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id="vector-page-titlebar-toc" class="vector-dropdown vector-page-titlebar-toc vector-button-flush-left" title="Table of Contents" > <input type="checkbox" id="vector-page-titlebar-toc-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-vector-page-titlebar-toc" class="vector-dropdown-checkbox " aria-label="Toggle the table of contents" > <label id="vector-page-titlebar-toc-label" for="vector-page-titlebar-toc-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--icon-only " aria-hidden="true" ><span class="vector-icon mw-ui-icon-listBullet mw-ui-icon-wikimedia-listBullet"></span> <span class="vector-dropdown-label-text">Toggle the table of contents</span> </label> <div class="vector-dropdown-content"> <div id="vector-page-titlebar-toc-unpinned-container" class="vector-unpinned-container"> </div> </div> </div> </nav> <h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Ivacaftor</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. Available in 12 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-12" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">12 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D8%A5%D9%8A%D9%81%D8%A7%D9%83%D8%A7%D9%81%D8%AA%D9%88%D8%B1" title="إيفاكافتور – Arabic" lang="ar" hreflang="ar" data-title="إيفاكافتور" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-azb mw-list-item"><a href="https://azb.wikipedia.org/wiki/%D8%A7%DB%8C%D9%88%D8%A7%DA%A9%D8%A7%D9%81%D8%AA%D9%88%D8%B1" title="ایواکافتور – South Azerbaijani" lang="azb" hreflang="azb" data-title="ایواکافتور" data-language-autonym="تۆرکجه" data-language-local-name="South Azerbaijani" class="interlanguage-link-target"><span>تۆرکجه</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Ivacaftor" title="Ivacaftor – Spanish" lang="es" hreflang="es" data-title="Ivacaftor" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fa mw-list-item"><a href="https://fa.wikipedia.org/wiki/%D8%A7%DB%8C%D9%88%D8%A7%DA%A9%D8%A7%D9%81%D8%AA%D9%88%D8%B1" title="ایواکافتور – Persian" lang="fa" hreflang="fa" data-title="ایواکافتور" data-language-autonym="فارسی" data-language-local-name="Persian" class="interlanguage-link-target"><span>فارسی</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Ivacaftor" title="Ivacaftor – French" lang="fr" hreflang="fr" data-title="Ivacaftor" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-he mw-list-item"><a href="https://he.wikipedia.org/wiki/%D7%90%D7%99%D7%91%D7%A7%D7%A4%D7%98%D7%95%D7%A8" title="איבקפטור – Hebrew" lang="he" hreflang="he" data-title="איבקפטור" data-language-autonym="עברית" data-language-local-name="Hebrew" class="interlanguage-link-target"><span>עברית</span></a></li><li class="interlanguage-link interwiki-nl mw-list-item"><a href="https://nl.wikipedia.org/wiki/Ivacaftor" title="Ivacaftor – Dutch" lang="nl" hreflang="nl" data-title="Ivacaftor" data-language-autonym="Nederlands" data-language-local-name="Dutch" class="interlanguage-link-target"><span>Nederlands</span></a></li><li class="interlanguage-link interwiki-or mw-list-item"><a href="https://or.wikipedia.org/wiki/%E0%AC%87%E0%AC%AD%E0%AC%BE%E0%AC%95%E0%AC%BE%E0%AC%AB%E0%AD%8D%E0%AC%9F%E0%AC%B0" title="ଇଭାକାଫ୍ଟର – Odia" lang="or" hreflang="or" data-title="ଇଭାକାଫ୍ଟର" data-language-autonym="ଓଡ଼ିଆ" data-language-local-name="Odia" class="interlanguage-link-target"><span>ଓଡ଼ିଆ</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Iwakaftor" title="Iwakaftor – Polish" lang="pl" hreflang="pl" data-title="Iwakaftor" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-sl mw-list-item"><a href="https://sl.wikipedia.org/wiki/Ivakaftor" title="Ivakaftor – Slovenian" lang="sl" hreflang="sl" data-title="Ivakaftor" data-language-autonym="Slovenščina" data-language-local-name="Slovenian" class="interlanguage-link-target"><span>Slovenščina</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Ivakaftor" title="Ivakaftor – Serbian" lang="sr" hreflang="sr" data-title="Ivakaftor" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-sh mw-list-item"><a href="https://sh.wikipedia.org/wiki/Ivakaftor" title="Ivakaftor – Serbo-Croatian" lang="sh" hreflang="sh" data-title="Ivakaftor" data-language-autonym="Srpskohrvatski / српскохрватски" data-language-local-name="Serbo-Croatian" class="interlanguage-link-target"><span>Srpskohrvatski / српскохрватски</span></a></li> </ul> <div class="after-portlet after-portlet-lang"><span class="wb-langlinks-edit wb-langlinks-link"><a href="https://www.wikidata.org/wiki/Special:EntityPage/Q6095693#sitelinks-wikipedia" 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noexcerpt noprint searchaux" style="display:none">Pharmaceutical compound</div> <style data-mw-deduplicate="TemplateStyles:r1269284339">@media screen{html.skin-theme-clientpref-night .mw-parser-output .dark_mode_safe img{background-color:var(--background-color-inverted,#f8f9fa)}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .dark_mode_safe img{background-color:var(--background-color-inverted,#f8f9fa)}}</style> <style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox" style="border-spacing:2px;"><caption class="infobox-title"><span title="International nonproprietary name (INN): Ivacaftor">Ivacaftor</span></caption><tbody><tr><td colspan="2" class="infobox-image"><span class="mw-default-size dark_mode_safe" typeof="mw:File/Frameless"><a href="/wiki/File:Ivacaftor.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/Ivacaftor.svg/220px-Ivacaftor.svg.png" decoding="async" width="220" height="133" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/Ivacaftor.svg/330px-Ivacaftor.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/7/77/Ivacaftor.svg/440px-Ivacaftor.svg.png 2x" data-file-width="512" data-file-height="309" /></a></span></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Clinical data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;">Pronunciation</th><td class="infobox-data"><span class="rt-commentedText nowrap"><span class="IPA nopopups noexcerpt" lang="en-fonipa"><a href="/wiki/Help:IPA/English" title="Help:IPA/English">/<span style="border-bottom:1px dotted"><span title="/ˌ/: secondary stress follows">ˌ</span><span title="/aɪ/: 'i' in 'tide'">aɪ</span><span title="'v' in 'vie'">v</span><span title="/ə/: 'a' in 'about'">ə</span><span title="/ˈ/: primary stress follows">ˈ</span><span title="'k' in 'kind'">k</span><span title="/æ/: 'a' in 'bad'">æ</span><span title="'f' in 'find'">f</span><span title="'t' in 'tie'">t</span><span title="/ər/: 'er' in 'letter'">ər</span></span>/</a></span></span><br /><a href="/wiki/Help:Pronunciation_respelling_key" title="Help:Pronunciation respelling key"><i title="English pronunciation respelling"><span style="font-size:90%">EYE</span>-və-<span style="font-size:90%">KAF</span>-tər</i></a> </td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Drug_nomenclature#Trade_names" title="Drug