{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T22:48:35Z","timestamp":1779230915600,"version":"3.51.4"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,12,7]],"date-time":"2022-12-07T00:00:00Z","timestamp":1670371200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["SATHE20A0"],"award-info":[{"award-number":["SATHE20A0"]}],"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":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The success of highly effective modulator therapy (HEMT) has led to consideration of simpler regimens for people with CF (PwCF) with opportunities to modify burdensome regimens. Despite the intuitive appeal of discontinuing chronic therapies no longer necessary, this process should be pursued systematically to ensure safety, adherence, and validate patient\u2010centered preferences. We designed a questionnaire to determine the state of use of acid\u2010suppressive medications (ASM) and pancreatic enzyme therapy (PERT), current self\u2010withdrawal and provider\u2010directed withdrawal practices, and interest in a standardized withdrawal study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In collaboration with CF Foundation (CFF), a questionnaire was developed and distributed to members of Community Voice (CV, comprised of PwCF and their loved ones), and CF providers regarding the need to study simplifying the gastrointestinal (GI) regimen for PwCF on HEMT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Approximately 20\u201040% of CV or CF providers have decreased or stopped ASM for those on HEMT. For PERT, CV and CF providers have decreased dose (34%\u201348% and approximately 25%, respectively) more often than having stopped it altogether (13%\u201324% and 3%\u201312%, respectively). Cumulatively, there is interest in pursuing research in this area (86% CV and 89% CF providers) and willingness to enroll in such a study (80% CV and 89% CF providers).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Systematically studying the withdrawal of common GI medications, ASM and PERT, is important to CV and CF providers. Decreases in dosing and withdrawal are already taking place without evidence to support this practice. This questionnaire is the first step in designing a GI medication simplification study in PwCF on HEMT.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26257","type":"journal-article","created":{"date-parts":[[2022,11,30]],"date-time":"2022-11-30T08:30:20Z","timestamp":1669797020000},"page":"811-818","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Need to study simplification of gastrointestinal medication regimen in cystic fibrosis in the era of highly effective modulators"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7088-8530","authenticated-orcid":false,"given":"Meghana","family":"Sathe","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition University of Texas Southwestern\/Children's Health  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Baha","family":"Moshiree","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Wake Forest\/Atrium Health  Charlotte North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enid","family":"Aliaj","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation: Community Partnerships  Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4329-506X","authenticated-orcid":false,"given":"MinJae","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Biostatistics, Department of Population &amp; Data Sciences University of Texas Southwestern  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica","family":"Hudson","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation: Community Partnerships  Bethesda Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex","family":"Gifford","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine University Hospitals Cleveland Medical Center  Ohio Cleveland USA"},{"name":"Division of Pediatric Pulmonology Rainbow Babies and Children's Hospital  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susan","family":"Attel","sequence":"additional","affiliation":[{"name":"Patient Representative, Children's Health  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Breck","family":"Gamel","sequence":"additional","affiliation":[{"name":"Patient Representative, Children's Health  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven D.","family":"Freedman","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology Beth Israel Deaconess Medical Center  Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah Jane","family":"Schwarzenberg","sequence":"additional","affiliation":[{"name":"University of Minnesota\/MHealth Fairview Masonic Children's Hospital  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. Jay","family":"Freeman","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Nationwide Children's Hospital\/Ohio State School of Medicine  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909825"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.05.018"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ctg.2017.10"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(15)00545-7"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(18)30202-9"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2022.877118"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2017-210473"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.11.010"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202010-1336SD"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.4251\/wjgo.v13.i9.1121"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.11.005"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12916-016-0718-z"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms20205203"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.4292\/wjgpt.v2.i3.17"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamainternmed.2015.42"},{"issue":"3","key":"e_1_2_8_18_1","first-page":"193","article-title":"Effect of long\u2010term proton pump inhibitors on bone mineral density","volume":"96","author":"Romdhane H","year":"2018","journal-title":"Tunis Med"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamainternmed.2015.7193"},{"issue":"21","key":"e_1_2_8_20_1","first-page":"1","article-title":"Effect of esomeprazole versus placebo on pulmonary excaberations in cystic fibrosis","volume":"14","author":"Dimango E","year":"2014","journal-title":"BMC Pulm Med"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.14740\/gr917w"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25431"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1177\/014107689809134S07"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0174463"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40271-019-00361-2"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu11020236"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcte.2021.100280"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.26257","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.26257","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.26257","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,18]],"date-time":"2023-08-18T12:07:38Z","timestamp":1692360458000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.26257"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,7]]},"references-count":26,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["10.1002\/ppul.26257"],"URL":"https:\/\/doi.org\/10.1002\/ppul.26257","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,7]]},"assertion":[{"value":"2022-08-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-25","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-07","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}