Sickle Cell Information Center Guidelines

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

Urinary Tract Symptoms 

by Antonio Guasch, MD

Urinary tract infections are more common in individuals with sickle cell syndromes. These are caused by the "usual" organisms seen in the general population. Asymptomatic urinary tract infections may be a precipitating event for a pain crisis. Pyelonephritis may precipitate life threatening complications in sickle cell patients. Sickle cell syndromes cause a renal concentrating defect which may cause frequency, nocturia, and contribute to enuresis.

Present Illness. Document the onset and duration of symptoms, presence of fever, chills, frequency, nocturia, dysuria, hematuria, nausea, vomiting, flank or back pain. In females, the history includes last menstrual periods date and flow, recent pregnancy, presence of vaginal discharge, itching, burning, or dyspareunia. Sexual history in males and females should include frequency of intercourse, past sexually transmitted disease, and symptoms in partner.

Review of Symptoms. Note presence of pain crisis, increased symptoms of anemia, edema, weight gain, and general review.

Past Medical History. Document previous infections, urologic or gynecologic surgery or manipulations, present medications, drug allergies, and G6PD status.

- Vital Signs. Temperature, pulse, blood pressure, and respiratory rate.

- General. Amount of distress and hydration status.

- HEENT. Increased scleral icterus or pallor.

- Abdomen. Bowel sounds, change in liver/spleen size, localized tenderness, rebound.

- GU. Costovertebral angle tenderness and suprapubic tenderness, masses.

- Rectal/pelvic. Prostate enlargement, tenderness, urethral discharge, guaiac/vaginal discharge, irritation, cervical tenderness, adnexal masses or tenderness.

- Minimum Evaluation. CBC with differential and reticulocyte count. Clean catch or catheterized urine for urinalysis and culture. Pregnancy test in reproductive age females. Chem profile including electrolytes, BUN, Creat, bilirubim, AST, and LDH, if not done in past one month or with hypertension, glucosuria, or proteinuria .

- Additional Evaluation. Suprapubic bladder aspiration for culture may be required in infants and young children if a clean catch, midstream sample cannot be obtained. GC smear and culture, wet mount and KOH prep for vaginal or urethral discharge, sterile pyuria, or cervical tenderness. For hematuria with negative cultures, see hematuria protocol. Consider intravenous pyelogram, ultrasound, and complete urologic evaluation for persistent or recurrent infections.

- Secondary to Sickle Cell Anemia. Sickle cell syndromes cause a renal concentrating defect which may cause frequency, nocturia, and contribute to enuresis. Diagnostic criteria for this assessment include unchanged symptoms, exclusion of other causes, and absence of hypertension. Onset of hypertension strongly suggests renal insufficiency.

- Urinary Tract Infection. Fever, leukocytosis with left shift, or CVA tenderness strongly suggest infection. A presumptive diagnosis of infection should be made if urinalysis shows: >5 WBCs/HPF or >10 Bacteria/HPF; (+) nitrite; or (+) leukocyte esterase. Clean catch culture of >10⁵ or a catheter or aspiration with >10⁴ is generally diagnostic of infection. Cystitis causes frequency, dysuria, hematuria, and positive urinalysis. Fever, leukocytosis, or CVA tenderness suggests upper tract infection with or without bactiuria.

-Urethritis / Prostatitis Sterile pyuria or prostatic tenderness with dysuria and frequency suggest urethritis and / or prostatitis. "Sterile pyuria" may be associated with acute urethritis, renal tuberculosis, foreign body or tumor of the urinary tract, nonbacterial infections in the genital tract, and a poorly understood entity called interstitial cystitis, which is typically seen in women with chronic dysuria and urgency.

- Vaginitis. Vaginal discharge, normal UA or UA with >5 WBCs or >3 RBCs and <10 bacteria/HPF suggest gynecological etiology. See Vaginitis Chapter.

- Diabetes Mellitus. Increasing frequency, nocturia, weight loss, and (+) glucose on urinalysis support this diagnosis.

- Renal Disease. The onset of urinary symptoms plus edema, weight gain, hypertension, hematuria, proteinuria, or increasing anemia may be secondary to renal insufficiency. A history of proteinuria in the past is often present.

- Renal Stone / Papillary Necrosis. Hematuria with negative cultures can result from these relatively frequent complications (See Hematuria Chapter).

- Secondary to Sickle Cell Anemia. No treatment for frequency or nocturia is necessary except to reinforce the importance of hydration with 3 - 4 liters of fluid a day. For sterile hematuria, see hematuria protocol.