nomenclature">Trade names</a></th><td class="infobox-data">Kalydeco</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;">Other names</th><td class="infobox-data">VX-770</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/American_Society_of_Health-System_Pharmacists" title="American Society of Health-System Pharmacists">AHFS</a>/<a href="/wiki/Drugs.com" title="Drugs.com">Drugs.com</a></th><td class="infobox-data"><span title="www.drugs.com"><a rel="nofollow" class="external text" href="https://www.drugs.com/monograph/ivacaftor.html">Monograph</a></span></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></th><td class="infobox-data"><span title="medlineplus.gov"><a rel="nofollow" class="external text" href="https://medlineplus.gov/druginfo/meds/a612012.html">a612012</a></span></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Regulation_of_therapeutic_goods" title="Regulation of therapeutic goods">License data</a></th><td class="infobox-data"><style data-mw-deduplicate="TemplateStyles:r1126788409">.mw-parser-output .plainlist ol,.mw-parser-output .plainlist ul{line-height:inherit;list-style:none;margin:0;padding:0}.mw-parser-output .plainlist ol li,.mw-parser-output .plainlist ul li{margin-bottom:0}</style><div class="plainlist"> <ul><li><small><abbr class="country-name" title="United States">US</abbr></small> <a href="/wiki/DailyMed" title="DailyMed">DailyMed</a>: <span title="dailymed.nlm.nih.gov"><a rel="nofollow" class="external text" href="https://dailymed.nlm.nih.gov/dailymed/search.cfm?labeltype=all&query=Ivacaftor">Ivacaftor</a></span></li></ul></div> </td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Pregnancy_category" title="Pregnancy category">Pregnancy<br />category</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"> <ul><li><small><abbr class="country-name" title="Australia">AU</abbr>:</small> B3<sup id="cite_ref-Drugs.com_pregnancy_1-0" class="reference"><a href="#cite_note-Drugs.com_pregnancy-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></li> <li class="mw-empty-elt"></li></ul> </div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Route_of_administration" title="Route of administration">Routes of<br />administration</a></th><td class="infobox-data"><a href="/wiki/Oral_administration" title="Oral administration">By mouth</a></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Anatomical_Therapeutic_Chemical_Classification_System" title="Anatomical Therapeutic Chemical Classification System">ATC code</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><a href="/wiki/ATC_code_R07" title="ATC code R07">R07AX02</a> (<span title="www.whocc.no"><a rel="nofollow" class="external text" href="https://www.whocc.no/atc_ddd_index/?code=R07AX02">WHO</a></span>) <a href="/wiki/ATC_code_R07" title="ATC code R07">R07AX30</a> (<span title="www.whocc.no"><a rel="nofollow" class="external text" href="https://www.whocc.no/atc_ddd_index/?code=R07AX30">WHO</a></span>), <a href="/wiki/ATC_code_R07" title="ATC code R07">R07AX31</a> (<span title="www.whocc.no"><a rel="nofollow" class="external text" href="https://www.whocc.no/atc_ddd_index/?code=R07AX31">WHO</a></span>), <a href="/wiki/ATC_code_R07" title="ATC code R07">R07AX32</a> (<span title="www.whocc.no"><a rel="nofollow" class="external text" href="https://www.whocc.no/atc_ddd_index/?code=R07AX32">WHO</a></span>),</li></ul></div></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Legal status</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Regulation_of_therapeutic_goods" title="Regulation of therapeutic goods">Legal status</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"> <ul><li><small><abbr class="country-name" title="Australia">AU</abbr>:</small> <a href="/wiki/Standard_for_the_Uniform_Scheduling_of_Medicines_and_Poisons#Schedule_4" title="Standard for the Uniform Scheduling of Medicines and Poisons">S4</a> (Prescription only)<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><small><abbr class="country-name" title="Canada">CA</abbr></small>: <a href="/wiki/Prescription_drug" title="Prescription drug"> ℞-only</a><sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></li> <li><small><abbr class="country-name" title="United Kingdom">UK</abbr>:</small> <a href="/wiki/Prescription_drug" title="Prescription drug">POM</a> (Prescription only)</li> <li><small><abbr class="country-name" title="United States">US</abbr>:</small> <a href="/wiki/Prescription_drug" title="Prescription drug">℞-only</a><sup id="cite_ref-Kalydeco_label_4-0" class="reference"><a href="#cite_note-Kalydeco_label-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><small><abbr class="country-name" title="European Union">EU</abbr>:</small> Rx-only<sup id="cite_ref-Kalydeco_EPAR_5-0" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li>In general: ℞ (Prescription only)</li></ul></div> </td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;"><a href="/wiki/Pharmacokinetics" title="Pharmacokinetics">Pharmacokinetic</a> data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Plasma_protein_binding" title="Plasma protein binding">Protein binding</a></th><td class="infobox-data">99%</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Drug_metabolism" title="Drug metabolism">Metabolism</a></th><td class="infobox-data">CYP3A</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Biological_half-life" title="Biological half-life">Elimination <span class="nowrap">half-life</span></a></th><td class="infobox-data">12 hrs (single dose)</td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Excretion" title="Excretion">Excretion</a></th><td class="infobox-data">88% faeces</td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Identifiers</th></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/IUPAC_nomenclature_of_chemistry" title="IUPAC nomenclature of chemistry">IUPAC name</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all; text-align:left; padding-left:1.5em; text-indent:-1.5em;"><li style="line-height: inherit; margin: 0"><div style="font-size: 97%;"><i>N</i>-(2,4-Di-<i>tert</i>-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide</div></li></ul> </div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/CAS_Registry_Number" title="CAS Registry Number">CAS Number</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="commonchemistry.cas.org"><a rel="nofollow" class="external text" href="https://commonchemistry.cas.org/detail?cas_rn=873054-44-5">873054-44-5</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/PubChem#CID" title="PubChem">PubChem</a> <span style="font-weight:normal"><abbr title="Compound ID">CID</abbr></span></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="pubchem.ncbi.nlm.nih.gov"><a rel="nofollow" class="external