Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor

Rysgaard, Ulla Kildall; Pedersen, Camilla Lundgren; Jensen, Janne Hastrup; Sørensen, Lotte; Philipsen, L.; Leo-Hansen, Christian; Olesen, Hanne Vebert

doi:10.1016/j.jcf.2022.05.009

Cited by 6 publications

(5 citation statements)

References 21 publications

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“…Exercise-based interventions are beneficial for CF patient in terms of muscle strength and cardiorespiratory endurance [48] . Muscle strength and power improved alongside VO 2 max and maximal workload after one year of either LI or TI [49] . Similar results regarding levels of aerobic workout were demonstrated in patients on ETI [50] .…”

Section: Discussionmentioning

confidence: 95%

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes

Lurquin

Gohy

Hermans

et al. 2023

Journal of Clinical & Translational Endocrinology

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Section: Discussionmentioning

confidence: 95%

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes

Lurquin

Gohy

Hermans

et al. 2023

Journal of Clinical & Translational Endocrinology

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“…A study from C auser et al [ 22 ] showed improvements in peak V ′ O 2 , maximal workload and the anaerobic threshold with 6 weeks of Kaftrio treatment. R ysgaard et al [ 23 ] performed a study involving 91 CF patients 12 years of age and older who were started on Orkambi and Symkevi, and showed significant improvements in peak V ′ O 2 and maximal workload on CPET after 1 year of treatment. A smaller study reviewed the long-term impact of Orkambi on exercise capacity in three CF adult patients and reported improvement in exercise capacity and daytime activity levels following 2 years of treatment.…”

Section: Discussionmentioning

confidence: 99%

“…A recent Danish study involving 91 patients showed statistically significant improvement in peak VO2 and maximal workload on CPET 12 months after initiation of treatment with Orkambi or Symkevi. 23 Other previous studies have reviewed the impact of Orkambi or Ivacaftor on exercise capacity in CF patients, two of these studies did not show any significant change in exercise capacity with these treatments. 24,25,26 The aim of our study was to assess the impact of CFTR modulators on exercise capacity in our cohort of CF adolescents.…”

Section: Introductionmentioning

confidence: 94%

“…A recent study of three CF adolescents on Kaftrio showed improvement in peak oxygen uptake ( V ′ O 2 ) after 6 weeks of Kaftrio treatment [ 22 ]. A recent Danish study involving 91 patients showed statistically significant improvement in peak V ′ O 2 and maximal workload on CPET 12 months after initiation of treatment with Orkambi or Symkevi [ 23 ]. Other previous studies have reviewed the impact of Orkambi or Ivacaftor on exercise capacity in CF patients; two of these studies did not show any significant change in exercise capacity with these treatments [ 24 – 26 ].…”

Section: Introductionmentioning

confidence: 99%

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Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis

Ahmed,

Dayman,

Blyth

et al. 2024

ERJ Open Res

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BackgroundExercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with CF. The aim of the present study was to assess the impact of CFTR modulators on exercise capacity in a cohort of adolescents with CF.MethodsA prospective single-centre cohort study was carried out. Cardiopulmonary exercise testing (CPET) was performed at baseline, prior to starting Symkevi or Kaftrio and between 4 and 8 months after starting treatment.Results19 adolescents with CF had CPET performed prior to and after CFTR modulator treatment, between December 2019 and March 2022. Breathing reserve improved in the whole cohort, with greater improvement in the modulator-naïve patients after starting treatment with Kaftrio. There was no improvement in peak oxygen uptake and anaerobic threshold after 4 to 8 months of treatment with CFTR modulators.ConclusionExercise testing with CPET can be used as an additional tool to monitor response to CFTR modulators. Breathing reserve on CPET may provide a surrogate marker to monitor the improvement in CF lung disease with CFTR modulator treatment.

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“…These data provided on triple combination of CFTR modulators therapy are higher than those achieved on double combination (lumacaftor/ivacaftor or tezacaftor/ivacaftor) in Danish patients with CF followed for the same period of use (VO 2 peak 1.07 mL/min/kg, maximal workload change 14.2W) [ 18 ]. Therefore, ETI might be more effective in improving CPET-derived parameters, but improvement is likely multifactorial, and further investigation in a larger patient cohort is necessary.…”

Section: Discussionmentioning

confidence: 99%

The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study

Stastna,

Hrabovska,

Homolka

et al. 2024

BMC Pulm Med

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Background Physical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO2peak) is an appropriate tool to evaluate the physical activity in these patients. However, there are several other valuable CPET parameters describing exercise tolerance (Wpeak, VO2VT1, VO2VT2, VO2/HRpeak, etc.), and helping to better understand the effect of specific treatment (VE, VT, VD/VT etc.). Limited data showed ambiguous results of this improvement after CFTR modulator treatment. Elexacaftor/tezacaftor/ivacaftor medication improves pulmonary function and quality of life, whereas its effect on CPET has yet to be sufficiently demonstrated. Methods We performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients. Results We observed significant improvement in peak workload, VO2 peak, VO2VT1, VO2VT2, VE/VCO2 slope, VE, VT, RQ, VO2/HR peak and RR peak. The mean change in VO2 peak was 5.7 mL/kg/min, or 15.9% of the reference value (SD ± 16.6; p= 0.014). VO2VT1 improved by 15% of the reference value (SD ± 0.1; p= 0.014), VO2VT2 improved by 0.5 (SD ± 0.4; p= 0.01). There were no differences in other parameters. Conclusion Exercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.

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Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor

Cited by 6 publications

References 21 publications

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes

Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis

The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study

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