Transplant tied to longer survival in CF patients with kidney failure
Study: Organs also remained functional longer in people with the disease
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Among people with cystic fibrosis (CF) who develop kidney failure, getting a kidney transplant may extend survival by nearly two decades compared with other types of care, a new study found.
The transplanted kidneys also showed favorable long-term outcomes in people with CF, remaining functional for about 7.2 years longer in those patients than in people from the general population who also developed kidney failure.
Because kidney failure is a common complication of CF, owing to long-term medication use and disease-related diabetes, the findings “demonstrate that positive outcomes are possible with kidney transplant in selected patients with CF and ESRD [end-stage renal disease] and suggest that the diagnosis of CF should not in itself be considered a contraindication to kidney transplant in appropriate patients,” researchers wrote.
The study, “Outcomes of Kidney Transplantation in Cystic Fibrosis,” was published in Transplantation Proceedings.
CF patients more likely to undergo kidney transplantation
CF is caused by genetic mutations that result in the loss or dysfunction of the CFTR protein, which normally helps cells regulate the flow of salt and water. A deficiency of this protein leads to the accumulation of thick, sticky mucus in various organs, ultimately driving the disease’s symptoms.
CF primarily affects the lungs. However, as people with CF live longer, other health challenges are becoming more common. Kidney disease, for example, may develop due to decades of antibiotic use, CF-related diabetes, and the use of medications to prevent organ rejection.
In advanced cases of kidney disease, patients may require dialysis or a kidney transplant. However, CF may sometimes be seen as a contraindication for receiving a transplant because it is linked to a shorter life expectancy and an increased risk of infections.
Understanding the outcomes of people with CF who have received a kidney transplant is therefore essential, and may support the referral for a transplant in select patients with kidney failure.
To address this, a team of researchers in the U.S. analyzed data from a database covering virtually all people in the country with chronic kidney failure requiring dialysis or a kidney transplant. From 2014 and 2018, more than 950,000 individuals were included in the database, 379 of whom had CF.
CF patients were more likely to undergo kidney transplantation than those without CF (50.6% vs. 26.1%) and were younger at the start of kidney failure care (36.4 years vs. 43.5 years).
These data provide a baseline for assessing the future impact of newer therapies on those with CF and [kidney failure] who may be eligible for and undergo kidney transplantation.
Compared with the general population, kidney failure in CF was more frequently linked to complications from other organ transplants (0.8% vs. 21.9%) — particularly lung transplants — as well as kidney injury caused by medications or toxins (0.7% vs. 12.5%).
In contrast, diabetes was less often the primary cause of kidney failure in CF than in the general population (14.6% vs. 25.5%), even though diabetes was more frequent among those with CF.
CF patients were also more likely to receive organs from living donors and spend less time on dialysis before receiving a transplant (1.95 year vs. 3.29 years). They were also less likely to experience transplant failure — in fact, data showed that transplanted kidneys survived for about 20.5 years among people with CF compared with 13.3 years for the general population.
Overall survival from the start of kidney failure care was shorter in CF patients who received a transplant than in those without CF (21.1 vs. 38.1 years). However, among CF patients specifically, those who underwent kidney transplantation lived markedly longer than those who did not (21.1 vs. 3.9 years).
The researchers noted that the study period predates the 2019 approval of Trikafta (elexacaftor/tezacaftor/ivacaftor), a highly effective CFTR modulator. As such, future studies will be needed to determine how this therapy may affect the risk of kidney disease and transplant outcomes in CF.
“These data provide a baseline for assessing the future impact of newer therapies on those with CF and [kidney failure] who may be eligible for and undergo kidney transplantation,” the researchers concluded.
