How are patients with CKD managed?
• Progression of CKD:
• Although angiotensin-converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARB) are commonly used to reduce proteinuria in addition to slowing CKD progression and lowering blood pressures in non–sickle cell nephropathies, significant antiproteinuric benefits have not been proven in patients with SCD. Nonetheless, the most updated Cochrane database review (in 2015) revealed a potential for reduction in microalbuminuria and proteinuria with the use of captopril among patients with SCD compared with those without the disease. Confirmatory studies using other ACEi and ARB are lacking.
• Hydroxyurea may have a role in reducing proteinuria and hyperfiltration. It has been suggested that the increase in fetal Hb with the use of hydroxyurea reduces sickling, which would lead to improved tissue oxygenation, decreased cardiac output, and reduced kidney blood flow, and therefore decreased hyperfiltration, glomerular injury, and proteinuria.
• End-stage kidney disease (ESKD): All forms of kidney replacement may be beneficial to patients with ESKD from sickle cell anemia.
• Dialysis: Both hemo and peritoneal dialytic therapies may be offered to patients reaching ESKD if there is no modality-specific contraindication. Both modalities confer their own theoretical advantages.
• Hemodialysis: Readily available vascular access may be used for the urgent or emergent need for standard and exchange blood transfusions.
• Peritoneal dialysis: The slow rate of ultrafiltration minimizes any acute rise in hematocrit, and therefore leads to a lower risk of vasoocclusive crisis.
• Kidney transplantation:
• Kidney transplantation may be hindered by high levels of panel reactive antibody due to numerous previous blood transfusions. There is also a higher infection risk due to autosplenectomy. Sickle cell transplant patients have higher risks of avascular necrosis with chronic steroid use and precipitating sickle cell crises due to anemia correction following a successful transplant.
• Kidney transplant may be also complicated by allograft venous thrombosis, deep vein thrombosis, and vasoocclusive crises.