text" href="https://pubchem.ncbi.nlm.nih.gov/compound/16220172">16220172</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Guide_to_Pharmacology" title="Guide to Pharmacology">IUPHAR/BPS</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.guidetopharmacology.org"><a rel="nofollow" class="external text" href="https://www.guidetopharmacology.org/GRAC/LigandDisplayForward?ligandId=4342">4342</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/DrugBank" title="DrugBank">DrugBank</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.drugbank.ca"><a rel="nofollow" class="external text" href="https://www.drugbank.ca/drugs/DB08820">DB08820</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChemSpider" title="ChemSpider">ChemSpider</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.chemspider.com"><a rel="nofollow" class="external text" href="https://www.chemspider.com/Chemical-Structure.17347474.html">17347474</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Unique_Ingredient_Identifier" title="Unique Ingredient Identifier">UNII</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="precision.fda.gov"><a rel="nofollow" class="external text" href="https://precision.fda.gov/uniisearch/srs/unii/1Y740ILL1Z">1Y740ILL1Z</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/KEGG" title="KEGG">KEGG</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.kegg.jp"><a rel="nofollow" class="external text" href="https://www.kegg.jp/entry/D09916">D09916</a></span><sup> <span typeof="mw:File"><span><img alt="☒" src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/7px-X_mark.svg.png" decoding="async" width="7" height="8" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/11px-X_mark.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/14px-X_mark.svg.png 2x" data-file-width="525" data-file-height="600" /></span></span><span style="display:none">N</span></sup></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChEBI" title="ChEBI">ChEBI</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:66901">CHEBI:66901</a></span><sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ChEMBL" title="ChEMBL">ChEMBL</a></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="www.ebi.ac.uk"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/chembl/explore/compound/ChEMBL2010601">ChEMBL2010601</a></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/CompTox_Chemicals_Dashboard" title="CompTox Chemicals Dashboard">CompTox Dashboard</a> <span style="font-weight:normal">(<abbr title="U.S. Environmental Protection Agency">EPA</abbr>)</span></th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="comptox.epa.gov"><a rel="nofollow" class="external text" href="https://comptox.epa.gov/dashboard/chemical/details/DTXSID00236281">DTXSID00236281</a> <span class="mw-valign-text-top noprint" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q6095693#P3117" title="Edit this at Wikidata"><img alt="Edit this at Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></li></ul></div></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/ECHA_InfoCard" class="mw-redirect" title="ECHA InfoCard"><span title="echa.europa.eu">ECHA InfoCard</span></a></th><td class="infobox-data"><a rel="nofollow" class="external text" href="https://echa.europa.eu/substance-information/-/substanceinfo/100.226.211">100.226.211</a> <span class="mw-valign-text-top noprint" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q6095693#P2566" title="Edit this at Wikidata"><img alt="Edit this at Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></td></tr><tr><th colspan="2" class="infobox-header" style="background:#ddd;color:inherit;">Chemical and physical data</th></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Chemical_formula" title="Chemical formula">Formula</a></th><td class="infobox-data"><span title="Carbon">C</span><sub>24</sub><span title="Hydrogen">H</span><sub>28</sub><span title="Nitrogen">N</span><sub>2</sub><span title="Oxygen">O</span><sub>3</sub></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;"><a href="/wiki/Molar_mass" title="Molar mass">Molar mass</a></th><td class="infobox-data"><span class="nowrap"><span data-sort-value="7002392499000000000♠"></span>392.499</span> g·mol<sup>−1</sup></td></tr><tr><th scope="row" class="infobox-label" style="line-height:1.2em;">3D model (<a href="/wiki/JSmol" class="mw-redirect" title="JSmol">JSmol</a>)</th><td class="infobox-data"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1126788409" /><div class="plainlist"><ul><li><span title="chemapps.stolaf.edu (3D interactive model)"><a rel="nofollow" class="external text" href="https://chemapps.stolaf.edu/jmol/jmol.php?model=O%3DC%5C2c1c%28cccc1%29N%2FC%3DC%2F2C%28%3DO%29Nc3cc%28O%29c%28cc3C%28C%29%28C%29C%29C%28C%29%28C%29C">Interactive image</a></span></li></ul></div></td></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/Simplified_molecular-input_line-entry_system" class="mw-redirect" title="Simplified molecular-input line-entry system">SMILES</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all;"><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">O=C\2c1c(cccc1)N/C=C/2C(=O)Nc3cc(O)c(cc3C(C)(C)C)C(C)(C)C</div></li></ul> </div></td></tr><tr><td colspan="2" class="infobox-full-data"><div class="collapsible-list mw-collapsible mw-collapsed" style="text-align: left;"> <div style="line-height: 1.6em; font-weight: bold;"><div><a href="/wiki/International_Chemical_Identifier" title="International Chemical Identifier">InChI</a></div></div> <ul class="mw-collapsible-content" style="margin-top: 0; margin-bottom: 0; line-height: inherit; list-style: none; margin-left: 0; word-break:break-all;"><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">InChI=1S/C24H28N2O3/c1-23(2,3)16-11-17(24(4,5)6)20(27)12-19(16)26-22(29)15-13-25-18-10-8-7-9-14(18)21(15)28/h7-13,27H,1-6H3,(H,25,28)(H,26,29)<sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></div></li><li style="line-height: inherit; margin: 0"><div style="word-wrap:break-word; text-indent:-1.5em; text-align:left; padding-left:1.5em; font-size:97%; line-height:120%;">Key:PURKAOJPTOLRMP-UHFFFAOYSA-N<sup> <span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup></div></li></ul> </div></td></tr><tr><td colspan="2" class="infobox-below"><style data-mw-deduplicate="TemplateStyles:r886047488">.mw-parser-output .nobold{font-weight:normal}</style><span class="nobold"> <sup><span typeof="mw:File"><span><img alt="☒" src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/7px-X_mark.svg.png" decoding="async" width="7" height="8" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/11px-X_mark.