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

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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Author

Phuong-Thu Pham, MD, FASN Clinical Professor of Medicine, Division of Nephrology, Department of Medicine, Director of Outpatient Service, Kidney Transplant Program, University of California, Los Angeles, David Geffen School of Medicine

Phuong-Thu Pham, MD, FASN is a member of the following medical societies: American Society of Nephrology, International Society of Nephrology, National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Edgar V Lerma, MD, FACP, FASN, FAHA, FASH, FNLA, FNKF Clinical Professor of Medicine, Section of Nephrology, Department of Medicine, University of Illinois at Chicago College of Medicine; Research Director, Internal Medicine Training Program, Advocate Christ Medical Center; Consulting Staff, Associates in Nephrology, SC

Edgar V Lerma, MD, FACP, FASN, FAHA, FASH, FNLA, FNKF is a member of the following medical societies: American Heart Association, American Medical Association, American Society of Hypertension, American Society of Nephrology, Chicago Medical Society, Illinois State Medical Society, National Kidney Foundation, Society of General Internal Medicine

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Astra Zeneca<br/>Author for: UpToDate, ACP Smart Medicine, Elsevier, McGraw-Hill, Wolters Kluwer.

Chief Editor

Vecihi Batuman, MD, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Interim Chair, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Acknowledgements

James W Lohr, MD Professor, Department of Internal Medicine, Division of Nephrology, Fellowship Program Director, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

James W Lohr, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, and Central Society for Clinical Research

Disclosure: Genzyme Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Christie P Thomas, MBBS, FRCP, FASN, FAHA Professor, Department of Internal Medicine, Division of Nephrology; Medical Director, Kidney and Kidney/Pancreas Transplant Program, University of Iowa Hospitals and Clinics

Christie P Thomas, MBBS, FRCP, FASN, FAHA is a member of the following medical societies: American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Nephrology, American Society of Transplantation, American Thoracic Society, International Society of Nephrology, and Royal College of Physicians

Disclosure: Genzyme Grant/research funds Other

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

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