Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine

Abstract

Objectives: Priapism is a common and currently unsatisfactorily managed complication of sickle cell disease (SCD). In June 1994, 6 SCD patients received a new therapeutic regimen to prevent the occurrence and recurrence of priapism.

Methods: The patients (5 with SS and 1 with SC) were adults and had frequent episodes of stuttering priapism (SP), and two of them had had acute episodes (AP) lasting more than 3 hours. The treatment consists of preventive oral administration of the alpha-adrenergic agent etilefrine, and self-administered intracavernous injection (SICI) of the same agent to reverse episodes lasting more than 1 hour.

Results: Since the beginning of treatment, all patients were protected against AP, 4 patients had no recurrence with the oral treatment alone, 2 had to use SICI, 1 occasionally and 1 constantly. There was no modification of sexual activity and no complications. Blood pressure was unaffected.

Conclusions: This treatment is simple, cheap, and self-administered. It should be proposed to all patients with SCD in all geographic areas as part of an educational program for active prevention of this severe complication.