SCD linked to longer wait for kidney transplant in children, youth

Study cites disparities in health outcomes for younger SCD patients with kidney failure

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

Share this article:

Share article via email
An adult stands at the bedside of a child receiving an intravenous infusion in a hospital bed.

Children and young adults with kidney failure due to sickle cell disease (SCD) wait nearly two times longer for a kidney transplant and have higher mortality than those whose kidney failure is not caused by SCD, a study found.

“Children in the U.S. with SCD now have higher survival rates thanks to new interventions and therapies, but this means that mortality from chronic end-organ damage has increased,” Rima Zahr, MD, said in a press release. Zahr is a nephrologist at Le Bonheur Children’s Hospital in Memphis, Tennessee, and the study’s first author.

“The results of our study highlight the disparities in health outcomes and access to transplants for children with kidney failure from SCD,” said Zahr, adding that “Further research is needed to better understand why these disparities exist and how we can address their causes.”

Recommended Reading
A half-full bottle of a liquid prescription medicine bears a label reading

Gene therapy lowers number of red blood cells that take sickle shape

The study, “Kidney failure outcomes in children and young adults with sickle cell disease in the United States Renal Data System,” was published in the journal Pediatric Nephrology.

SCD is an inherited blood disorder that leads to progressive damage in several organs, including the kidneys. Patients often develop kidney disease early in childhood, and some may go on to require dialysis (a procedure to remove waste and excess fluid from the blood when the kidneys stop working properly) or a kidney transplant.

“In adults with kidney failure who initiate dialysis, 1-year mortality is approximately three times higher in individuals with SCD than those without SCD,” the researchers wrote.

However, “in pediatric individuals with SCD kidney failure, the survival outcomes and risk for premature death are not well established; this information is necessary to provide guidance for care teams,” the researchers wrote.

Data culled from the U.S. Renal Data System

Now, Zahr and colleagues drew on the U.S. Renal Data System from 1998 to 2019 to compare data between pediatric patients, ages 21 and younger, who had kidney failure due to SCD, and those whose kidney failure was unrelated to SCD.

The analysis included 97 patients (67% males, median age 19 years) in the SCD group and 96 patients in the non-SCD group who matched for age, sex, race, ethnicity, initial kidney failure, and year of service.

Results showed the mean survival time after a diagnosis of kidney failure was significantly shorter, by about 5.6 years, for children and young adults with SCD than it was for those without the blood disease (8.4 vs. 14 years).

Children and young adults with SCD also waited nearly twice as long for a kidney transplant than those without SCD (12.1 vs. 7.3 years).

Longer wait times for kidney transplant in children with SCD

Overall, these findings highlight that children and young adults with SCD kidney failure “have worse survival” and “experience longer wait times for kidney transplants than matched controls,” the researchers wrote.

“It is vital that the transplant community identify and reduce the barriers to kidney transplantation in children and young adults with SCD kidney failure,” the team wrote, adding that “Equitable access to transplantation may mitigate the decreased survival seen in this study.”

“In the immediate future, nephrology centers can start to work toward improving access to transplantation and establishing sickle cell-specific transplant protocols,” they concluded.