DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS  the Sickle Cell Disease Care Consortium (Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

DEFINITION:  Prolonged priapism is a painful erection of the penis that lasts more than 2-4 hours and may result eventually in impotence. Most episodes are successfully treated with outpatient aspiration and irrigation with epinephrine (see p. 24). This inpatient care path is for patients who fail to respond to outpatient management.

CONSULTS: Hematology, Urology

MONITORING:

1. Vital signs q 2-4 h.

2. Record I+O, daily weight.

3. Strongly consider continuous pulse ox if receiving parenteral narcotics.

DIAGNOSTICS (if not previously obtained):

1. CBC, diff, platelet count, and reticulocyte count initially and daily until improving. (Compare with patient's baseline data.)

2. Consider type and crossmatch. Consider requesting, if available, minor-antigen-matched, sickle-negative, and leukocyte-depleted RBC.

3. Urinalysis and urine culture.

4. Blood culture if febrile. Consider other cultures (e.g. CSF).

FLUIDS, GENERAL CARE:

1. IV fluids - 10 cc/kg over 1 hr, then IV + PO = 1½ x maintenance

2. Encourage ambulation

3. Incentive spirometry - 10 breaths q 2 hr when awake if on parenteral narcotics

 MEDICATION/TREATMENT:

1. Aspiration and irrigation if not performed as outpatient (see p 24) - may be repeated 3-4 times or daily if needed for recurrence of priapism.

2. Never use ice or cold packs.

3. Morphine sulfate 0.05-0.15 mg/kg/dose IV q 2 hr or 0.05-0.1 mg/kg/hr continuous infusion or via PCA. (For PCA give 1/3- 1/2 of total maximum dose by continuous infusion, with 1/2-2/3 via PCA boluses.) Total morphine dose, continuous infusion plus boluses, above 0.1 mg/kg/hr may occasionally be required but should be used with caution. In most cases, prn analgesic orders are not appropriate. Alternative analgesics including hydromorphone (Dilaudid) 0.015-0.02 mg/kg IV q 3-4 hr may be appropriate in selected cases. Consider use of ketoralac (Toradol) 0.5 mg/kg (30 mg maximum dose) IV q 6-8 hr in addition to opioid analgesia if no contraindication present (i.e. gastritis, ulcer, coagulopathy, dehydration, or renal impairment). Do not use ibuprofen with ketorolac. Repeated doses of meperidine (Demerol) should be avoided because of the risk of seizures.

4. Mild to moderately severe pain - acetaminophen with codeine (1 mg/kg) po q 4 hr.

5. Ibuprofen 10 mg/kg po q 6-8 h if no contraindication present (i.e. ketorolac, gastritis, ulcer, coagulopathy, or renal impairment). Limit more frequent dosing to 72 hr maximum duration.

6. Reassess pain control at least twice daily. Analgesics may be weaned as tolerated by decreasing dose, not by prolonging interval between doses.

7. Cefotaxime or cefuroxime 50 mg/kg IV q 8 h if febrile. Substitute clindamycin 10 mg/kg IV q 6 h for known or suspected cephalosporin allergy. Strongly consider adding vancomycin 10-15 mg/kg IV q 8 hr for severe febrile illness or for proven or suspected CNS infection.

8. If applicable, continue prophylactic penicillin. Prophylactic penicillin should be discontinued while patient is receiving broad-spectrum antibiotics.

9. 0₂ by nasal cannula or face mask if needed to keep pulse ox ³ 92% or ³ patient's baseline value (if >92%). The etiology of a new or increasing supplemental O₂ requirement should be investigated. Avoid excessive or unnecessary 0₂, which may suppress the reticulocyte count and exacerbate anemia.

10. Consider transfusion if no evidence of detumescence within 12 hrs:

a) Partial exchange or erythrocytapheresis to Hb 10 gm/dl and Hb S (patient's RBC) £ 30%.

b) May consider simple transfusion as alternative to partial exchange transfusion if Hb <6-7 gm/dl (do not transfuse acutely to Hb >10 gm/dl, hct >30%).

11. Winter shunt (spongiosum-cavernosum shunt) may be considered if priapism persists for >24 hrs, unresponsive to supportive care, aspiration and irrigation, and transfusions, but is controversial.

12. Observe for severe headache or neurologic signs or symptoms. (Ischemic stroke may occur 1-10 days after onset of priapism, especially following transfusion.)

13. Consider sleep study if obstructive sleep apnea suspected.

14. See other Clinical Care Paths for acute chest syndrome, acute splenic sequestration, aplastic crisis, stroke, if present.

DISCHARGE CRITERIA:

1. Priapism resolving (complete detumescence and resolution of edema after discharge may take 3-4 weeks)

2 Taking adequate oral fluids and able to take po medications (e.g. prophylactic penicillin) if applicable

3. Adequate pain relief on oral analgesics

4. Afebrile ³ 24 hr. with negative cultures ³ 24-48 hr. if applicable.

5. Resolution of any pulmonary symptoms or documentation of adequate oxygenation on room air

6. Consider starting pseudoephedrine 30 mg po hs (<10 years-of-age) or 60 mg po hs (>10 years-of-age) for priapism prophylaxis.

7. Follow up arranged.

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References

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Last modified: November 01, 2002