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a2/X_mark.svg/14px-X_mark.svg.png 2x" data-file-width="525" data-file-height="600" /></span></span><span style="display:none">N</span><span typeof="mw:File"><span><img alt="check" src="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/7px-Yes_check.svg.png" decoding="async" width="7" height="7" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/11px-Yes_check.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/f/fb/Yes_check.svg/14px-Yes_check.svg.png 2x" data-file-width="600" data-file-height="600" /></span></span><span style="display:none">Y</span></sup> <a href="/wiki/Wikipedia:WikiProject_Chemicals/Chembox_validation" title="Wikipedia:WikiProject Chemicals/Chembox validation">(what is this?)</a></span><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r886047488" /><span class="nobold">  <span class="reflink plainlinks nourlexpansion"><a class="external text" href="https://en.wikipedia.org/w/index.php?title=Special:ComparePages&rev1=669804571&page2=Ivacaftor">(verify)</a></span></span></td></tr></tbody></table> <p><b>Ivacaftor</b> is a <a href="/wiki/Medication" title="Medication">medication</a> used to treat <a href="/wiki/Cystic_fibrosis" title="Cystic fibrosis">cystic fibrosis</a> in people with certain mutations in the <a href="/wiki/Cystic_fibrosis_transmembrane_conductance_regulator" title="Cystic fibrosis transmembrane conductance regulator">cystic fibrosis transmembrane conductance regulator</a> (CFTR) gene (primarily the G551D mutation), who account for 4–5% cases of cystic fibrosis.<sup id="cite_ref-pmid19747007_6-0" class="reference"><a href="#cite_note-pmid19747007-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid23616952_7-0" class="reference"><a href="#cite_note-pmid23616952-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> It is also included in <a href="/wiki/Combination_medication" class="mw-redirect" title="Combination medication">combination medications</a>, <a href="/wiki/Lumacaftor/ivacaftor" title="Lumacaftor/ivacaftor">lumacaftor/ivacaftor</a>, <a href="/wiki/Tezacaftor/ivacaftor" class="mw-redirect" title="Tezacaftor/ivacaftor">tezacaftor/ivacaftor</a>, and <a href="/wiki/Elexacaftor/tezacaftor/ivacaftor" title="Elexacaftor/tezacaftor/ivacaftor">elexacaftor/tezacaftor/ivacaftor</a> which are used to treat people with cystic fibrosis.<sup id="cite_ref-Orkambi_Label_8-0" class="reference"><a href="#cite_note-Orkambi_Label-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Symdeko_label_9-0" class="reference"><a href="#cite_note-Symdeko_label-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Symkevi_EPAR_10-0" class="reference"><a href="#cite_note-Symkevi_EPAR-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p><p>Ivacaftor was developed by <a href="/wiki/Vertex_Pharmaceuticals" title="Vertex Pharmaceuticals">Vertex Pharmaceuticals</a> in conjunction with the <a href="/wiki/Cystic_Fibrosis_Foundation" title="Cystic Fibrosis Foundation">Cystic Fibrosis Foundation</a> and is the first medication that treats the underlying cause rather than the symptoms of the disease. It was approved by the U.S. <a href="/wiki/Food_and_Drug_Administration" title="Food and Drug Administration">Food and Drug Administration</a> (FDA) in January 2012. It is one of the most expensive drugs, costing over <span style="white-space: nowrap">US$300,000</span> per year, which has led to criticism of the high cost. The combination drug lumacaftor/ivacaftor was approved by the FDA in July 2015. </p><p>Cystic fibrosis is caused by any one of several defects in the CFTR protein, which regulates fluid flow within cells and affects the components of sweat, digestive fluids, and mucus. One such defect is the G551D mutation, in which the amino acid <a href="/wiki/Glycine" title="Glycine">glycine</a> (G) in position 551 is replaced with <a href="/wiki/Aspartic_acid" title="Aspartic acid">aspartic acid</a> (D). G551D is characterized by a dysfunctional CFTR protein on the cell surface. In the case of G551D, the protein is trafficked to the correct area, the epithelial cell surface, but once there the protein cannot transport chloride through the channel. Ivacaftor, a CFTR <a href="/wiki/Potentiator" title="Potentiator">potentiator</a>, improves the transport of chloride through the ion channel by binding to the channels directly to induce a non-conventional mode of gating which in turn increases the probability that the channel is open.<sup id="cite_ref-pmid22942289_11-0" class="reference"><a href="#cite_note-pmid22942289-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid19846789_12-0" class="reference"><a href="#cite_note-pmid19846789-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid20829696_13-0" class="reference"><a href="#cite_note-pmid20829696-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> </p> <style data-mw-deduplicate="TemplateStyles:r886046785">.mw-parser-output .toclimit-2 .toclevel-1 ul,.mw-parser-output .toclimit-3 .toclevel-2 ul,.mw-parser-output .toclimit-4 .toclevel-3 ul,.mw-parser-output .toclimit-5 .toclevel-4 ul,.mw-parser-output .toclimit-6 .toclevel-5 ul,.mw-parser-output .toclimit-7 .toclevel-6 ul{display:none}</style><div class="toclimit-3"><meta property="mw:PageProp/toc" /></div> <div class="mw-heading mw-heading2"><h2 id="Medical_uses">Medical uses</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=1" title="Edit section: Medical uses"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ivacaftor is used for the treatment of <a href="/wiki/Cystic_fibrosis" title="Cystic fibrosis">cystic fibrosis</a> in people having one of several specific mutations in the <a href="/wiki/Cystic_fibrosis_transmembrane_conductance_regulator" title="Cystic fibrosis transmembrane conductance regulator">cystic fibrosis transmembrane conductance regulator</a> (CFTR) protein: E56K, G178R, S549R, K1060T, G1244E, P67L, E193K, G551D, A1067T, S1251N, R74W, L206W, G551S, G1069R, S1255P, D110E, R347H, D579G, R1070Q, D1270N, D110H, R352Q, S945L, R1070W, G1349D, R117C, A455E, S977F, F1074L, R117H, S549N, F1052V, D1152H.<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_label_4-1" class="reference"><a href="#cite_note-Kalydeco_label-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </p><p>Ivacaftor is also included in a combination product, <a href="/wiki/Lumacaftor/ivacaftor" title="Lumacaftor/ivacaftor">lumacaftor/ivacaftor</a>, in a single pill, which is used to treat people with <a href="/wiki/Cystic_fibrosis" title="Cystic fibrosis">cystic fibrosis</a> who have the <a href="/wiki/%CE%94F508" class="mw-redirect" title="ΔF508">F508del</a> mutation in CFTR.<sup id="cite_ref-2015revKuk_16-0" class="reference"><a href="#cite_note-2015revKuk-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-CW_17-0" class="reference"><a href="#cite_note-CW-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Orkambi_Label_8-1" class="reference"><a href="#cite_note-Orkambi_Label-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Orkambi_Snapshot_18-0" class="reference"><a href="#cite_note-Orkambi_Snapshot-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> </p><p>Ivacaftor is also included in the combination product <a href="/wiki/Tezacaftor" title="Tezacaftor">tezacaftor</a>/ivacaftor with ivacaftor sold as Symdeko and as Symkevi.