Hydroxyurea is one of the most successfully used
therapies for
sickle cell disease. Results of many clinical trials point to
hydroxyurea administration for patients with frequent painful crises and
acute chest syndrome.
Priapism is one of the complications that could be prevented by
hydroxyurea, but there are few reports demonstrating the results. Since November 1993,
hydroxyurea has been used in our clinic for preventing
priapism in patients with
stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed
stuttering priapism.
Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the
hydroxyurea dosage increased, the number or length of
priapism episodes decreased. One to two months after the maximal dose (20-35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg
hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity.
Hydroxyurea was discontinued in two patients, but
stuttering priapism reappeared.
Hydroxyurea was then re-introduced, and
priapism disappeared. One patient, using 20 mg
hydroxyurea/kg, had a 6-year remission of
priapism after
hydroxyurea administration; however, he experienced
stuttering priapism, 1 month before a major attack, that progressed to
impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that
hydroxyurea may prevent
priapism attacks in
sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention.