ABSTRACT

Priapism is a devastating complication of sickle cell disease (SCD) with no uniformly accepted prophylaxis. Hydroxyurea (HU) has shown benefit in preventing chronic complications of SCD. We sought to determine the association between HU therapy and the prevention of priapism episodes in men with SCD. A retrospective case series study was conducted between July 1 and September 30, 2014, recruiting men treated at the Sickle Cell Unit, The University of the West Indies, Mona, Jamaica, who had a history of priapism and exposure to HU therapy. A questionnaire on priapism was administered, and changes in the characteristics of priapism episodes relative to HU therapy were recorded. Ten men with a mean age of 26.2 ± 10.7 years consented for the study. The most frequent indication for HU use was prevention of recurrent painful crises (50%). Three patients had baseline priapism episodes occurring daily or on alternate days. The baseline duration of the priapism episodes was greater than two hours in 50% of those interviewed, with 30% lasting over five hours. Five (50%) of the patients had cessation of episodes while on HU. Of the patients who continued to have priapism while on HU therapy, four had episodes lasting less than one hour, with one patient having episodes lasting beyond five hours. Chronic HU therapy appeared to be associated with an amelioration of recurrent priapism episodes in men with SCD.