<sup id="cite_ref-Symdeko_label_9-1" class="reference"><a href="#cite_note-Symdeko_label-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Symdeko_Snapshot_19-0" class="reference"><a href="#cite_note-Symdeko_Snapshot-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-21" class="reference"><a href="#cite_note-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Symkevi_EPAR_10-1" class="reference"><a href="#cite_note-Symkevi_EPAR-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p><p>Symdeko is indicated to treat people aged six and older who have two copies of the F508del mutation in CFTR.<sup id="cite_ref-Symdeko_label_9-2" class="reference"><a href="#cite_note-Symdeko_label-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Symdeko_Snapshot_19-1" class="reference"><a href="#cite_note-Symdeko_Snapshot-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-FDA_Symdeko_PR_22-0" class="reference"><a href="#cite_note-FDA_Symdeko_PR-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> </p><p>Symkevi is indicated in a combination regimen with ivacaftor 150 mg tablets for the treatment of people with cystic fibrosis (CF) aged twelve years and older who are homozygous for the F508del mutation or who are heterozygous for the F508del mutation and have one of the following mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: P67L, R117C, L206W, R352Q, A455E, D579G, 711+3A→G, S945L, S977F, R1070W, D1152H, 2789+5G→A, 3272 26A→G, and 3849+10kbC→T.<sup id="cite_ref-Symkevi_EPAR_10-2" class="reference"><a href="#cite_note-Symkevi_EPAR-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p><p>Ivacaftor is available in a combination product with <a href="/wiki/Elexacaftor" title="Elexacaftor">elexacaftor</a> and <a href="/wiki/Tezacaftor" title="Tezacaftor">tezacaftor</a> called <a href="/wiki/Trikafta" class="mw-redirect" title="Trikafta">Trikafta</a> for the treatment of people with cystic fibrosis who have the <a href="/wiki/F508del" class="mw-redirect" title="F508del">F508del</a> mutation or other mutations.<sup id="cite_ref-Trikafta_FDA_label_23-0" class="reference"><a href="#cite_note-Trikafta_FDA_label-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Adverse_effects">Adverse effects</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=2" title="Edit section: Adverse effects"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The most common adverse reactions experienced by patients who received ivacaftor in the pooled placebo-controlled Phase III studies were abdominal pain (15.6% versus 12.5% on placebo), <a href="/wiki/Diarrhea" title="Diarrhea">diarrhoea</a> (12.8% versus 9.6% on placebo), dizziness (9.2% versus 1.0% on placebo), rash (12.8% versus 6.7% on placebo), upper respiratory tract reactions (including upper respiratory tract infection, nasal congestion, pharyngeal erythema, oropharyngeal pain, rhinitis, sinus congestion, and nasopharyngitis) (63.3% versus 50.0% on placebo), headache (23.9% versus 16.3% on placebo) and bacteria in sputum (7.3% versus 3.8% on placebo). One patient in the ivacaftor group reported a serious adverse reaction: abdominal pain.<sup id="cite_ref-url_EMA_Kalydeco_24-0" class="reference"><a href="#cite_note-url_EMA_Kalydeco-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_EPAR_5-1" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Pharmacology">Pharmacology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=3" title="Edit section: Pharmacology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Pharmacodynamics">Pharmacodynamics</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=4" title="Edit section: Pharmacodynamics"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ivacaftor is a "potentiator" of CFTR, meaning it increases the probability that the defective channel will be open and allow chloride ions pass through the channel pore.<sup id="cite_ref-2015revKuk_16-1" class="reference"><a href="#cite_note-2015revKuk-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Pharmacokinetics">Pharmacokinetics</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=5" title="Edit section: Pharmacokinetics"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading4"><h4 id="Distribution">Distribution</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=6" title="Edit section: Distribution"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ivacaftor is approximately 99% bound to plasma proteins, primarily to <a href="/wiki/Orosomucoid" title="Orosomucoid">alpha 1-acid glycoprotein</a> and <a href="/wiki/Albumin" title="Albumin">albumin</a>. Ivacaftor does not bind to human red blood cells.<sup id="cite_ref-url_EMA_Kalydeco_24-1" class="reference"><a href="#cite_note-url_EMA_Kalydeco-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_EPAR_5-2" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading4"><h4 id="Biotransformation">Biotransformation</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=7" title="Edit section: Biotransformation"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ivacaftor is extensively metabolised in humans. <a href="/wiki/In_vitro" title="In vitro">In vitro</a> and <a href="/wiki/In_vivo" title="In vivo">in vivo</a> data indicate that ivacaftor is primarily metabolised by CYP3A. M1 and M6 are the two major metabolites of ivacaftor in humans. M1 has approximately one-sixth the potency of ivacaftor and is considered pharmacologically active. M6 has less than one-fiftieth the potency of ivacaftor and is not considered pharmacologically active.<sup id="cite_ref-url_EMA_Kalydeco_24-2" class="reference"><a href="#cite_note-url_EMA_Kalydeco-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_EPAR_5-3" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading4"><h4 id="Elimination">Elimination</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=8" title="Edit section: Elimination"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Following oral administration, the majority of ivacaftor (87.8%) is eliminated in the faeces after metabolic conversion. The major metabolites M1 and M6 accounted for approximately 65% of total dose eliminated with 22% as M1 and 43% as M6. There was negligible urinary excretion of ivacaftor as unchanged parent. The apparent terminal half-life was approximately 12 hours following a single dose in the fed state. The apparent clearance (CL/F) of ivacaftor was similar for healthy subjects and patients with CF. The mean (±SD) of CL/F for the 150 mg dose was 17.3 (8.4) L/h in healthy subjects at steady state.