Longitudinal changes in bone mineral density after initiation of elexacaftor-tezacaftor-ivacaftor in youth and adults with cystic fibrosis: PROMISE-ENDO

Journal of Cystic Fibrosis 2026 Open access

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Authors: Meghan K. Shirley, Babette S. Zemel, R. Gallop, Rachel Walega, Scott H. Donaldson, C. Frederick, Steven D. Freedman, Daniel Gelfond, Lucas R. Hoffman, Michael R. Narkewicz, Steven M. Rowe, Scott D. Sagel
+38 more Sarah Jane Schwarzenberg, George Marty Solomon, Christine L. Chan, Andrea Kelly, Joanne Billings, Holly Carveth, Elliott C. Dasenbrook, Raksha Jain, Claire L. Keating, Kevin Kirchner, Katie Larson Ode, Kelvin D. MacDonald, Susanna A. McColley, Ryan C. Perkins, Clement L. Ren, Christopher Richards, Iman Sami, Alvin Singh, Heather Thomas, Karen Z. Voter, Mary Bailey, Jenna Bucher, Erin Canning, Joy Dangerfield, Erin Donnelly, Margot Hardcastle, Barbara A. Johnson, Olivia Killilea, Donna Lach, Jean Malpass, Maria Mcleod, James Melson, Larissa Rugg, M. Teresi, Jane B. Vroom, David Weaver, Danielle Wolfe, Maria Ycaza
DOI: 10.1016/j.jcf.2026.01.006
Cited by: 0
References: 40

Abstract

BACKGROUND: ) in the 24-30 months (mos) following ETI initiation. METHODS: Participants had CF, ≥1 F508del mutation, and were aged ≥12 years (y). Dual-energy X-ray absorptiometry (DXA) scans of the WB, LS, TH, and FN were collected before and following 12-18 mos and 24-30 mos of ETI therapy. Changes in aBMD Z-scores (aBMDZ) were examined with longitudinal mixed effects models. RESULTS: Baseline aBMDZ was below-average at all skeletal sites in youth and adults (aBMDZ -0.11). CONCLUSIONS: Youth aBMDZ was lower at multiple skeletal sites 12-18 mos after ETI initiation, and these changes persisted at 24-30 mos. Adult aBMDZ generally remained unchanged.

Fields of Study & Subjects

Cystic Fibrosis Research Advances Pharmacological Effects and Toxicity Studies Bone health and osteoporosis research

Publication details

Journal / source
Journal of Cystic Fibrosis
Volume
25
Issue
3
Pages
457–465
Publication date
30 January 2026
Language
EN