How is end-stage renal disease (ESRD) treated in patients with sickle cell nephropathy (SCN)?

Updated: Sep 12, 2019
  • Author: Phuong-Thu Pham, MD, FASN; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

Patients with sickle cell disease (SCD) comprised only 410 of the 442,017 patients with end-stage renal disease (ESRD) who started hemodialysis from June 1, 2005 to May 31, 2009 in the US Centers for Medicare and Medicaid systems. [36] The relatively small size of the SCD-ESRD population has limited the development of optimal management strategies. Hemodialysis is reportedly the leading form of renal replacement therapy for SCD-ESRD patients, [37] but therapeutic options for these patients also include peritoneal dialysis and kidney transplantation.

Both hemodialysis and peritoneal dialysis may confer their own theoretical advantages. Hemodialysis may be used for urgent or emergent need for standard and exchange blood transfusions. In contrast, peritoneal dialysis and its inherent slow rate of ultrafiltration may minimize any acute rise in hematocrit and thus lower the risk of vaso-occlusive crisis. [38]

Of interest, only 6.8% of SCD patients began dialysis with a functioning arteriovenous fistula, despite similar rates of predialysis nephrology care. Mortality in SCD patients is approximately 26% during the first year of therapy for ESRD, which is nearly threefold higher than in ESRD patients without SCD. However, SCD patients who received pre-dialysis nephrology care had a lower death rate than those who did not receive such care. [36]

Kidney transplantation may offer survival advantage over remaining on dialysis for appropriately selected patients with ESRD due to SCN. As in the general population, allograft survival for patients with SCN is greater in those with a living donor than in those with a deceased donor. In the current era of transplantation, desensitization protocols may allow highly sensitized patients (related to multiple blood transfusions) to undergo a successful kidney transplant; for discussion of this topic, see Renal Transplantation.

Although survival of transplant recipients with SCD is inferior to that of matched African-American recipients without the disease, survival of SCD patients is comparable with that of matched diabetic patients. One-year graft survival exceeds 60% to 80%. [39] Complications specific to the SCD population include higher infection risk due to autosplenectomy and precipitation of sickle cell crises with anemia correction following a successful transplant. Kidney transplant may be also complicated by allograft venous thrombosis, deep vein thrombosis, and vaso-occlusive crises. [40, 41, 42] Recurrent disease in the allograft 3.5 years post-transplant has been reported. [40, 43] However, SCN is not a contraindication for transplantation.

Suggested maneuvers to decrease the incidence of post-transplant complications in these patients include the following [41, 42] :

  • Preoperative blood transfusions to decrease hemoglobin S levels
  • Preoperative oxygen supplementation with 40% oxygen
  • Pretransplantation warming of the kidney allograft using 37º C saline
  • Intraoperative and postoperative dopamine infusion at 4 μg/kg/min

Fluid intake and output should be closely monitored. Compared with the general population, these patients have an increased risk of intravascular volume depletion, especially secondary to volume losses from diarrhea and vomiting, thus increasing the risk of an acute sickle cell crisis. Intravenous fluid and partial exchange transfusions may be considered in patients who develop sickle crises. However, management of the kidney transplant candidate and recipient with SCD should be individualized.


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