{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T03:23:25Z","timestamp":1767842605326,"version":"3.49.0"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T00:00:00Z","timestamp":1709856000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100011082","name":"School of Medicine, University of North Carolina at Chapel Hill","doi-asserted-by":"publisher","award":["T35\u2010DK007386"],"award-info":[{"award-number":["T35\u2010DK007386"]}],"id":[{"id":"10.13039\/100011082","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Cystic fibrosis (CF)\u00a0treatment has increasingly focused on highly effective modulators. Despite measurable benefits of modulators, there is little guidance for CF care team members on providing education and support to patients regarding initiation of these therapies. We aimed to explore patient, caregiver, and clinician perceptions of modulators and influences on decisions about starting cystic fibrosis transmembrane regulator (CFTR) modulators.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted semistructured interviews with CF clinicians, adults with CF, and caregivers of children with CF. We reviewed audio recordings and coded responses to identify central themes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We interviewed 8 CF clinicians, 9 adults with CF, and 11 caregivers of children with CF. Themes centered on emotional responses to modulator availability, influences on decision\u2010making, concerns about side effects, impact of modulators on planning for the future, the benefits of the multidisciplinary CF care team in supporting treatment decisions, and the unique needs of people with CF who are not eligible for modulators. Clinicians described changes in conversations about modulators since the approval of elexacaftor\/tezacaftor\/ivacaftor, specifically greater willingness to prescribe with less nuanced conversations with patients and\/or caregivers regarding their use.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Based on perspectives and experiences of CF clinicians, adults with CF, and caregivers of children with CF, we suggest clinicians approach conversations about CFTR modulators thoughtfully and thoroughly, utilizing the multidisciplinary model of CF care in exploring patient and caregiver emotions while filling in knowledge gaps, asking about treatment goals beyond potential clinical benefit, and having compassionate conversations with those who are ineligible for modulators.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26953","type":"journal-article","created":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T13:04:35Z","timestamp":1709903075000},"page":"1614-1621","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Exploring perceptions of and decision\u2010making about CFTR modulators"],"prefix":"10.1002","volume":"59","author":[{"given":"Lee","family":"Landess","sequence":"first","affiliation":[{"name":"University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary G.","family":"Prieur","sequence":"additional","affiliation":[{"name":"Department of Psychiatry University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashley R.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Psychiatry University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3794-6329","authenticated-orcid":false,"given":"Elisabeth P.","family":"Dellon","sequence":"additional","affiliation":[{"name":"University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,3,8]]},"reference":[{"key":"e_1_2_10_2_1","unstructured":"Cystic Fibrosis Foundation.About cystic fibrosis.\u00a0Accessed October 2023.\u00a0http:\/\/www.cff.org\/AboutCF\/"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2018.04.036"},{"key":"e_1_2_10_5_1","unstructured":"Food and Drug Administration. FDA approves new breakthrough therapy for cystic fibrosis.\u00a02019."},{"key":"e_1_2_10_6_1","unstructured":"Cystic Fibrosis Foundation Patient Registry 2022 Annual Data Report. Bethesda Maryland 2022.\u00a0Accessed October 2023.\u00a0https:\/\/www.cff.org\/sites\/default\/files\/2021-11\/Patient-Registry-Annual-Data-Report.pdf"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2021.01.1038"},{"issue":"2","key":"e_1_2_10_8_1","first-page":"281","article-title":"CFTR modulators: transformative therapies for cystic fibrosis","volume":"27","author":"Dwight M","year":"2021","journal-title":"J Manag Care Spec Pharm"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.psym.2020.06.021"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.psym.2017.04.001"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.05.008"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/jhn.12967"},{"key":"e_1_2_10_13_1","unstructured":"Malik. CFcommunity blog.\u00a0Cystic Fibrosis Foundation. Accessed October 2023. 2019.\u00a0https:\/\/www.cff.org\/CF-Community-Blog\/Posts\/2019\/How-CFTRModulators%E2%80%90Changed%E2%80%90My%E2%80%90Reality\/"},{"key":"e_1_2_10_14_1","unstructured":"FoxC.\u00a0CFcommunity blog.\u00a0The Cystic Fibrosis Foundation.\u00a0Accessed October 2023. 2019.\u00a0https:\/\/www.cff.org\/community-posts\/2019-10\/i-have-two-nonsense-mutations-heres-what-path-cure-means-me"},{"key":"e_1_2_10_15_1","unstructured":"AmberE. CF community blog.\u00a0The Cystic Fibrosis Foundation.\u00a02019.\u00a0https:\/\/www.cff.org\/community-posts\/2019-11\/what-does-better-mean-trikafta"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1369-6513.2000.00076.x"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pec.2016.01.020"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11606-020-05768-4"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMp1209500"},{"key":"e_1_2_10_20_1","unstructured":"BurgessBE. Cognitive factors and treatment adherence in individuals with cystic fibrosis. Accessed October 2023.\u00a02020.\u00a0https:\/\/www.proquest.com\/openview\/5920f3fb54c5f718f18ee1906bf9e45f\/1?pq-origsite=gscholar&cbl=18750&diss=y"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(23)00324-7"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.26953","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T14:32:26Z","timestamp":1737124346000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.26953"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,8]]},"references-count":20,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,6]]}},"alternative-id":["10.1002\/ppul.26953"],"URL":"https:\/\/doi.org\/10.1002\/ppul.26953","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,8]]},"assertion":[{"value":"2023-10-06","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-02-27","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-08","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}