How do I treat a child with sickle cell disease presenting with hematuria?

Treatment of Hematuria in Children with Sickle Cell Disease

For children with sickle cell disease presenting with hematuria, initiate conservative management with aggressive hydration and bed rest, while simultaneously ruling out renal papillary necrosis and renal medullary carcinoma through appropriate imaging. 1, 2

Initial Assessment and Risk Stratification

When a child with SCD presents with hematuria, immediately determine:

• Whether the hematuria is painful or painless - painless hematuria is typically benign and responds to conservative therapy, while painful hematuria may indicate renal papillary necrosis or other serious complications 1, 3
• Whether it is macroscopic (gross) or microscopic - gross hematuria requires more aggressive evaluation but usually responds to conservative management 2
• Presence of proteinuria - proteinuria (>2+ by dipstick) with hematuria suggests glomerular disease and chronic sickle cell nephropathy requiring different management 4, 1

Immediate Management: Conservative Therapy

For painless hematuria (the most common presentation):

• Aggressive hydration - this is the cornerstone of treatment, as dehydration promotes sickling in the hyperosmolar renal medulla 1, 2
• Bed rest - reduces metabolic demands and promotes resolution 2, 5
• Forced diuresis - helps clear the renal medulla and prevent further sickling 2, 5

This conservative approach typically results in resolution within 3-7 days in most cases 2, 5

Critical Exclusions Before Conservative Management

Before assuming benign hematuria, you must rule out:

• Renal medullary carcinoma - a rare but aggressive malignancy almost exclusively seen in patients with sickle cell trait or disease; requires urgent imaging 1
• Renal papillary necrosis - look for flank pain, fever, or passage of tissue in urine; imaging shows characteristic findings including ball-on-tee sign, clubbed calyces, and necrotic cavities that fill with contrast 3

Imaging approach: Start with renal ultrasound to assess kidney size, position, and screen for structural lesions 4. If renal papillary necrosis is suspected based on clinical presentation (painful hematuria, passage of tissue), proceed to CT imaging despite radiation concerns, as early identification is critical 3

When Conservative Management Fails

If hematuria persists beyond 7 days or is massive:

Consider a six-drug medical regimen that has shown excellent results with hematuria clearing within 24 hours, designed to alter conditions favorable for sickling in the renal medulla 5. This includes:

• Alkalinization
• Diuresis
• Oxygenation
• Correction of acidosis
• Maintenance of adequate blood pressure
• Prevention of stasis 5

Alternative therapy: Oral urea has been reported to stop hematuria within 3 days in sickle cell trait patients by inhibiting gelation of deoxygenated sickle hemoglobin, though this is based on limited case reports 6

Long-term Management Considerations

If proteinuria is present with hematuria:

• Screen for albuminuria - begin annual screening at age 10 years using first morning urine sample or two consecutive untimed samples 4
• Consider ACE inhibitors or ARBs - for children with confirmed albuminuria, these agents reduce proteinuria and may slow progression of chronic kidney disease 4, 7
• Monitor for hyperkalemia - check serum creatinine and potassium within 1 week of starting therapy 7

Optimize disease-modifying therapy:

• Hydroxyurea - should be offered to every child with HbSS or Sβ0-thalassemia starting at 9 months of age, as it reduces vaso-occlusive complications that contribute to renal damage 4
• Monitor renal function - obtain baseline blood urea nitrogen, serum creatinine, and complete blood count 8

Common Pitfalls to Avoid

• Assuming all hematuria is benign - always rule out renal medullary carcinoma and renal papillary necrosis before attributing hematuria solely to sickling 1, 3
• Inadequate hydration - the hyperosmolar environment of the renal medulla promotes sickling; aggressive hydration is essential 1, 2
• Missing glomerular disease - check for proteinuria, red blood cell casts, and dysmorphic red blood cells which indicate glomerulonephritis requiring different management 4
• Delaying imaging when indicated - while ultrasound is preferred for radiation protection, do not hesitate to proceed to CT if renal papillary necrosis is suspected, as early diagnosis is critical 3