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Document Type
Poster
Description
CysticFibrosis(CF)isanautosomalrecessivedisorderthatshortensone’slifeduetoitseffectonthecysticfibrosistransmembraneregulator(CFTR)gene.CFTRmodulatormedicationscancorrecttheCFTRgeneandconsistofapotentiator(ivacaftor),andcorrector(lumacaftor,tezacaftorandelexacaftor).ThisresearchandliteraturereviewsoughttoevaluateiflungfunctionimprovedinanindividualwithgeneticallyconfirmedCFafterbeingstartedonCFTRmodulatormedications.Tocompletethereview,sixdatabasesweresearched:CINAHL,PubMed,ClinicalKey,CochraneLibrary,EmbaseandDynamedPlus.BothkeywordandmeshtermswereusedtodefineasetoftheliteraturediscussingCFandCFTRmodulatoruse.Atotalof24studieswereincludedandmettheinclusioncriteriaof:peerreviewed,publishedwithinthepastsixyears,greaterthan20studyparticipants,andwerenotlimitedcasereports.Phase2studieswereincludedforelexacaftor-tezacaftor-ivacaftorandtezacaftor-ivacaftor,duetolimitedresearchinthisarea.Researchindicatedthativacaftorimprovedpercentpredictedforcedexpiratoryvolumeinonesecond(ppFEV1)inindividualswithG551D,non-G551Dgatingmutations,Gly-Asp-CFTRandARG117HISmutations.Elexacaftor-tezacaftor-ivacaftorimprovedppFEV1inheterozygousandhomozygousPhe508delgreaterthanivacaftororlumacaftor-ivacaftor.Ivacaftorandlumacaftor-ivacaftormayhaveagedependentchangesonppFEV1.IndividualswithppFEV1<40%appeartohaveapositiveresponsetotreatment,howevertheymayhaveincreasedsideeffectsandresponsemaybedelayed.Additionalresearchisneededonagedependent,longitudinalresponse,andefficacyoftreatment.
Department
Physician Assistant Studies
Date of Work
2021
Publication Date
2021
Keywords
Cystic Fibrosis, CFTR Modulators, Respiratory Function, autosomal recessive disorder, multi-system disorder, genetic mutation CFTR gene, obstructive lung disease, Ivacaftor monotherapy
Disciplines
Medicine and Health Sciences
Recommended Citation
Severson, Melissa, "Effect of CFTR Modulators on Respiratory Function in Adults with Cystic Fibrosis" (2021). Physician Assistant Scholarly Project Posters. 226.
https://commons.und.edu/pas-grad-posters/226