The combination treatment of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA; Trikafta®) may provide benefits for patients with cystic fibrosis (CF) after lung transplants, according to research published in the Journal of Cystic Fibrosis.

ELX/TEZ/IVA has been prescribed for the treatment of nonpulmonary symptoms including sinus disease (68%), patient preference (45%), gastrointestinal symptoms (39%), and low body mass index (BMI;19%) in a small subset of patients with CF (94 out of 734 patients; 13%) who had previously received a lung transplant.

Patients in this subset experienced a decrease in the frequency of antibiotic prescriptions, an increase in hemoglobin levels in anemic patients, and a decrease in hemoglobin A1c levels. A1c was reduced by 0.4% (P =.003) in the 44 patients who had data before and after ELX/TEZ/IVA initiation. The 20 patients with A1c >6.5% prior to treatment had an average decrease of 0.8% (P =.004).

No significant change (P =.054) was observed in hemoglobin levels in the full group of patients receiving ELX/TEZ/IVA but those with anemia did have significant increases in levels (0.6 g/dL; P =.007). In the 6 months prior to ELX/TEZ/IVA initiation, patients received 1.9 courses of antibiotics but this decreased to 1.0 courses in the 6 months following initiation.

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No significant change in aspartate aminotransferase and alanine transaminase liver enzymes was observed following ELX/TEZ/IVA initiation. BMI was not significantly different (P =.150) following treatment, either. A possible trend towards an increased BMI (0.6 increase) was observed in patients with BMI <18.5 kg/m2 or who had low BMI as an indication for treatment, however, it did not reach significance levels (P =.051).

The median time after lung transplant until initiation of ELX/TEZ/IVA was 4.6 years (interquartile range, 2.5-8.8). A total of 8 patients (9%) received treatment within 12 months of transplant and 4 within 6 months (4%). The authors attributed the long delay in treatment initiation to the timing of ELX/TEZ/IVA drug approval rather than a statement of the ideal time to start therapy.

“Further study is needed to determine the risks and benefits of [ELX/TEZ/IVA] in the greater CF lung transplant population given the potential for drug interactions, side effects leading to discontinuation of [ELX/TEZ/IVA], and the possible mechanisms for [ELX/TEZ/IVA] to positively impact long-term post-transplant outcomes for CF lung transplant recipients,” the authors said.

For the study, electronic health records searches of 11 clinical sites between October 2019 and September 2020 resulted in a total of 94 out of 734 patients with CF being prescribed ELX/TEZ/IVA following a liver transplant. Four of these patients did not initiate treatment, however. A total of 40 patients (42%) discontinued the use of ELX/TEZ/IVA during the study period. The median time until discontinuation was 56 days.


Ramos KJ, Guimbellot JS, Valapour M, et al. Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients. J Cyst Fibros. Published online April 23, 2022. doi:10.1016/j.jcf.2022.04.009