<sup id="cite_ref-url_EMA_Kalydeco_24-3" class="reference"><a href="#cite_note-url_EMA_Kalydeco-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_EPAR_5-4" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=9" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ivacaftor was developed by <a href="/wiki/Vertex_Pharmaceuticals" title="Vertex Pharmaceuticals">Vertex Pharmaceuticals</a> in conjunction with the <a href="/wiki/Cystic_Fibrosis_Foundation" title="Cystic Fibrosis Foundation">Cystic Fibrosis Foundation</a> and is the first medication that treats the underlying cause rather than the symptoms of the disease.<sup id="cite_ref-prnewswire_25-0" class="reference"><a href="#cite_note-prnewswire-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Society_and_culture">Society and culture</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=10" title="Edit section: Society and culture"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Legal_status">Legal status</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=11" title="Edit section: Legal status"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The U.S. <a href="/wiki/Food_and_Drug_Administration" title="Food and Drug Administration">Food and Drug Administration</a> (FDA) approved ivacaftor in January 2012,<sup id="cite_ref-26" class="reference"><a href="#cite_note-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> <sup id="cite_ref-27" class="reference"><a href="#cite_note-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-28" class="reference"><a href="#cite_note-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-29" class="reference"><a href="#cite_note-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p><p>Soon afterwards so too did the <a href="/wiki/European_Medicines_Agency" title="European Medicines Agency">European Medicines Agency</a> (EMA)<sup id="cite_ref-url_EMA_Kalydeco_24-4" class="reference"><a href="#cite_note-url_EMA_Kalydeco-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Kalydeco_EPAR_5-5" class="reference"><a href="#cite_note-Kalydeco_EPAR-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> and Canada<sup id="cite_ref-30" class="reference"><a href="#cite_note-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> and across some European countries.<sup id="cite_ref-31" class="reference"><a href="#cite_note-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-32" class="reference"><a href="#cite_note-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-33" class="reference"><a href="#cite_note-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> </p><p>Lumacaftor/ivacaftor was approved by the FDA in July 2015, under <a href="/wiki/Breakthrough_therapy" title="Breakthrough therapy">breakthrough therapy</a> status and under a <a href="/wiki/Priority_review_(FDA)" class="mw-redirect" title="Priority review (FDA)">priority review</a>.<sup id="cite_ref-34" class="reference"><a href="#cite_note-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-35" class="reference"><a href="#cite_note-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Economics">Economics</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=12" title="Edit section: Economics"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>It is one of the most expensive drugs, costing over <span style="white-space: nowrap">US$300,000</span> per year, which has led to criticism of the high cost. The cost of ivacaftor is <span style="white-space: nowrap">US$311,000</span> per year, roughly similar to the price of other medications for extremely rare diseases.<sup id="cite_ref-36" class="reference"><a href="#cite_note-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> In the first nine months of its second year on the market (2014), ivacaftor sales were $339M, representing 54% of Vertex's product sales revenue. During the same period, total drug development expenses were $458M, most of which was spent on cystic fibrosis-related research.<sup id="cite_ref-37" class="reference"><a href="#cite_note-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> </p><p>An editorial in JAMA called the price of ivacaftor "exorbitant", citing the support by the <a href="/wiki/Cystic_Fibrosis_Foundation" title="Cystic Fibrosis Foundation">Cystic Fibrosis Foundation</a> in its development and the contribution made by fundamental scientific research performed by the <a href="/wiki/National_Institutes_of_Health" title="National Institutes of Health">National Institutes of Health</a> and relied upon by Vertex in its cystic fibrosis drug discovery programs.<sup id="cite_ref-exorbitant_38-0" class="reference"><a href="#cite_note-exorbitant-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> The company responded in an email that "while publicly funded academic research provided important early understanding of the cause of cystic fibrosis, it took Vertex scientists 14 years of their own research, funded mostly by the company, before the drug won approval."<sup id="cite_ref-39" class="reference"><a href="#cite_note-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup> </p><p>The Cystic Fibrosis Foundation, a non-profit organization dedicated to improving healthcare for people with cystic fibrosis, provided $150 million of the funding for the development for ivacaftor in exchange for royalty rights in the event that the medication was successfully developed and commercialized. In 2014, the Foundation sold these royalty rights for $3.3 billion. The Foundation has stated that it intends to spend these funds in support of further research.<sup id="cite_ref-40" class="reference"><a href="#cite_note-40"><span class="cite-bracket">[</span>40<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-41" class="reference"><a href="#cite_note-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </p><p>Vertex said it would make the medication available free to patients in the United States with no insurance and a household income of under $150,000.<sup id="cite_ref-42" class="reference"><a href="#cite_note-42"><span class="cite-bracket">[</span>42<span class="cite-bracket">]</span></a></sup> In 2012, 24 US doctors and researchers involved in the development of the medication wrote to Vertex to protest the price of the medication, which had been set at about $300,000 per year. In the UK, the company provided the medication free for a limited time for certain patients, then left the hospitals to decide whether to continue to pay for it for those patients. UK agencies estimated the cost per quality adjusted life year (<a href="/wiki/QALY" class="mw-redirect" title="QALY">QALY</a>) at between £335,000 and £1,274,000 —well above the <a href="/wiki/National_Institute_for_Health_and_Care_Excellence" title="National Institute for Health and Care Excellence">National Institute for Health and Care Excellence</a> thresholds.