Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis

Caley, L.R.; Jarosz-Griffiths, Heledd; Smith, Graham; Gale, Lone; Barrett, J.; Kinsey, L.; Davey, Valerie A.; Nash, Marc; Jones, Andrew M.; Whitehouse, J.L.; Shimmin, D.; Floto, R. Andres; White, H; Peckham, Daniel

doi:10.1016/j.jcf.2023.06.010

Cited by 13 publications

(6 citation statements)

References 22 publications

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“…Thus, baseline BMI category is the main determinant of the observed increases in the analyzed adipose tissues. Recent evidence showed that weight gain with ETI may not simply be attributed to increased energy intake 24 . Also, study participants did not routinely receive professional diet advice upon ETI initiation at our center.…”

Section: Discussionmentioning

confidence: 99%

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Westhölter,

Haubold,

Welsner

et al. 2024

Sci Rep

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A poor nutritional status is associated with worse pulmonary function and survival in people with cystic fibrosis (pwCF). CF transmembrane conductance regulator modulators can improve pulmonary function and body weight, but more data is needed to evaluate its effects on body composition. In this retrospective study, a pre-trained deep-learning network was used to perform a fully automated body composition analysis on chest CTs from 66 adult pwCF before and after receiving elexacaftor/tezacaftor/ivacaftor (ETI) therapy. Muscle and adipose tissues were quantified and divided by bone volume to obtain body size-adjusted ratios. After receiving ETI therapy, marked increases were observed in all adipose tissue ratios among pwCF, including the total adipose tissue ratio (+ 46.21%, p < 0.001). In contrast, only small, but statistically significant increases of the muscle ratio were measured in the overall study population (+ 1.63%, p = 0.008). Study participants who were initially categorized as underweight experienced more pronounced effects on total adipose tissue ratio (p = 0.002), while gains in muscle ratio were equally distributed across BMI categories (p = 0.832). Our findings suggest that ETI therapy primarily affects adipose tissues, not muscle tissue, in adults with CF. These effects are primarily observed among pwCF who were initially underweight. Our findings may have implications for the future nutritional management of pwCF.

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Section: Discussionmentioning

confidence: 99%

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Westhölter,

Haubold,

Welsner

et al. 2024

Sci Rep

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“…While it is known that BMI and dietary intake increase with ETI therapy [ 1 ], it has not yet been evaluated for malnutrition risk before and after modulator therapies. The current study described the risk of malnutrition over time and its relationship to other variables in patients with and without modulator therapies.…”

Section: Discussionmentioning

confidence: 99%

“…The CFTR protein is also found in the pancreas. This can lead to pancreatic insufficiency, inflammation, dysmotility, and malabsorption [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Effect of Modulator Therapies on Nutritional Risk Index in Adults with Cystic Fibrosis: A Prospective Cohort Study

Yalçın,

Akman,

Karcıoğlu

et al. 2024

Nutrients

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Background: Modulator therapies improve weight and body mass index (BMI) in cystic fibrosis (CF) patients. We aimed to compare the nutritional risk index (NRI) in adult CF patients receiving modulator (MT) or only non-modulator (conventional) therapies (non-MT). Methods: A single-center prospective cohort study was conducted between June and December 2023. The NRI based on weight gain and albumin was calculated at beginning and end of a 12-week period in both groups. This design was pragmatic, since it was based on individual patient access to MT for 12 weeks. Results: In total, 107 patients were included [mean (SD) age: 23.85 (4.98) years, 54.7% male, 46.7% MT]. In the MT group, mean (SD) weight (kg) and albumin (g/dL) increased significantly [changes: +3.09 (2.74) and +0.17 (0.37); p < 0.001]. In the non-MT group, weight and albumin decreased significantly [changes: −0.99 (1.73) and −0.12 (0.30); p < 0.001]. Compared to the MT group, baseline mean (SD) NRI in the non-MT group was significantly higher [100.65 (11.80) vs. 104.10 (10.10); p = 0.044]. At the end of the 12 weeks, mean (SD) NRI in the MT group was higher than in the non-MT group [104.18 (10.40) vs. 102.58 (12.39); p = 0.145]. In the MT group, the NRI category improved in 22 (44%), and worsened in 3 (6%) patients (p < 0.001). In the non-MT group, the NRI category improved in 2 (3.5%), and worsened in 10 (17.5%) patients (p < 0.001). Conclusions: This is the first study reporting on a positive effect of MT on NRIs, based on weight gain and albumin. Personalized nutrition and routine follow-up of adults with CF based on NRI is recommended prior to MT initiation.

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“…Use of ETI has also been associated with significant increases in weight and BMI compared to placebo 8,29,30 . In a phase 3, randomized, double‐blind, placebo‐controlled trial designed to test the safety and efficacy of ETI in subjects 12 years and older with F508Del CFTR mutations, BMI was higher at 24‐week follow‐up as compared to baseline with a mean treatment difference of 1.04 in the treatment group 8 .…”

Section: Nutritional Status For Cf In the Modern Eramentioning

confidence: 99%

Nutritional status in the era of highly effective CFTR modulators

Bass,

Alvarez

2024

Pediatric Pulmonology

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Advances in cystic fibrosis (CF) diagnostics and therapeutics have led to improved health and longevity, including increased body weight and decreased malnutrition in people with CF. Highly effective CFTR modulator therapies (HEMT) are associated with increased weight through a variety of mechanisms, accelerating trends of overweight and obesity in the CF population. Higher body mass index (BMI) is associated with improved pulmonary function in CF, yet the incremental improvement at overweight and obese BMIs is not clear. Improvements in pulmonary health with increasing BMI are largely driven by increases in fat‐free mass (FFM), and impact of HEMT on FFM is uncertain. While trends toward higher weight and BMI are generally seen as favorable in CF, the increased prevalence of overweight and obesity has raised concern for potential risk of traditional age‐ and obesity‐related comorbidities. Such comorbidities, including impaired glucose tolerance, hypertension, cardiac disease, hyperlipidemia, fatty liver, colon cancer, and obstructive sleep apnea, may occur on top of pre‐existing CF‐related comorbidities. CF nutrition recommendations are evolving in the post‐modulator era to more individualized approaches, in contrast to prior blanket high‐fat, high‐calorie prescriptions for all. Ultimately, it will be essential to redefine goals for optimal weight and nutritional status to allow for holistic health and aging in people with CF.

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Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis

Cited by 13 publications

References 22 publications

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Effect of Modulator Therapies on Nutritional Risk Index in Adults with Cystic Fibrosis: A Prospective Cohort Study

Nutritional status in the era of highly effective CFTR modulators

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