Patients with sickle cell disease who develop end‐stage kidney disease continue to experience poor survival — A 19‐year United States Renal Data System study

Abstract

Patients with sickle cell disease (SCD) experience increased morbidity and early mortality, due in part to organ damage, including end-stage kidney disease (ESKD). 1 Decline in kidney function is progressive with initial hyperfiltration, followed by declines in glomerular filtration rate (GFR) and eventual ESKD in adults. Individuals with SCD experience a more rapid decline in GFR than the general African American population and this rapid decline is associated with increased mortality. 2,3 Prior studies suggest the one-year mortality rate after initiating dialysis is higher in patients with SCD but early care by a nephrologist may decrease mortality. 4 Additionally, individuals with ESKD due to SCD (SCD-ESKD) are less likely to receive an arteriovenous fistula (AVF) when initiating haemodialysis (HD) 5,6 despite studies demonstrating improved survival when dialyzed with an AVF or graft. 7,8 Finally, referral for transplantation and transplantation rates are lower among individuals with SCD-ESKD despite the improved survival benefit of kidney transplantation. [9][10][11] It is important to characterize the mortality of and disparities experienced by patients with SCD-ESKD. These data can highlight the need to improve clinical care, monitor kidney disease progression, and develop therapies to prevent SCD-ESKD. Despite improvement in care over the last two decades, the prognosis of SCD-ESKD remains uncertain. This study aimed to characterize the outcomes of individuals with SCD-ESKD from 1998 toi 2017 enrolled in the United States Renal Data System (USRDS) registry. We hypothesized that survival of patients with SCD-ESKD has improved over the course of the last two decades.We performed an institutional review board (IRB)approved retrospective analysis using the USRDS database from 1998 to 2017. Data including age, sex, race, ethnicity, primary diagnosis, outcome dates, first ESKD treatment modality, and comorbid conditions (atherosclerotic heart disease, congestive heart failure, cerebrovascular disease, diabetes mellitus, peripheral vascular disease and hypertension) were abstracted from the patient profile using Centers for Medicare and Medicaid Services (CMS) Medical Evidence Form 2278.Age was summarized by mean (standard deviation), median (interquartile range), and by number and percentage

Blood pressure thresholds for the diagnosis of hypertensive disorders of pregnancy in sickle cell disease

“…In SCD, end-stage kidney disease is associated with poorer overall survival and disparities in access to kidney transplant. 26,27 Standardizing BP thresholds to define pre-eclampsia in SCD has diagnostic, treatment and prognostic significance.…”

Sickle Cell Disease and CKD: An Update

“…However, the progression of CKD still appears to be more rapid in these non-SCA SCD genotypes compared to non-SCD controls [35]. Both the rate of eGFR decline and the prevalence of more advanced stages of CKD are among the most consistent predictors for increased mortality in individuals with SCD [27, 36–38].…”

“…Several studies demonstrate that renal replacement therapy is likely to be initiated in patients with SCD with hemodialysis using a central catheter compared to an arteriovenous fistula (AVF) or peritoneal dialysis [38, 126, 131]. Studies in non-SCD patients demonstrate improved survival when dialyzed with an AVF or graft [133, 134].…”

“…This finding was again demonstrated in an analysis examining the USRDS over a 19-year period. Patients with SCD were less likely to receive a kidney transplant and had lower odds of receiving a pre-emptive kidney transplant as compared to being started on dialysis [38]. Table 1 lists studies assessing transplant outcomes in patients with SCD-associated kidney failure in the USA.…”

Machine Learning Predicts Acute Kidney Injury in Hospitalized Patients with Sickle Cell Disease

“…Early AKI detection is vital to improving patient outcomes, specifically in individuals with SCD who have poor outcomes associated with ESRD diagnosis [10, 11]. As AKI defined by serum creatinine is often a delayed manifestation of kidney pathology, it is important to identify approaches to prevent the development of AKI prior to the rise in serum creatinine.…”