Sickle Cell Information Center Protocols

by James Eckman, M.D. and Allan Platt, PA-C

Priapism

Priapism is the persistent, painful erection of the penis. It is relatively common in young males with sickle cell anemia. The onset may be in early childhood or adolescence. Recurrent or severe episodes can result in future impotence. Treatment is often not satisfactory.

Present Illness. Note time of onset of symptoms, frequency and duration of attacks, time of night, pain, dysuria, frequency, discharge. Association with dreams, sexual activity, or masturbation. Determine response to previous therapy.

- Vital Signs: Temperature, blood pressure.

- Genitourinary: Document size and turgor of penis. Soft glans and ability to urinate indicate little involvement of the corpora spongiosa. Glans involvement indicates tricorporal priapism. Rule out discharge and inguinal adenopathy.

- Rectal: Prostate size and tenderness.

Minimum Evaluation. CBC with differential, reticulocyte count, and urinalysis.

Additional Evaluation. Nucleotide imaging, flow Doppler, MRI and pH/PO₂ of aspirated blood can differentiate low flow from normal flow priapism. Urine culture and sensitivity if urinalysis positive or if prostate tender. Smear and culture if urethral discharge is present.

- Priapism. The diagnosis of priapism is usually obvious from the physical examination. Patients usually present with the spontaneous onset of erection which frequently occurs in the middle of the night. Can be precipitated by prolonged intercourse or masturbation. Penis remains erect and is very painful. If there is a history of nocturnal erection, pain and persistence for more than thirty minutes must be present to differentiate priapism from normal nocturnal erections. There are three basic patterns seen with priapism.

- Stuttering Priapism. Many patients, especially children, have a pattern of multiple short episodes over a period of days or several weeks. The priapism is often normal flow and prognosis is generally good and therapy is conservative.

- Acute, Severe Priapism. These are characterized by persistence of the erection for hours, days, or weeks with moderate to severe pain. Impotence is probably increased in most patients with this pattern.

- Chronic, Relapsing Priapism. Patient have chronic partial or painful erections or frequent relapses over months. Priapism is usually low flow and fibrosis is often present making treatment less satisfactory and impotence a common sequela.

- Secondary Priapism. Adults with priapism may have associated urinary tract infection, prostatitis, or venereal disease. Fever, discharge, dysuria, frequency suggest infection.

Treatment

The therapy for priapism is not well defined and is generally not very effective. All patients should be treated with bed rest, intravenous hydration, analgesics and sedation. warm sitz baths may be effective at onset. The aggressiveness of therapy must be based on the severity and duration of symptoms. There are no controlled studies which determine the effectiveness of treatment in arresting the acute problem and in preventing subsequent sexual dysfunction.

- Stuttering Priapism. Most patients are treated with hydration and pain control for each episode. Intervention with oral pseudoephedrine has bee effective for treatment and prevention. Other pharmacologic interventions that have been tried include vasodilators, and calcium channel blockers. Chronic transfusion for six months has been recommended if attacks are frequent.

- Acute, Severe Priapism. These are initially treated with bed rest, I.V. hydration with D5W and run at 250 cc./hour (Children - 5 cc./kg./hour), administration of Morphine Sulfate 0.15 mg./kg. (8-15) I.M. q 4 hours with hydroxyzine HCl (Vistaril) 25 mg. I.M. q 4 hours, administer Amyl nitrate 0.3 ml by nasal inhalation and monitor blood pressure, or sublingual or dermal nitroglycerine. We have also found nifedipine 10 mg P.O. effective in some patients. Penile aspiration and irrigation with alpha adrenergic agents (phenylephrine and epinephrine) has been found to be effective in children and adults with high flow priapism. If unimproved in two hours or if improved but persistent for eight hours, admit to hospital for transfusion and consideration for surgery. Consider for immediate exchange if acute and persistent and chronic regular transfusion if recurrent. In general, the results of exchange transfusion have been inconsistent and can be associated with acute neurological events. Chronic transfusion does appear to be effective in reducing frequency of recurrent episodes. Although initial control of an acute episode may be accomplished with surgical shunts, we find recurrences are frequent and impotence may be increased. Shunts are of little benefit in tricorporal priapism.

- Chronic, Relapsing Priapism. Patients with chronic or relapsing episodes are best treated with hypertransfusion for a period of six months. Patients with nocturnal episodes occasionally respond to nitroglycerine by dermal patch or pseudoephedrine at bed time. Estrogens, gonadotropin-releasing hormone analogues, surgical corpectomy with penile prosthesis are reported to benefit those with severe, frequent attacks.

- Secondary Priapism. The underlying infection must be treated to prevent recurrent episodes.

