High one year mortality in adults with sickle cell disease and end-stage renal disease

Abstract

Adequate pre-dialysis care reduces mortality among end-stage renal disease (ESRD) patients. We tested the hypothesis that individuals with ESRD due to sickle cell disease (SCD-ESRD) receiving pre-ESRD care have lower mortality compared to individuals without pre-ESRD care. We examined the association between mortality and pre-ESRD care in incident SCD-ESRD patients who started haemodialysis between 1 June, 2005 and 31 May, 2009 using data provided by the Centers for Medicare and Medicaid Services (CMS). SCD-ESRD was reported for 410 (0·1%) of 442 017 patients. One year after starting dialysis, 108 (26·3%) patients with incident ESRD attributed to SCD died; the hazard ratio (HR) for mortality among patients with SCD-ESRD compared to those without SCD as the primary cause of renal failure was 2·80 (95% confidence interval [CI] 2·31-3·38). Patients with SCD-ESRD receiving pre-dialysis nephrology care had a lower death rate than those with SCD-ESRD who did not receive pre-dialysis nephrology care (HR = 0·67, 95% CI 0·45-0·99). The one-year mortality rate following an ESRD diagnosis was almost three times higher in individuals with SCD when compared to those without SCD but with ESRD and could be attenuated by pre-dialysis nephrology care.

Fig 1

Kaplan–Meier mortality survival curves (Up to 1,553 d (4·25 years) of survival) for incident end-stage renal disease (ESRD) haemodialysis patients with and without sickle cell disease as the primary cause of renal failure, (Log rank test: P < 0·0001) June 2005 to May 2009, N = 442 015. Incident ESRD patients without sickle cell disease (SCD) as the primary cause of renal failure, as recorded on Form CMS-2728. Incident ESRD patients with SCD as the primary cause of renal failure.