Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

Cystic fibrosis

Initial treatment - New patient (non-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 at least one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data; AND

Patient must not have either: (i) G551D mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene; (ii) other gating (class III) mutation in the CFTR gene; 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; AND

Patient must be aged 4 months or older.

For the purposes of this restriction, the list of mutations considered to be responsive to ivacaftor is defined in the TGA approved Product Information.

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 the specific mutation considered to be responsive to ivacaftor as listed in the TGA-approved Product Information. Quote each of the: (a) the specific 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).

Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

Cystic fibrosis

Continuing treatment (non-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 received PBS-subsidised initial therapy with ivacaftor, given concomitantly with standard therapy, for this condition; AND

Patient must not receive more than 24 weeks of treatment under this restriction per authority application; AND

The treatment must be given concomitantly with standard therapy for this condition; AND

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 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).