[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-455235-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-455235-en":130},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","impact-of-elexacaftor-tezacaftor-ivacaftor-on-quality-of-life-in-children-with-cystic-fibrosis","Impact of Elexacaftor-Tezacaftor-Ivacaftor on Quality of Life in Children With Cystic Fibrosis","","CFTR modulators have transformed cystic fibrosis care by acting on the defective protein, yet limited data exist on quality of life (QoL) after Elexacaftor-Tezacaftor-Ivacaftor (ETI), especially in children. A prospective observational study followed children with CF (6–17 years) and their caregivers, measuring QoL with the CF Questionnaire Revised (CFQ-R) at baseline and after 3 months of ETI. Total QoL increased significantly at M3, mainly in physical domains, with minimal emotional/social/school improvement, and weak within-family 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question does the study address about ETI?","Question",{"text":63,"@type":64},"It evaluates how Elexacaftor-Tezacaftor-Ivacaftor affects children’s quality of life, comparing patient and caregiver perspectives.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"How was quality of life measured and when?",{"text":68,"@type":64},"Quality of life was assessed using the CF Questionnaire Revised (CFQ-R) at baseline and again 3 months after starting ETI, for both children and caregivers.",{"name":70,"@type":61,"acceptedAnswer":71},"Which QoL domains improved after ETI, and which did not?",{"text":72,"@type":64},"QoL increased significantly at 3 months, especially in physical domains, while emotional, social, and school domains showed little improvement.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},455235,1790790196,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,109,114,119,122,126],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & 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of Life in Children With Cystic Fibrosis  \nSara Kümmerli1  | Clara Fernandez Elviro1 | Nicolas Regamey2  | Anne Mornand3 | Mohamed Faouzi4 | Isabelle Rochat1  | Sylvain Blanchon1   \n1Pediatric Pulmonology and Cystic Fibrosis Unit, Division of Pediatrics, Department Woman‐Mother‐Child, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland | 2Pediatric Pulmonology and Cystic Fibrosis Unit, Children's Hospital of Central Switzerland, Lucerne,  \nSwitzerland | 3Pediatric Pulmonology and Cystic Fibrosis Unit, University Hospital of Geneva, Geneva, Switzerland | 4Division of Biostatistics, Center for Primary Care and Public Health (Unisanté), University of Lausanne, Lausanne, Switzerland  \nCorrespondence: Sylvain Blanchon (sylvain.blanchon@chuv.ch)  \nReceived: 5 December 2024 | Revised: 10 November 2025 | Accepted: 29 December 2025  \nFunding: Cystic Fibrosis Switzerland; Fondation Respirer  \nKeywords: CFTR modulator | children | cystic fibrosis (CF) | cystic fibrosis quality of life questionnaire (CFQ‐R) | Elexacaftor‐Tezacaftor‐Ivacaftor | quality of life  \nABSTRACT  \nObjectives: CFTR modulators have revolutionized cystic fibrosis (CF) management by targeting the defective protein rather than its consequences. Their impact on quality of life (QoL) have been studied in numerous trials, but few data are available on QoL in patients receiving Elexacaftor‐Tezacaftor‐Ivacaftor (ETI), notably in children given its recent authorization for this age group. We aimed to assess the impact of ETI on children's QoL.  \nMethods: This prospective observational study included children with CF (6−17 years), assessing QoL using the CF Questionnaire Revised (CFQ‐R) before (baseline) and 3 months after (M3) starting ETI treatment for children and their caregivers. Results: We included 23 children (median [range]) age 10.2 [6−17.2] years, 13 (57%) with homozygous F508del genotype. The total QoL score at baseline ([mean (SD)] children: 74.07 [10.86]; caregivers: 73.21 [10.38]) reflected severe disease impact, particularly regarding treatment burden in the children's perspective (63.28 [21.04]) and digestive domains in the caregivers'perspective (digestive symptoms: 66.67 [17.37]; eating disorder: 67.54 [32.14]; weight: 61.40 [33.82]) . At M3, there was a significant increase in reported QoL (p = 0.0001), particularly regarding physical domains. Emotional/social/school domains barely showed improvement. Although QoL mean scores were comparable between children and caregivers' groups, they were poorly correlated within the same family. Homozygous F508del genotype was associated with better QoL improvement at M3 compared to composite heterozygous genotypes (p \u003C 0.001) .  \nConclusion: ETI treatment has a significant impact on children's QoL, particularly in physical health domains. Other QoL domains that are not improved by ETI need to be addressed, in particular, psycho‐social components. Both children's and caregivers' perspectives must be considered for a holistic picture of children's QoL.  \n1 | Introduction  \nCystic fibrosis (CF) is a rare autosomal recessive genetic disease due to bi‐allelic pathogenic variants in the gene Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), including  \nF508del, the most common pathogenic variant. CF is characterized by persistent airway infections, malabsorption, and a large panel of systemic manifestations. Due to chronic symptoms, time‐ consuming daily treatments, repeated hospitalizations, and  \nThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.  \n© 2026 The Author(s) . Pediatric Pulmonology published by Wiley Periodicals LLC.  \nPediatric Pulmonology, 2026; 61:e71461 1 of 8  \n[https://doi.org/10.1002/ppul.71461](https://doi.org/10.1002/ppu","cbCaid8iT7V5OtvH","https://ap.wps.com/l/cbCaid8iT7V5OtvH","pdf",868945,"English","# Abstract\n## Objectives\n## Methods\n## Results\n## Conclusion\n# Introduction\n# Methods\n## Study Design and Subjects","[{\"question\":\"What question does the study address about ETI?\",\"answer\":\"It evaluates how Elexacaftor-Tezacaftor-Ivacaftor affects children’s quality of life, comparing patient and caregiver perspectives.\"},{\"question\":\"How was quality of life measured and when?\",\"answer\":\"Quality of life was assessed using the CF Questionnaire Revised (CFQ-R) at baseline and again 3 months after starting ETI, for both children and caregivers.\"},{\"question\":\"Which QoL domains improved after ETI, and which did not?\",\"answer\":\"QoL increased significantly at 3 months, especially in physical domains, while emotional, social, and school domains showed little improvement.\"}]","Impact of Elexacaftor-Tezacaftor-Ivacaftor on Quality of Life in Children With Cystic Fibrosis | PDF",1790742438]