Treatment of Priapism in Pediatric Patients With Sickle Cell Disease

Brandon L. Maples; Tracy M. Hagemann

Disclosures

Am J Health Syst Pharm. 2004;61(4) 

In This Article

Diethylstilbestrol

Diethylstilbestrol is one of the few agents for which placebo-controlled trials exist. Its primary use has been in aborting and preventing attacks of SP. The exact mechanism by which diethylstilbestrol works is unknown, although existing theories suggest a neutralizing effect on male hormones or a direct effect on erythrocyte characteristics.[18]

A double-blind, placebo-controlled crossover study was conducted in 11 SCD patients (age 18-29 years) with SP who arrived at SCD clinics in Jamaica over a nine-month period.[18] Patients kept records of frequency, time, and duration of attacks for a two-week baseline period. They were then randomized to either the placebo or treatment group (oral diethylstilbestrol 5 mg daily) for two weeks. Patients who did not respond to treatment were given the alternative treatment for an additional two weeks. A cure was defined as the absence of attacks during the second week of the two-week treatment period. Nine patients completed the trial. Five patients were initially randomized to the placebo group, and four of these five continued having attacks after randomization (attacks in the fifth patient ceased immediately, but a pain crisis occurred six days into the study, after which priapistic attacks recommenced). All five patients were then given diethylstilbestrol, and attacks ceased immediately in four patients and gradually over eight days in the fifth patient. Of the four patients initially randomized to diethylstilbestrol, attacks ceased immediately in two, after three days in the third, and after four days in the fourth. Analysis of initial, crossover, and repeat treatments in the nine patients who completed the trial showed that attacks of priapism ceased with the use of stilbestrol (p = 0.031). If cessation of attacks after eight days is viewed as a response, then the statistical significance increases (p < 0.001). Adverse effects were minimal and included gynecomastia, mild gastrointestinal symptoms, and loss of normal erections.

After cessation of therapy, attacks resumed within two to eight weeks in five patients. In these five patients, attempts were made to adjust the minimum dosage necessary to allow normal erections while preventing recurrence of SP. These dosages varied within and between patients such that specific dosage ranges could not be recommended for use in all patients. However, it was possible for four of the five patients to find an individualized dosage that prevented priapism while allowing normal erections to occur. Overall, the authors concluded that diethylstilbestrol 5 mg daily for three to four days could abort attacks of priapism, and individualized dosages lower than 5 mg daily could be used to prevent priapism.

A report was published of two patients with SCD who were given diethylstilbestrol for the treatment of SP.[19] The first patient was a 20-year-old man given diethylstilbestrol after failure of treatment with oral etilefrine (30 mg every evening) for one month in combination with self-administered intracavernosal etilefrine injections. Diethylstilbestrol was administered as follows: 5 mg once during a 10-day period, 5 mg twice a week for one week, 2.5 mg twice a week for one week, 2.5 mg once a week for one week, 1 mg once a week for one week, and 0.5 mg once a week for one week. SP had decreased by the third day of treatment and stopped by the eighth day. Total detumescence persisted as the dosage was decreased to 2.5 mg twice a week, at which point SP returned, and etilefrine was reinstituted. A year later, the patient was using the injections daily, resulting in significant penile edema. Diethylstilbestrol was reintroduced and stopped SP for approximately three weeks, resulting in a total regression of the penile edema. The only reported adverse effect was gynecomastia.

The second patient was a 26-year-old man with SP occurring almost daily for three months. Etilefrine 30 mg orally was administered daily for two months without improvement. This patient was also started on diethylstilbestrol in a manner similar to the first patient. SP ceased on the sixth day of treatment but recurred when the diethylstilbestrol dosage was decreased to 1 mg weekly. Gynecomastia again was the only adverse effect reported. The authors indicated that diethylstilbestrol was effective for stopping prolonged erections but were unable to find a minimum effective dosage that could be applied to all patients.

Diethylstilbestrol has not been studied for the treatment of AP, but it does appear to be a viable option for the treatment and prevention of SP. It is one of the few agents to actually be studied in a controlled trial, giving it more credibility as a pharmacologic option for treatment of priapism. Adverse effects commonly experienced with diethylstilbestrol include breast tenderness and enlargement, nausea, vomiting, abdominal cramps, headache, dizziness, weight changes, edema, changes in libido, hirsutism, and testicular atrophy.[20] It is these adverse effects that prevent the recommendation and use of diethylstilbestrol as a first-line option for the treatment and prevention of SP.

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