Nursing Considerations

 Determine hydration status and manage fluid volume deficit, bladder catherization may be needed.

Encourage the patient to seek counseling and evaluation of persistent erection - persistent erection is an embarrassing health problem with significant personal and interpersonal implications for affected individuals.

 Assist the male patient to alter behavior such as cigarette smoking, drinking alcohol or taking illicit drugs which may predispose the persistent erection or sickle cell pain.

 Explain that other therapies are available.

 Monitor body temperature and other symptoms. Early detection of infections and treatments may prevent dissemination pathogens.

 Be sensitive and compassionate to patient’s need for privacy and feelings of quilt and fear.

 Encourage patients to empty their bladder before bedtime to prevent nighttime onset.

 Severe disease may cause infertility or impotence.

 Home therapy includes bladder emptying, hydration, warm baths and analgesia at the first onset of symptoms.

The prevention of recurrent episodes is often problematic. Activities that precipitate attacks should be avoided. Early aggressive treatment with exchange transfusion is recommended in severe cases and surgery often performed if the erection persists for greater than 48 hours even though no controlled studies document the effectiveness of either in terminating an acute episode, preventing recurrence, or reducing the incidence of impotence. Pseudoephedrine may be the most consistent prophylactic medication.

The onset of priapism is frightening, embarrassing, and often associated with guilt. The patient and parents of children should be given education about the relationship of priapism to the sickle syndrome in a compassionate and professional manner. Parents of children and patients should be taught to initiate immediate treatment and to seek medical assistance for attacks. Immediate emptying the bladder, oral hydration and warm baths may terminate the acute episode. Patients should be told to avoid activities which precipitate acute attacks. Individuals with severe or recurrent attacks must be educated about the potential for impotence in the future. Those with impotence need education about the availability of penile prosthetic implants to regain sexual function.

Education Brochures for printing in PDF format at http://www.sicklecell-info.org/EdMaterials.html

Ohene-Frempong K Steinberg MH. Clinical aspects of sickle cell anemia in adults and children. in Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Steinberg MH, Forget BG, Higgs DR, Nagel RL. Cambridge University Press Cambridge UK 2001. Pp. 644-648.

Seeler RA. Priapism in children with sickle cell anemia. Successful management with liberal red cell transfusions. Clin. Pediatr. 10(7):418-419, 1971.

Rifkind S, Waisman J, Thompson R, Goldfinger D. RBC exchange pheresis for priapism in sickle cell disease. J.A.M.A. 242(21):2317-2318, 1979.

Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch. Intern. Med. 140:1434-1437, 1980.

Noe HN, Wilimas J, Jerkins GR. Surgical management of priapism in children with sickle cell anemia. J. Urol. 126:770-771, 1981.

Gradisek RE. Priapism in sickle cell disease. Ann. Emerg. Med. 12(8):510-512, 1983.

Walker EM, Mithcum EN, Rous SN, et al. Automated ery-throcytopheresis for relief of priapism in sickle cell hemoglobinopathies. J. Urol. 130:912-916,1983.

Pantaleo-Gandais M, Chacon O, Chalbaud R, Plaza N. Priapism: Evaluation and treatment. 24(4):345-346, 1984.

Bertram RA, Carson CC, Webster GD. Implantation of penile prostheses in patients impotent after priapism. Urology 26(4):325-327, 1985.

Douglas l Flectcher H, Serjeant GR. Penile prosthesis in the management of impotency in sickle cell disease. Brit J Urol 65:533-535, 1990.

Hamre MR, Harmon EP, Kirkpatrick DV, Stern MJ, and Humbert JR. Priapism as a complication of sickle cell disease. J Urol 145:1-5, 1991.

Fowler JE, Koshey M, Strub M, and Chinn SK. Priapism associated with the sickle cell hemoglobinopathies: Prevalence, natural history, and sequelae. J Urol 145:65-68, 1991.

Siegel JF, Rich MA, Brock WA. Association of sickle cell disease, priapism, exchange transfusion and neurological Events: Aspen Syndrome. J Urol 150:1480-1482.

Levine LA, Guss SP. Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia associated Priapism. J Urol 150:475-477, 1993.

Sharpsteen JR, Powars D, Johnson et al. Multisystem damage associate with tricorporal priapism in sickle cell disease. Amer J Med 94:289-294, 1993.

Miller ST, Roa SP, Dunn EK, Glassberg KI. Priaapism in children with sickle cell disease. J Urol 154:844-847, 1995.

Powars DR, Johnson CS. Priapism Hemeatol/Oncol Clin N Amer 10:13631372, 1996.

Mantadakis E, Cavender J, Rogers ZR, Buchanan GR. Outpatient peile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism.  Blood 95:78-82, 2000.

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Last modified: September 06, 2006