{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T16:07:44Z","timestamp":1773245264323,"version":"3.50.1"},"reference-count":17,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T00:00:00Z","timestamp":1726617600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T00:00:00Z","timestamp":1726617600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>In 2015, a survey of cystic fibrosis (CF) physicians showed significant gaps in lung transplant (LTx) referral knowledge. Subsequently, LTx referral guidelines for people with CF were published, and elexacaftor\/tezacaftor\/ivacaftor (ETI) became available for &gt;80% of people in the United States (US). We sought to assess physicians\u2019 LTx referral knowledge and self\u2010reported referral practices.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>CF center directors in the US were surveyed about LTx. Questions addressed transplant referral indications, contraindications, testing, and the impact of ETI on referral timing. Thematic analysis was used to assess responses to open\u2010ended questions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      There were 110\/309 (36%) responses. Respondents identified several referral indications, including rapid decline in FEV\n                      <jats:sub>1<\/jats:sub>\n                      (93%), recurrent hemoptysis (80%), hypoxemia (79%), and pulmonary hypertension (75%). Over 70% of respondents reported using oximetry, echocardiogram, and blood gas to assess disease severity. Respondents were more likely to find early LTx discussions useful for patients not on modulators versus on modulators (87% vs. 63%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u2009.005). Most respondents (66%) reported delaying LTx referral for some patients with FEV\n                      <jats:sub>1<\/jats:sub>\n                      30%\u201340% who met criteria, while 26% had delayed referral for patients with FEV\n                      <jats:sub>1<\/jats:sub>\n                      \u2009&lt;\u200930%. Uncertainty regarding optimal LTx referral timing for patients on ETI was a prominent theme of the qualitative analysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>While physician knowledge about LTx referral indications appears improved since the CF referral guidelines were published, uncertainty about referral timing is pervasive, and the guidelines will need to be updated as more data become available about the long\u2010term effectiveness of ETI in advanced lung disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.27273","type":"journal-article","created":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T07:41:19Z","timestamp":1726645279000},"page":"3641-3649","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A survey of cystic fibrosis physicians' views on lung transplant referral in the era of elexacaftor\/tezacaftor\/ivacaftor"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-4610-4769","authenticated-orcid":false,"given":"Nora","family":"Burdis","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tijana","family":"Milinic","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauren E.","family":"Bartlett","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louisa","family":"Goss","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erin","family":"Tallarico","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abigail","family":"Boyle","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abigail","family":"Thaxton","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grant A.","family":"Turner","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of California at Los Angeles Los Angeles California USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luke J.","family":"Benvenuto","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Columbia University New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Albert","family":"Faro","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher H.","family":"Goss","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"},{"name":"Division of Pulmonary and Allergy, Department of Pediatrics University of Washington Seattle Washington USA"},{"name":"Center for Respiratory Biology and Therapeutics Seattle Children's Research Institute Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siddhartha G.","family":"Kapnadak","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathleen J.","family":"Ramos","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine University of Washington Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,9,18]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-017-0367-9"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.03.002"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202011-4153OC"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11041021"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.arbres.2023.05.017"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chpulm.2024.100077"},{"key":"e_1_2_11_10_1","volume-title":"Registry CFFP. 2022 annual data 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