<sup id="cite_ref-BMJ-2014_43-0" class="reference"><a href="#cite_note-BMJ-2014-43"><span class="cite-bracket">[</span>43<span class="cite-bracket">]</span></a></sup> </p><p>The medication was not covered under the <a href="/w/index.php?title=Ontario_Drug_Benefit&action=edit&redlink=1" class="new" title="Ontario Drug Benefit (page does not exist)">Ontario Drug Benefit</a> plan until June 2014, when the <a href="/wiki/Government_of_Ontario" title="Government of Ontario">Government of Ontario</a> and the manufacturer negotiated for what "Ontario Health Minister <a href="/wiki/Deb_Matthews" title="Deb Matthews">Deb Matthews</a> had called a "fair price" for taxpayers". The negotiations took 16 months and it was estimated that around 20 Ontarians required the medication at the time.<sup id="cite_ref-44" class="reference"><a href="#cite_note-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> </p><p>The province of <a href="/wiki/Alberta" title="Alberta">Alberta</a> began covering the medication in July 2014, and in September the province of <a href="/wiki/Saskatchewan" title="Saskatchewan">Saskatchewan</a> became the third province to include it in its provincial medication plan.<sup id="cite_ref-45" class="reference"><a href="#cite_note-45"><span class="cite-bracket">[</span>45<span class="cite-bracket">]</span></a></sup> </p><p>Delay in agreement on a price for Vertex to charge national health plans led to patient group protests in Wales,<sup id="cite_ref-46" class="reference"><a href="#cite_note-46"><span class="cite-bracket">[</span>46<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-47" class="reference"><a href="#cite_note-47"><span class="cite-bracket">[</span>47<span class="cite-bracket">]</span></a></sup> England,<sup id="cite_ref-48" class="reference"><a href="#cite_note-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> and Australia.<sup id="cite_ref-49" class="reference"><a href="#cite_note-49"><span class="cite-bracket">[</span>49<span class="cite-bracket">]</span></a></sup> </p><p>As of March 2016<sup class="plainlinks noexcerpt noprint asof-tag update" style="display:none;"><a class="external text" href="https://en.wikipedia.org/w/index.php?title=Ivacaftor&action=edit">[update]</a></sup>, <span class="clarify-content" style="padding-left:0.1em; padding-right:0.1em; color:var(--color-subtle, #54595d); border:1px solid var(--border-color-subtle, #c8ccd1);">the combination medication Orkambi</span><sup class="noprint Inline-Template Template-Clarify" style="margin-left:0.1em; white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Please_clarify" title="Wikipedia:Please clarify"><span title="which combination drug? (March 2024)">clarify</span></a></i>]</sup> cost $259,000 a year in the United States.<sup id="cite_ref-FierceNICE_50-0" class="reference"><a href="#cite_note-FierceNICE-50"><span class="cite-bracket">[</span>50<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Research">Research</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=13" title="Edit section: Research"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The clinical trials used in the regulatory approval of ivacaftor are described here. </p> <div class="mw-heading mw-heading3"><h3 id="G551D_mutation">G551D mutation</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=14" title="Edit section: G551D mutation"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Of the approximately 70,000 cases of cystic fibrosis worldwide, 4% (~3,000) are due to a mutation called G551D.<sup id="cite_ref-51" class="reference"><a href="#cite_note-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-52" class="reference"><a href="#cite_note-52"><span class="cite-bracket">[</span>52<span class="cite-bracket">]</span></a></sup> The safety and efficacy of ivacaftor for the treatment of cystic fibrosis in patients with this mutation was examined in two clinical trials.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (November 2019)">citation needed</span></a></i>]</sup> </p><p>The first trial was performed in adults having baseline respiratory function (FEV1) between 32% and 98% of normal for persons of similar age, height, and weight. The baseline average was 64%. Improvement in FEV1 was rapid and sustained. At the end of 48 weeks, people treated with ivacaftor had on average an absolute increase in FEV1 of 10.4%, vs. a decline of 0.2% in the placebo group. Pulmonary exacerbations were reduced by about half in the ivacaftor group relative to the placebo group.<sup id="cite_ref-Kalydeco_label_4-2" class="reference"><a href="#cite_note-Kalydeco_label-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>In a second trial conducted in children age six to 11, the average improvement in FEV1 was an absolute increase of 12.5% in the ivacaftor group at 48 weeks, compared to a very slight decline in the placebo group.<sup id="cite_ref-Kalydeco_label_4-3" class="reference"><a href="#cite_note-Kalydeco_label-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Other_mutations">Other mutations</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=15" title="Edit section: Other mutations"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>A third clinical trial examined the efficacy of ivacaftor in people with cystic fibrosis due to G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R mutations. This trial, which included 39 people of age greater than six years, used a crossover design. The people in the trial had FEV1 averaging 78% of normal at baseline. The people in the trial were randomized to receive either ivacaftor or placebo for eight weeks. This was followed by a four to eight week washout period, then each group received the opposite treatment from what it received in the first part of the trial. At week 8, the people on treatment with ivacaftor experienced an average absolute improvement in FEV1 of 13.8%, but there was a strong dependence of the efficacy on the exact mutation that a patient had. The detailed data for different mutation types is shown in the U.S. package insert.<sup id="cite_ref-Kalydeco_label_4-4" class="reference"><a href="#cite_note-Kalydeco_label-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=16" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-Drugs.com_pregnancy-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-Drugs.com_pregnancy_1-0">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://www.drugs.com/pregnancy/ivacaftor.html">"Ivacaftor (Kalydeco) Use During Pregnancy"</a>. <i>Drugs.com</i>. 9 November 2019<span class="reference-accessdate">. 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"Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening". <i>Human Mutation</i>. <b>19</b> (6): <span class="nowrap">575–</span>606. