Improving Pancreatic Function With Elexacaftor/Tezacaftor/Ivacaftor.
Madison Pranske1, Alizay Paracha2, Carson Loncarich3
1Oklahoma State University College of Osteopathic Medicine, Tulsa, Oklahoma, USA.
Pediatric Pulmonology
|April 8, 2026
Summary
Elexacaftor/tezacaftor/ivacaftor (ETI) treatment shows potential for reversing pancreatic insufficiency in cystic fibrosis (CF) patients. Early ETI initiation and monitoring fecal elastase (FE-1) are crucial for improving pancreatic function.
Area of Science:
- Medical Research
- Pulmonology
- Gastroenterology
Background:
- Cystic Fibrosis (CF) leads to pancreatic cell dysfunction, necessitating enzyme replacement therapy.
- Elexacaftor/tezacaftor/ivacaftor (ETI) is a novel treatment for CF.
- The impact of ETI on pancreatic function in CF patients requires investigation.
Purpose of the Study:
- To evaluate the effect of ETI treatment on pancreatic function in individuals with CF.
- To determine if ETI can improve pancreatic enzyme levels (fecal elastase - FE-1).
Main Methods:
- Retrospective analysis of CF Foundation Patient Registry data from the Tulsa CF Center.
- Inclusion of individuals aged 0-21 years treated with ETI.
- Collection of demographic data, FE-1 values, BMI percentiles, and CFTR mutation information.
- Statistical analysis including descriptive statistics and Spearman Correlation.
Main Results:
- Most patients on ETI (n=49) had severe pancreatic insufficiency post-treatment (79.6%).
- A small percentage (10.2%) achieved pancreatic sufficiency post-ETI.
- Early ETI initiation and homozygous F508del mutation were associated with improved pancreatic function.
- Among 24 patients with pre- and post-ETI FE-1 values, 20.8% showed conversion to moderate or sufficient pancreatic status.
Conclusions:
- ETI therapy may potentially reverse pancreatic insufficiency in CF patients.
- Early initiation of ETI therapy is important for pancreatic function.
- Standardized monitoring of FE-1 values is recommended to track treatment efficacy.
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