Spectrum of kidney injury in SCD
Childhood
- Hyperfiltration: GFR often >150 ml/min/1.73m² by age 7
- Defective urinary concentration (isosthenuria): nocturia, enuresis, dehydration risk
- Distal renal tubular acidosis (incomplete)
- Hyperkalaemia tendency
Adolescence / Young Adulthood
- Microalbuminuria (ACR 3-30 mg/mmol) appears in 20-30% by age 18
- Progresses to overt proteinuria over years
Adulthood
- Focal segmental glomerulosclerosis (FSGS) — the dominant glomerular lesion
- Membranoproliferative-like patterns
- Papillary necrosis — flank pain, haematuria, sloughed papillae in urine
- Loss of medullary architecture on imaging
- Progressive CKD; ~12% require dialysis or transplant by age 40 in HbSS
- Acute kidney injury — pre-renal during crises, contrast and rhabdomyolysis-related
Rare
- Renal medullary carcinoma — almost exclusive to sickle trait; aggressive, presents with haematuria + flank mass; refer urology urgently
How to assess kidney function
Gfr Estimation
- Creatinine-based eGFR (CKD-EPI) systematically OVERESTIMATES true GFR in SCD
- Reasons: low muscle mass, enhanced tubular secretion of creatinine
- Use cystatin C-based or combined creatinine-cystatin C eGFR for any decision-making
- Measured GFR (iohexol, EDTA) for transplant work-up or research
Albuminuria
- Annual ACR from age 10
- Persistent ACR ≥3 mg/mmol = treat
Blood Pressure
- Baseline BP in SCD is typically 10-15 mmHg below the general population (low SVR, vasodilatation)
- 'Normal-range' BP (e.g. 130/80) may represent relative hypertension and a cardiovascular and renal risk factor
- Aim for BP <120/70 in proteinuric SCD
Imaging
- Ultrasound annually if CKD established
- MRI for suspected medullary carcinoma (urgent referral)
When To Biopsy
- Nephrotic-range proteinuria of unclear cause
- Rapidly falling eGFR
- Atypical features (active sediment, low complement)
- Always discuss with haematology — biopsy in SCD carries higher bleeding risk; pre-procedure exchange transfusion may be needed
Kidney Vitality is a daily multivitamin developed by a UK Consultant Nephrologist using renal nutrition principles. It contains no added potassium, magnesium, phosphorus or iron, and no herbal blends. See the formulation.
Treatment
Proteinuria / CKD
- ACE inhibitor or ARB — first-line for any microalbuminuria, regardless of BP
- SGLT2 inhibitor — emerging evidence supports use in proteinuric SCD CKD; monitor for euglycaemic ketoacidosis and dehydration during crises
- Statin for cardiovascular risk
- Salt restriction
- Avoid NSAIDs — major precipitant of AKI in SCD
- Avoid iodinated IV contrast unless essential; use IV hydration if unavoidable
Disease-modifying
- Hydroxycarbamide (hydroxyurea) — proven to slow CKD progression in HbSS; check for indication in all patients
- Crizanlizumab — reduces vaso-occlusive crises
- Voxelotor — reduces haemolysis (regulatory status changing in 2024-25)
- L-glutamine — reduces crises
- Chronic exchange transfusion — selected, severe disease
- Gene therapy (exa-cel, lovo-cel) — approved in the UK from 2024 in eligible patients
Hydration
- Maintain euvolaemia
- Encourage 2-3 L/day of water in stable adults (less in established CKD)
- Beware over-diuresis: triggers crisis
Anaemia Of CKD
- Iron studies and erythropoietin levels — but interpret with caution due to baseline haemolysis
- ESA use must be coordinated with haematology — can worsen sickling if Hb rises too high
Dialysis
- Both HD and PD are feasible
- Vascular access can be challenging — preserve veins early
- Be alert to crises in the dialysis chair
Transplant
- Excellent outcomes in selected patients
- Pre-transplant work-up by joint haematology-nephrology team
- Standard immunosuppression
- Recurrence of sickle nephropathy in the graft is recognised; hydroxycarbamide continued where possible
Special situations
Pregnancy
- High-risk; joint maternal-medicine, haematology and nephrology care
- Pre-conception optimisation
- Aspirin from 12 weeks for pre-eclampsia prevention
- Continue ACE/ARB before pregnancy but stop on confirmation
- Watch for AKI in third trimester and post-partum
Paediatric Transition
- Structured transition from paediatric to adult sickle service
- Continue annual ACR and eGFR screening
- Educate young people on NSAID avoidance and hydration
Sickle Trait
- Reassure: low risk of CKD overall
- Counsel on avoidance of extreme exertion in hot, hypoxic environments
- Investigate persistent haematuria promptly — urology referral for MRI to exclude medullary carcinoma
KEY MESSAGE: sickle cell nephropathy is a preventable cause of dialysis. Routine annual screening and early ACE/ARB plus hydroxycarbamide change long-term outcomes.






