MANAGEMENT OF SICKLE CELL DISEASE

NIH Publication No. 02-2117. Revised May28, 2002 (Forth Edition) National Institutes of Health, National Heart, Lung, and Blood Institute. Download the entire PDF  file for Adobe   Click Here to return to Contents

Chapter 20  PRIAPISM

Priapism, defined as a sustained, painful, and unwanted erection, is a well recognized complication of sickle cell disease (SCD) (1-3). According to one study, the mean age at which priapism occurs is 12 years, and by the age of 20, as many as 89 percent of males with SCD will have experienced one or more episodes of priapism (1). Priapism in males with SCD is due to vaso-occlusion, which causes obstruction of the venous drainage of the penis. Priapism can be classified as prolonged if it lasts for more than three hours or stuttering if it lasts for more than a few minutes but less than three hours and resolves spontaneously; however, such stuttering episodes may recur or develop into more prolonged events. Prolonged priapism is an emergency that requires urologic consultation.

Recurrent episodes of priapism can result in fibrosis and impotence, even when adequate treatment is attempted. Currently, there is no single standard of care for the treatment of priapism; the information provided below represents current efforts with respect to the treatment of this complication of SCD.

PSYCHOSOCIAL AND COUNSELING ASPECTS OF PRIAPISM

Beginning in early boyhood, males need to know that priapism is one aspect of SCD and that this is not an event that should embarrass them. One study found that only 7 percent of boys and men with SCD who had not experienced priapism knew that it could be a complication of SCD (1). This information prompted the authors to prepare a brochure explaining priapism, which was distributed to all males and their families. Boys and young men, as well as their families, need to know that they should be prepared to seek medical attention as soon as an episode begins and that if untreated, priapism can result in impotence in the future. The males should know that a full bladder can trigger priapism, and they therefore need to urinate regularly. They also should avoid prolonged sexual activity, which can trigger an episode. If they have had more than one episode, medications can be prescribed that may prevent recurrences.

When evaluating a patient with priapism, the physician or nurse should document the time of onset of the episode as well as the presence of any other inciting factors, such as trauma, infections, or the use of drugs (e.g., cocaine, alcohol, psychotropic agents, sildenafil, testosterone) (4-6). A careful physical examination should reveal a hard penis with a soft glans. 

The goal of therapy is to ease pain, make the erection go away, and preserve future erectile function. If treatment is given within 4 to 6 hours, the erection can generally be reduced with medication and conservative therapy. Most of the articles in the literature concern anecdotal reports and few randomized trials are available.

Patients should be advised to drink extra fluids, use oral analgesics, and attempt to urinate as soon as priapism begins.

EPISODES LASTING MORE THAN TWO HOURS

Patients should go to the emergency room to receive intravenous hydration and parenteral analgesia. According to one protocol (7), if detumescence does not occur in 1 hour after the patient has arrived in the emergency room, penile aspiration is initiated (procedure should be performed within 4 to 6 hours from onset of priapism). The patient receives conscious sedation and local anesthesia; blood is then aspirated from the corpus cavernosum with a 23-gauge needle followed by irrigation of the corpora with a 1:1,000,000 solution of epinephrine in saline. In a prospective nonrandomized unblinded study, this procedure was successful in producing immediate detumescence in 15 males on 37 of 39 occasions (7). This study was performed in males who were teenagers or younger and has not been validated in an older population. 

The concomitant use of automated red cell exchange transfusions to reduce the sickle hemoglobin (Hb S) level to less than 30 percent can also be considered, especially if early intervention with irrigation fails (8). It is unclear if simple transfusion is equivalent to exchange transfusion. The clinical response to exchange transfusions is variable, and side-effects range from headaches or seizures to obtundation requiring ventilatory support. The association of SCD, priapism, exchange transfusion, and neurological events has been given the name ASPEN syndrome (9). Recurrent priapism is strongly associated with the development of impotence, therefore some physicians transfuse patients as though they were on a stroke protocol (maintenance of Hb S level below 30 percent). These programs should be limited in duration (6 to 12 months), and patients should be assessed often.

If there is recurrence despite aspiration and local instillation of vaso-active drugs, shunting may be considered. In this procedure, known as the Winter procedure, a shunt is created between the glans penis and the distal corpora cavernosa with a Tru-cut biopsy needle; this allows blood from the distended corpora cavernosa to drain into the uninvolved corpus spongiosa (10). A larger shunt can be created if this is not successful.

