{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,12]],"date-time":"2026-11-12T14:25:06Z","timestamp":1794493506451,"version":"4.9.1"},"reference-count":0,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T00:00:00Z","timestamp":1785888000000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"Abstract<\/jats:title>\n \n Rationale<\/jats:title>\n Given improvement in symptoms and lung function in people with cystic fibrosis (CF) taking elexacaftor\/tezacaftor\/ivacaftor (ETI), there is increasing interest in de-escalation of care although the effect on outcomes remains unknown.<\/jats:p>\n <\/jats:sec>\n \n Objectives<\/jats:title>\n To determine if self-reported discontinuation of chronic daily therapies (CDT) is associated with changes in pulmonary exacerbations and lung function in individuals taking ETI.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n The Home Reported Outcomes in Cystic Fibrosis 2 study was a 12-month prospective observational study in individuals age 12 and older taking ETI. This remote study collected patient-reported use and prior discontinuation of CDT at study entry via the Folia Health application. Primary outcomes were lung function and intravenous-antibiotic treated pulmonary exacerbations (PEx) as reported in the CF Foundation Patient Registry. Inverse probability weighting was used to compare outcomes between those that did and did not discontinue a CDT.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n A total of 860 individuals consented, and 709 were included in these analyses. At baseline, 41.5% self-reported discontinuation of 1 or more CDTs. Over 12 months, those who discontinued CDT did not have any significant difference in the probability of PEx (mean difference -2.0%, 95% CI -6.7, 2.8%) or rate of change in lung function (mean difference 0.41%, 95% CI -1.40, 2.22).<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n In individuals taking ETI, self-reported discontinuation of CDT was not associated with an increased risk of PEx or decline in lung function.<\/jats:p>\n <\/jats:sec>","DOI":"10.1093\/annalsats\/aaoag234","type":"journal-article","created":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T11:40:56Z","timestamp":1785930056000},"source":"Crossref","is-referenced-by-count":1,"title":["Pulmonary Outcomes in People with Cystic Fibrosis who Stop Chronic Daily Therapies: The HERO-2 Study"],"prefix":"10.1093","author":[{"given":"Cynthia D","family":"Brown","sequence":"first","affiliation":[{"name":"Indiana University School of Medicine Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, , Indianapolis, IN,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin J","family":"Psoter","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Department of Pediatrics, , Baltimore, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn A","family":"Sabadosa","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation , Bethesda, MD,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua S","family":"Ostrenga","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation , Bethesda, MD,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley H","family":"Rosen","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Indiana University School of Medicine , Indianapolis, IN,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgene","family":"Hergenroeder","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Children\u2019s Hospital of Philadelphia , Philadelphia, PA,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Bendy","sequence":"additional","affiliation":[{"name":"Riley Hospital for Children , Indianapolis, IN,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Connie","family":"Zhang","sequence":"additional","affiliation":[{"name":"Folia Health , Cambridge, MA,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nell Meosky","family":"Luo","sequence":"additional","affiliation":[{"name":"Folia Health , Cambridge, MA,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clement L","family":"Ren","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Children\u2019s Hospital of Philadelphia , Philadelphia, PA,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,8,5]]},"container-title":["Annals of the American Thoracic Society"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/annalsats\/advance-article-pdf\/doi\/10.1093\/annalsats\/aaoag234\/70492579\/aaoag234.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/annalsats\/advance-article-pdf\/doi\/10.1093\/annalsats\/aaoag234\/70492579\/aaoag234.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T11:40:57Z","timestamp":1785930057000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/annalsats\/advance-article\/doi\/10.1093\/annalsats\/aaoag234\/8752384"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,5]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1093\/annalsats\/aaoag234","relation":{},"ISSN":["2325-6621"],"issn-type":[{"value":"2325-6621","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,5]]},"article-number":"aaoag234"}}