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Ivacaftor is a medication used to treat cystic fibrosis in people with certain mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (primarily the G551D mutation), who account for 4–5% cases of cystic fibrosis. It is also included in combination medications, lumacaftor/ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor which are used to treat people with cystic fibrosis.
Read the full article on WikipediaCystic fibrosis Clinical criteria: Treatment Phase: Initial treatment - New patient (gating mutations) Patient must be assessed through a cystic fibrosis clinic/centre which is under the control of specialist respiratory physicians with experience and expertise in the management of cystic fibrosis. If attendance at such a unit is not possible because of geographical isolation, management (including prescribing) may be in consultation with such a unit, AND Patient must have G551D mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene on at least 1 allele; OR Patient must have other gating (class III) mutation in the CFTR gene on at least 1 allele, AND Patient must not receive more than 24 weeks of treatment under this restriction, AND The treatment must be given concomitantly with standard therapy for this condition. Population criteria: Patient must be aged 4 months or older. Dosage of ivacaftor must not exceed the dose of one tablet (150 mg) or one sachet twice a week, if the patient is concomitantly receiving one of the following strong CYP3A4 drugs inhibitors: boceprevir, clarithromycin, conivaptan, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, voriconazole. Where a patient is concomitantly receiving a strong CYP3A4 inhibitor, a single supply of 56 tablets or sachets of ivacaftor will last for 28 weeks. Dosage of ivacaftor must not exceed the dose of one tablet (150 mg) or one sachet once daily, if the patient is concomitantly receiving one of the following moderate CYP3A4 inhibitors: amprenavir, aprepitant, atazanavir, darunavir/ritonavir, diltiazem, erythromycin, fluconazole, fosamprenavir, imatinib, verapamil. Where a patient is concomitantly receiving a moderate CYP3A4 inhibitor, a single supply of 56 tablets or sachets of ivacaftor will last for 8 weeks. Ivacaftor is not PBS-subsidised for this condition as a sole therapy. Ivacaftor is not PBS-subsidised for this condition in a patient who is currently receiving one of the following CYP3A4 inducers: Strong CYP3A4 inducers: avasimibe, carbamazepine, phenobarbital, phenytoin, rifabutin, rifampicin, St. John's wort Moderate CYP3A4 inducers: bosentan, efavirenz, etravirine, modafinil, nafcillin Weak CYP3A4 inducers: armodafinil, echinacea, pioglitazone, rufinamide. The authority application must be made via the Online PBS Authorities System, or in writing via HPOS form upload or mail and must include: (1) details of the pathology report substantiating G551D mutation or other gating (Class III) mutation on the CFTR gene - quote each of the: (a) the specific CFTR mutation listed in the TGA-approved Product Information, (b) name of the pathology report provider, (c) date of pathology report, (d) unique identifying number/code that links the pathology result to the individual patient; and (2) current CYP3A4 inhibitors, CYP3A4 inducers and IV antibiotics. If the application is submitted through HPOS form upload or mail, it must include: (i) details of the proposed prescription; and (ii) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
“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.”
“12 hrs (single dose)”
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