Academic Journal
People with cystic fibrosis' experience of symptom burden while taking elexacaftor/tezacaftor/ivacaftor.
| Τίτλος: | People with cystic fibrosis' experience of symptom burden while taking elexacaftor/tezacaftor/ivacaftor. |
|---|---|
| Συγγραφείς: | Muther EF; University of Colorado Anschutz Medical Campus, Aurora, CO, USA. Electronic address: Emily.muther@childrenscolorado.org., Gibson SL; Boston Children's Hospital, Boston, MA, USA., Ford C; Johns Hopkins University, Baltimore, MD, USA., Duncan C; Oklahoma State University, Stillwater, OK, USA., Riekert KA; Johns Hopkins University, Baltimore, MD, USA., Lyons E; University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Hoppe JE; University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Prickett M; Northwestern University School of Medicine, Chicago, IL, USA., Isenburg MV; Community Partner, Durham, NC, USA., Everhart RS; Virginia Commonwealth University, Richmond, VA, USA. |
| Πηγή: | Respiratory medicine [Respir Med] 2026 Aug; Vol. 259, pp. 108908. Date of Electronic Publication: 2026 May 27. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE |
| Imprint Name(s): | Publication: 2003- : Oxford : Elsevier Original Publication: London : Baillière Tindall, in association with the British Thoracic Society, [c1989- |
| Ιατρικοί όροι (MeSH): | Benzodioxoles*/therapeutic use , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/physiopathology , Cystic Fibrosis*/psychology , Indoles*/therapeutic use , Pyrazoles*/therapeutic use , Pyridines*/therapeutic use , Pyrroles*/therapeutic use , Quinolones*/therapeutic use , Aminophenols*/adverse effects , Aminophenols*/therapeutic use, Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Young Adult ; Drug Combinations ; Surveys and Questionnaires ; Symptom Burden ; Quinolines |
| Περίληψη: | Background: Despite improvements seen with elexacaftor/tezacaftor/ivacaftor (ETI), many people with CF (PWCF) still experience symptom burden. This study aimed to evaluate current perceived symptom burden and contrast it with PWCF's recollection of symptoms before starting ETI. Methods: Participants were 367 PWCF, ages 7-74 years (M age = 26.2), in the "Your ETI" (yETI) study. PWCF or caregivers of children (7-12 years) completed the Change in Perceptions of Health on ETI (CHETI) survey which assessed how often they were bothered by symptoms (respiratory, gastrointestinal/nutrition, sinus/pain, sleep/fatigue, and mental health/cognitive symptoms) before starting ETI and in the past month. A change score was calculated and categorized as less bothered, stable, or more bothered. Age differences were investigated. Results: Participants were on ETI for an average of 3.9 years. A notable percentage reported being less bothered by respiratory symptoms including cough (72%) and chest tightness (66%), and gastrointestinal symptoms including stomach pain/discomfort (48%), constipation, gas, bloating (46%), and difficulty gaining weight (51%). A smaller proportion indicated more bother related to weight gain (26%), increased appetite (23%), sleep difficulties (17%), and mental health/cognitive symptoms (14-22%). Participants reported the most current bother from constipation/gas/bloating (48%), anxiety (44%), sleep difficulties (44%), and anger/irritability (43%). Adults, notably those >36 years old, typically reported these symptoms more burdensome. Conclusions: While PWCF were bothered less by symptoms now compared to before starting ETI, some symptoms still caused a high degree of bother, particularly among 'older' adults with CF. Findings underscore the need for ongoing strategies to monitor and manage non-respiratory symptoms. (Copyright © 2026 Elsevier Ltd. All rights reserved.) |
| Competing Interests: | Declaration of competing interest All authors received grant or salary support from the Cystic Fibrosis Foundation. |
| Contributed Indexing: | Keywords: Cystic fibrosis; Elexacaftor/tezacaftor/ivacaftor; Symptom burden |
| Substance Nomenclature: | 0 (Benzodioxoles) 0 (Drug Combinations) 0 (Indoles) 0 (Pyrazoles) 0 (Pyridines) 0 (Pyrroles) 0 (Quinolones) 0 (elexacaftor, ivacaftor, tezacaftor drug combination) 0 (Aminophenols) 0 (Quinolines) |
| Entry Date(s): | Date Created: 20260528 Date Completed: 20260612 Latest Revision: 20260622 |
| Update Code: | 20260622 |
| DOI: | 10.1016/j.rmed.2026.108908 |
| PMID: | 42208687 |
| Βάση Δεδομένων: | MEDLINE |
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