Additional medications used for reversal of priapism have included a-agonists and -agonists such as terbutaline (11-13). Clinicians in France and West Africa have used an a-agonist (etilefrine) that can be injected by patients into the cavernous sinus (12-13); however, this agent is not available in the United States.

None of these agents has been validated in a well-controlled trial and thus cannot be endorsed at this time.

Complications of priapism and treatment include bleeding from the holes placed in the penis as part of the aspiration or shunting procedures, infections, skin necrosis, damage or strictures of the urethra, fistulas, and impotence. If impotence persists for 12 months, the patient may wish to consider implantation of a semirigid penile prosthesis (14).

There are no large clinical studies documenting ways to prevent priapism. Some physicians prescribe 30 mg of oral pseudoephedrine at night as an attempt to prevent further episodes in those who have had priapism and have required aspiration and irrigation (7).

Injections of leuprolide, a gonadotropinreleasing hormone analogue that suppresses the hypothalamic-testicular axis and the production of testosterone, also has also been used with some degree of success as prophylaxis against further episodes (15). A small (11 patients) double-blind, placebo-controlled crossover study found that oral stilbestrol in doses of 5 mg daily for 3 to 4 days could abort episodes of priapism and that much smaller doses could prevent recurrence (16).

Although hydroxyurea may potentially be of benefit (17), clinical studies to determine its efficacy in preventing priapism have not been performed.

1. Mantadakis E, Cavender JD, Rogers ZR, et al. Prevalence of priapism in children and adolescents with sickle cell anemia. Am J Pediatr Hematol Oncol 1999;21:518-22.

2. Powars DR, Johnson CS. Priapism. Hematol Oncol Clin North Am 1996;10:1363-72.

3. Miller ST, Rao SP, Dunn EK, et al. Priapism in children with sickle cell disease. J Urol 1995;154:844-7.

4. Saenz-de-Tejada I, Ware JC, Blanco R, et al. Pathophysiology of prolonged penile erection associated with trazodone use. J Urol 1991;145:60-4.

5. Kassim AA, Fabry ME, Nagel RL. Acute priapism associated with the use of sildenafil in a patient with sickle cell trait. Blood 2000;95:1878-9.

6. Slayton W, Kedar A, Schatz D. Testosterone induced priapism in two adolescents with sickle cell disease. J Pediatr Endocrinol Metab 1995;8:199-203.

7. Mantadakis E, Ewalt DH, Cavender JD, et al. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 2000;95:78-82.

8. Walker EM Jr, Mitchum EN, Rous SN, et al. Automated erythrocytopheresis for relief of priapism in sickle cell hemoglobinopathies. J Urol1983;130:912-6.

9. Rackoff WR, Ohene-Frempong K, Month S, et al. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992;120:882-5.

10. Winter CC. Priapism cured by creation of fistulasbetween glans penis and corpora cavernosa. J Urol 1978;119:27

11. Lowe FC, Jarow JP. Placebo controlled study of oral terbutaline and pseudoephedrine in management of prostaglandin E1-induced prolonged erections. Urology 1993;42:51-3.

12. Virag R, Bachir D, Floresco J, et al. Ambulatory treatment and prevention of priapism using alphaagonists. Apropos of 172 cases. Chirurgie 1997;121;648-52.

13. Jarmon JD, Ginsberg PC, Nachmann MM, et al. Stuttering priapism in a liver transplant patient  with toxic levels of FK506. Urology 1999;54:366.

14. Douglas L, Fletcher H, Serjeant GR. Penile prostheses in the management of impotence in sickle cell disease. Br J Urol 1990;65:533-5.

15. Levine LA, Guss SP. Gonadotropin-releasing hormone analogues in the treatment of sickle-cell anemia associated priapism. J Urol 1993;150:475-7.

16. Serjeant GR, DeCeulaer K, Maude GH. Stilbestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 1985;2:1274-6.

17. Jam’a AH, Al Dobbous IA. Hydroxyurea in the treatment of sickle cell associated priapism. J Urol 1998;159:1642.

Steidle C. Priapism. In: The Impotence Sourcebook. New York: RGA Publishing Group, 1998.

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Last modified: November 09, 2002