Racial Disparities Widen in Modern Cystic Fibrosis Care

TOPLINE:

The introduction of highly effective modulator (elexacaftor/tezacaftor/ivacaftor) therapy has improved the disease trajectory of cystic fibrosis (CF). However, its benefits have been less pronounced for lung transplant candidates of Black or Hispanic ethnicity than for those of White ethnicity.

METHODOLOGY:

• Researchers evaluated the differences in lung transplantation outcomes by race/ethnicity in patients with CF using data from the United States Scientific Registry of Transplant Recipients (May 1, 2005, through February 2, 2023).
• Among 43,476 lung transplant candidates who joined the waitlist during the study period, 4321 were diagnosed with CF. They were grouped as per the year of listing or transplant:
  • Pre–highly effective modulator therapy era (on or before January 30, 2012)
  • Ivacaftor era (on or after January 31, 2012)
  • Elexacaftor/tezacaftor/ivacaftor era (on or after October 21, 2019)
• Percentage of patients listed, delisted/died, or transplanted were compared on the basis of race/ethnicity and era.

TAKEAWAY:

• Among the candidates listed for lung transplant, those with an indication of CF decreased from 13.3% in the pre–highly effective modulator therapy era to 11.2% in the ivacaftor era to 2.1% in the elexacaftor/tezacaftor/ivacaftor era (P < .001).
• Among lung transplant candidates across the eras, the proportion of White candidates decreased (82.0% vs 78.6% vs 71.0%), while proportions of Black (9.2% vs 10.0% vs 10.3%) and Hispanic (6.4% vs 8.6% vs 13.6%) candidates increased (P < .001).
• Similarly, the percentage of lung transplant recipients with CF of White race also decreased significantly, while that of Black or Hispanic race increased (P < .001).
• Across all racial groups, patients with CF removed from the waitlist because of deterioration or death consistently decreased from the pre–highly effective modulator therapy era to the ivacaftor era to the elexacaftor/tezacaftor/ivacaftor era.

IN PRACTICE:

“In order to reduce disparities in CF outcomes, we therefore need not only to better understand and address historic causes, including social determinants of health, but also to expand CF treatments for rare and novel mutations,” the authors wrote.

SOURCE:

This study was led by Jessica M. Ruck, Johns Hopkins University School of Medicine, Baltimore, and was published online on January 16, 2025, in Journal of Cystic Fibrosis.

LIMITATIONS:

This study did not account for concurrent events like COVID-19 that could have influenced racial disparities in lung transplantation. The registry data used could have certain patients with their diagnosis miscoded. This study also did not examine the timing of initiation, termination, dosing, and adherence of highly effective modulator therapy. Additionally, it did not account for social factors.

DISCLOSURES:

This study was supported by grants from the National Institute on Aging and the National Institute of Diabetes and Digestive and Kidney Diseases. One of the authors reported receiving consulting fees and speaker honoraria from several organizations. Others reported having no conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.