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1002%2Fhumu.10041">10.1002/humu.10041</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/12007216">12007216</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a> <a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:35428054">35428054</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Human+Mutation&rft.atitle=Cystic+fibrosis%3A+a+worldwide+analysis+of+CFTR+mutations--correlation+with+incidence+data+and+application+to+screening&rft.volume=19&rft.issue=6&rft.pages=%3Cspan+class%3D%22nowrap%22%3E575-%3C%2Fspan%3E606&rft.date=2002-06&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A35428054%23id-name%3DS2CID&rft_id=info%3Apmid%2F12007216&rft_id=info%3Adoi%2F10.1002%2Fhumu.10041&rft.aulast=Bobadilla&rft.aufirst=JL&rft.au=Macek%2C+M&rft.au=Fine%2C+JP&rft.au=Farrell%2C+PM&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Ivacaftor&action=edit&section=17" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://druginfo.nlm.nih.gov/drugportal/name/ivacaftor">"Ivacaftor"</a>. <i>Drug Information Portal</i>. U.S. National Library of Medicine.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Drug+Information+Portal&rft.atitle=Ivacaftor&rft_id=https%3A%2F%2Fdruginfo.nlm.nih.gov%2Fdrugportal%2Fname%2Fivacaftor&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://druginfo.nlm.nih.gov/drugportal/name/ivacaftor%20mixture%20with%20lumacaftor">"Ivacaftor mixture with lumacaftor"</a>. <i>Drug Information Portal</i>. U.S. National Library of Medicine.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Drug+Information+Portal&rft.atitle=Ivacaftor+mixture+with+lumacaftor&rft_id=https%3A%2F%2Fdruginfo.nlm.nih.gov%2Fdrugportal%2Fname%2Fivacaftor%2520mixture%2520with%2520lumacaftor&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://druginfo.nlm.nih.gov/drugportal/name/ivacaftor%20regimen%20with%20tezacaftor">"Ivacaftor regimen with Tezacaftor"</a>. <i>Drug Information Portal</i>. U.S. National Library of Medicine.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Drug+Information+Portal&rft.atitle=Ivacaftor+regimen+with+Tezacaftor&rft_id=https%3A%2F%2Fdruginfo.nlm.nih.gov%2Fdrugportal%2Fname%2Fivacaftor%2520regimen%2520with%2520tezacaftor&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://medlineplus.gov/druginfo/meds/a615037.html">"Lumacaftor and Ivacaftor"</a>. <i>MedlinePlus</i>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=MedlinePlus&rft.atitle=Lumacaftor+and+Ivacaftor&rft_id=https%3A%2F%2Fmedlineplus.gov%2Fdruginfo%2Fmeds%2Fa615037.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://medlineplus.gov/druginfo/meds/a618022.html">"Tezacaftor and Ivacaftor"</a>. <i>MedlinePlus</i>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=MedlinePlus&rft.atitle=Tezacaftor+and+Ivacaftor&rft_id=https%3A%2F%2Fmedlineplus.gov%2Fdruginfo%2Fmeds%2Fa618022.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222" /><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://medlineplus.gov/druginfo/meds/a619061.html">"Elexacaftor, Tezacaftor, and Ivacaftor"</a>. <i>MedlinePlus</i>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=MedlinePlus&rft.atitle=Elexacaftor%2C+Tezacaftor%2C+and+Ivacaftor&rft_id=https%3A%2F%2Fmedlineplus.gov%2Fdruginfo%2Fmeds%2Fa619061.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3AIvacaftor" class="Z3988"></span></li></ul> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist ul{margin:0;padding:0}.mw-parser-output .hlist dd,.mw-parser-output .hlist dt,.mw-parser-output .hlist li{margin:0;display:inline}.mw-parser-output .hlist.inline,.mw-parser-output .hlist.inline dl,.mw-parser-output .hlist.inline ol,.mw-parser-output .hlist.inline ul,.mw-parser-output .hlist dl dl,.mw-parser-output .hlist dl ol,.mw-parser-output .hlist dl ul,.mw-parser-output .hlist ol dl,.mw-parser-output .hlist ol ol,.mw-parser-output .hlist ol ul,.mw-parser-output .hlist ul dl,.mw-parser-output .hlist ul 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aria-labelledby="Other_respiratory_system_products_(R07)198" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374" /><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output 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</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Respiratory stimulants</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Almitrine" title="Almitrine">Almitrine</a></li> <li><a href="/wiki/Amiphenazole" title="Amiphenazole">Amiphenazole</a></li> <li><a href="/wiki/Bemegride" title="Bemegride">Bemegride</a></li> <li><a href="/wiki/Dimefline" title="Dimefline">Dimefline</a></li> <li><a href="/wiki/Doxapram" title="Doxapram">Doxapram</a></li> <li><a href="/wiki/Etamivan" title="Etamivan">Etamivan</a></li> <li><a href="/wiki/GAL-021" class="mw-redirect" title="GAL-021">GAL-021</a></li> <li><a href="/wiki/Mepixanox" title="Mepixanox">Mepixanox</a></li> <li><a href="/wiki/Nikethamide" title="Nikethamide">Nikethamide</a></li> <li><a href="/wiki/Pentylenetetrazol" title="Pentylenetetrazol">Pentetrazol</a></li> <li><a href="/wiki/Prethcamide" title="Prethcamide">Prethcamide</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/5-HT4_receptor" title="5-HT4 receptor">5-HT4 receptor</a> agonists</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/BIMU8" title="BIMU8">BIMU-8</a></li> <li><a href="/wiki/Zacopride" title="Zacopride">Zacopride</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other agents for treating respiratory depression</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a class="mw-selflink selflink">Ivacaftor</a> (+ <a href="/wiki/Lumacaftor/ivacaftor" title="Lumacaftor/ivacaftor">lumacaftor</a>, + <a href="/wiki/Ivacaftor/tezacaftor" class="mw-redirect" title="Ivacaftor/tezacaftor">tezacaftor</a>, + <a href="/wiki/Elexacaftor/tezacaftor/ivacaftor" title="Elexacaftor/tezacaftor/ivacaftor">elexacaftor/tezacaftor</a>)</li> <li><a href="/wiki/Nitric_oxide" title="Nitric oxide">Nitric oxide</a></li> <li><a href="/wiki/Vanzacaftor/tezacaftor/deutivacaftor" title="Vanzacaftor/tezacaftor/deutivacaftor">Vanzacaftor/tezacaftor/deutivacaftor</a></li> <li><a href="/wiki/BW373U86" title="BW373U86">BW373U86</a></li> <li><a href="/wiki/CX-546" title="CX-546">CX-546</a></li> <li><a href="/wiki/CX717" title="CX717">CX-717</a></li></ul> </div></td></tr></tbody></table></div> <style data-mw-deduplicate="TemplateStyles:r1130092004">.mw-parser-output .portal-bar{font-size:88%;font-weight:bold;display:flex;justify-content:center;align-items:baseline}.mw-parser-output .portal-bar-bordered{padding:0 2em;background-color:#fdfdfd;border:1px solid #a2a9b1;clear:both;margin:1em auto 0}.mw-parser-output .portal-bar-related{font-size:100%;justify-content:flex-start}.mw-parser-output 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