{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:29:01Z","timestamp":1790803741246,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,7,15]],"date-time":"2025-07-15T00:00:00Z","timestamp":1752537600000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>The HERO\u20102 study is a prospective, observational study investigating chronic daily therapy (CDT) changes among people with cystic fibrosis (PwCF)\u2009\u2265\u200912 years of age taking elexacaftor\/tezacaftor\/ivacaftor (ETI). The goal of this analysis was to describe baseline characteristics and patterns of CDT discontinuation reported at study enrollment and identify clinical features associated with CDT discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Study participants were recruited through their CF center, community engagement, or a smartphone application (Folia Health). Study enrollment and participation took place remotely through Folia Health. Participants gave consent to link their data with the CF Foundation Patient Registry (CFFPR). At enrollment, participants completed a baseline survey describing CDT use since starting ETI. Descriptive statistics were used to summarize HERO\u20102 participant characteristics. Logistic regression evaluated the association of selected individual characteristics with odds of CDT discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 860 PwCF enrolled in HERO\u20102, and 755 (87.8%) completed the enrollment survey and were linked to the CFFPR. At enrollment, 313 participants (41.5%) reported discontinuation of at least one CDT. The most frequently discontinued CDT was airway clearance (22.4%). Intravenous (IV) antibiotic treatment for a pulmonary exacerbation in the prior year and public insurance were associated with lower odds of CDT discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this large cohort of PwCF taking ETI, approximately 42% reported discontinuation of at least one CDT. Providers should be aware of these CDT discontinuation patterns and engage in open dialogue with PwCF and their families about CDT de\u2010escalation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71190","type":"journal-article","created":{"date-parts":[[2025,7,15]],"date-time":"2025-07-15T11:10:43Z","timestamp":1752577843000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Baseline Characteristics and Self\u2010Reported Use of Chronic Daily Therapies of the Home Reported Outcomes With CFTR Modulator Therapy (HERO\u20102) Cohort"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1793-2173","authenticated-orcid":false,"given":"Georgene E.","family":"Hergenroeder","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin J.","family":"Psoter","sequence":"additional","affiliation":[{"name":"Division of General Pediatrics, Department of Pediatrics Johns Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia D.","family":"Brown","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Department of Medicine Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josh","family":"Ostrenga","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3855-770X","authenticated-orcid":false,"given":"Christine","family":"Ford","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine Johns Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Bendy","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy, and Sleep Medicine, Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley H.","family":"Rosen","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Department of Medicine Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nell Meosky","family":"Luo","sequence":"additional","affiliation":[{"name":"Folia Health Inc Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Connie","family":"Zhang","sequence":"additional","affiliation":[{"name":"Folia Health Inc Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn A.","family":"Sabadosa","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4431-0644","authenticated-orcid":false,"given":"Clement L.","family":"Ren","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,7,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"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\/S0140-6736(19)32597-8"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.11.007"},{"issue":"40","key":"e_1_2_10_6_1","first-page":"S74","article-title":"Tackling the Increasing Complexity of CF Care","volume":"50","author":"Sawicki G. S.","year":"2015","journal-title":"Pediatric Pulmonology"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2012.12.009"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2022-001420"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(22)00434-9"},{"key":"e_1_2_10_10_1","unstructured":"ISRCTNregistry. Can People With Cystic Fibrosis Safely Stop Taking Some of Their Nebulised Treatments If They Are Established on the New Modulator Therapy Kaftrio? 2024 09\/11\/2024 11\/04\/2024].https:\/\/www.isrctn.com\/ISRCTN14081521."},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201308-276RM"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.08.008"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202108-1986OC"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.27018"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.05.001"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.05.005"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.11.004"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/17534666241235054"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chpulm.2024.100080"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201511-781OC"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25285"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71190","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71190","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71190","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T12:55:56Z","timestamp":1771332956000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71190"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7]]},"references-count":20,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2025,7]]}},"alternative-id":["10.1002\/ppul.71190"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71190","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7]]},"assertion":[{"value":"2025-01-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71190"}}