{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T09:34:44Z","timestamp":1794044084369,"version":"4.8.0"},"reference-count":43,"publisher":"European Respiratory Society (ERS)","issue":"173","license":[{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir Rev"],"accepted":{"date-parts":[[2024,6,18]]},"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators. Adequate medication adherence is a prerequisite for their effectiveness, but little is known about adherence to CFTR modulators. We aimed to assess the extent of medication adherence to CFTR modulators in patients with CF and assess which characteristics are associated with adherence.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic review following PRISMA guidelines was performed. Studies needed to report adherence to CFTR modulators. Main outcomes were: 1) level of medication adherence and 2) associations of demographic and\/or clinical characteristics with adherence.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 4082 articles were screened and 21 full-text papers were assessed for eligibility. Ultimately, seven studies were included. Most studies were retrospective and focused on adherence to ivacaftor or lumacaftor\u2013ivacaftor with only one focusing on elexacaftor\u2013tezacaftor\u2013ivacaftor. The majority used pharmacy refill data with adherence determined with the proportion of days covered (PDC) or the medication possession ratio (MPR). One study additionally used electronic monitoring and patient self-reported adherence. Adherence was 0.62\u20130.99 based on pharmacy data (PDC or MPR), 61%<jats:italic>via<\/jats:italic>electronic monitoring and 100%<jats:italic>via<\/jats:italic>self-report. Age &lt;18\u2005years appeared to be associated with good adherence, as was a higher lung function.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Despite the wide variety of adherence methods used, adherence to CFTR modulators is suboptimal, based on objective measures such as pharmacy refill data or electronic monitoring. CFTR modulator adherence measurement and definitions requires more standardisation with a preference for objective and granular methods.<\/jats:p><\/jats:sec>","DOI":"10.1183\/16000617.0060-2024","type":"journal-article","created":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T08:42:35Z","timestamp":1723624955000},"page":"240060","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":28,"title":["Medication adherence to CFTR modulators in patients with cystic fibrosis: a systematic review"],"prefix":"10.1183","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-1468-0103","authenticated-orcid":false,"given":"Carina M.E.","family":"Hansen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna J.","family":"Breukelman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia M.L.A.","family":"van den Bemt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annelies M.","family":"Zwitserloot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liset","family":"van Dijk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Job F.M.","family":"van Boven","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2024,8,14]]},"reference":[{"key":"2024102404231134000_33.173.240060.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.01.009"},{"key":"2024102404231134000_33.173.240060.2","doi-asserted-by":"crossref","first-page":"e87","DOI":"10.1016\/j.lpm.2017.04.012","article-title":"The changing epidemiology and demography of cystic fibrosis","volume":"46","author":"Stephenson","year":"2017","journal-title":"Presse Med"},{"key":"2024102404231134000_33.173.240060.3","first-page":"500","article-title":"CFTR modulators for the treatment of cystic fibrosis","volume":"39","author":"Pettit","year":"2014","journal-title":"PT"},{"key":"2024102404231134000_33.173.240060.4","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1016\/j.jcf.2015.05.009","article-title":"Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor","volume":"14","author":"Siracusa","year":"2015","journal-title":"J Cyst Fibros"},{"key":"2024102404231134000_33.173.240060.5","doi-asserted-by":"crossref","first-page":"30","DOI":"10.7326\/M18-0543","article-title":"ESPACOMP medication adherence reporting guideline (EMERGE)","volume":"169","author":"De Geest","year":"2018","journal-title":"Ann Intern Med"},{"key":"2024102404231134000_33.173.240060.6","doi-asserted-by":"crossref","first-page":"802","DOI":"10.1016\/j.jaip.2016.05.019","article-title":"What we mean when we talk about adherence in respiratory medicine","volume":"4","author":"Vrijens","year":"2016","journal-title":"J Allergy Clin Immunol Pract"},{"key":"2024102404231134000_33.173.240060.7","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.jcf.2023.12.002","article-title":"Standards for the care of people with cystic fibrosis; establishing and maintaining health","volume":"23","author":"Southern","year":"2024","journal-title":"J Cyst Fibros"},{"key":"2024102404231134000_33.173.240060.8","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1002\/ppul.26774","article-title":"Assessment of long-term medication adherence with cystic fibrosis: an integrated approach","volume":"59","author":"Platt","year":"2023","journal-title":"Pediatr Pulmonol"},{"key":"2024102404231134000_33.173.240060.9","doi-asserted-by":"publisher","DOI":"10.1111\/apa.15155"},{"key":"2024102404231134000_33.173.240060.10","doi-asserted-by":"crossref","first-page":"1497","DOI":"10.2147\/PPA.S211769","article-title":"Adherence to long-term therapies in cystic fibrosis: a French cross-sectional study linking prescribing, dispensing, and hospitalization data","volume":"13","author":"Rouz\u00e9","year":"2019","journal-title":"Patient Prefer Adherence"},{"key":"2024102404231134000_33.173.240060.11","doi-asserted-by":"crossref","first-page":"3017","DOI":"10.1002\/ppul.26126","article-title":"Pulmonary medication adherence among children and adults with cystic fibrosis: is there an association with disease severity?","volume":"57","author":"Hatziagorou","year":"2022","journal-title":"Pediatr Pulmonol"},{"key":"2024102404231134000_33.173.240060.12","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-1926"},{"key":"2024102404231134000_33.173.240060.13","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMp1009202"},{"key":"2024102404231134000_33.173.240060.14","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pmed.1000097"},{"key":"2024102404231134000_33.173.240060.15","doi-asserted-by":"publisher","DOI":"10.1186\/s13643-016-0384-4"},{"key":"2024102404231134000_33.173.240060.16","unstructured":"Wells GA , Shea BJ , O'Connel D , et al. 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