- Enuresis is treated by reassuring parents and the child that this is likely in part related to the disease. Bladder control training during the day, full emptying of the bladder at bedtime, and positive reinforcement will benefit about a third of children. Alarms may allow training and control the problem in most of the rest. Imipramine 1 mg/kg at bedtime is advocated by some for short-term control which may be useful for camp, overnights, and short trips. This drug has many potential side effects and use should be closely supervised.

- Urinary Tract Infections.

-Cystitis or asymptomatic pyuria. Trimethoprim 8 mg/kg/day and sulfamethoxazole 40 mg/kg/day in two divided doses P.O. q 12 hours for 10 days. In G6PD deficient patients, amoxicillin 50 mg/kg/day in four divided doses P.O. q 6 hours for 10 days. Patient should phone in 48 hours for culture results and sensitivity, return in 2 days if symptoms persist, and return in 2 weeks for F/U culture to assure cure.

- Pyelonephritis. Patients with infection signs and fever, or pain crisis, or nausea and vomiting, or CVA tenderness should be admitted for observation parenteral antibiotics, and intravenous hydration. Parenteral regimens include TMP/SMX, third-generation cephalosporins, fluoroquinolones, or aminoglycosides (with or without a beta-lactam antimicrobial). If enterococcal infection is suspected on the basis of a urine Gram stain, ampicillin, 1 g IV q6h, with or without gentamicin, 1 mg/kg IV q8h, is appropriate.. Empiric therapy should be modified based on cultures, sensitivities, and response to therapy. Evaluation for obstruction and perinephric abscess is indicated if symptoms or fever persist despite adequate antibiotics. Therapy should be administered for 14 days.

- Cystitis with Pain Episode. Admission is usually indicated.

- Urethritis. Smear for GC, wet prep, culture for GC and bacteria are used to guide therapy. If negative, treat for non-specific urethritis with tetracycline 500 mg. P.O. q 6 hours for 14 days or doxycycline, 100 mg PO bid for 7 days,. Azithromycin, 1 g PO in a single dose, is an alternative. Erythromycin, 500 mg PO qid for 7 days, should be used in pregnant women with nongonococcal urethritis For trichomonas, give Flagyl 2 grams P.O. STAT, treat partner, and counsel patient not to use alcohol. Persistent sterile pyuria should prompt evaluation for tuberculosis.

- Prostatitis - TMP/SMX, 160 mg/800 mg (DS) PO bid for 14 days, is an effective, economical treatment for acute infections. Quinolones are useful alternatives. Patients with chronic bacterial prostatitis should receive prolonged therapy (for at least 1 month with the quinolones or 3 months with TMP/SMX

- Vaginitis. See vaginitis protocol.

- Diabetes Mellitus. Patients with symptomatic, new onset diabetes should be admitted for evaluation, treatment and education. If asymptomatic and blood glucose <250 mg.%, refer to endocrinologist for rigorous control of the blood sugar.

- Renal Disease. New onset renal failure with creatinine >2 mg.% should be evaluated in the hospital. For gradual increase in creatinine or proteinuria, referral for evaluation by a nephrologist is indicated.

- Renal Stone / Papillary Necrosis. See Hematuria Chapter

Nursing Considerations

Assess factors that place the patient at increased risk for urinary tract infection - feeling of incomplete bladder emptying, sexual activity and history of urinary calculi, or history of known neurogenic bladder dysfunction or incontinence.

 Assess coping strategies, including medically prescribed regimens, medication and hydration.

Advise the patient to maintain an adequate intake of fluids and avoid dehydration .

 Advise patient of importance of adherence to anti-infective medication regimen to reduce likelihood of recurrence or persistence of infection.

Teach women proper perineal hygiene "wipe from urethral meatus to anal area rather than the opposite and to wear loose fitting cotton underwear.

Education about proper hygiene may prevent some infections in females. Recurrent infections require evaluation of urologic abnormalities. Children should be instructed about sexually transmitted diseases and barrier methods as they approach adolescence.

It is very important to discuss the renal concentrating defect with patients, parents, and teachers to assure that there is sufficient intake of water and so the child can be allowed to urinate as frequently as may be required without stigmatization. The same understanding approach and positive reward systems must be stressed in the child with enuresis.

Patients must be instructed about the importance of completing full courses of antibiotics as prescribed and in the importance of obtaining full-up cultures to assure bacteriological cure.

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Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000