously inaccessible group of patients and medical prophylaxis with oral α-adrenergic agonists is feasible. Future international collaborative efforts using some of the lessons learnt in this study should be undertaken.