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

Cystic fibrosis - one residual function (RF) mutation

Initial treatment

Must be treated by a specialist respiratory physician with expertise in cystic fibrosis or in consultation with a specialist respiratory physician with expertise in cystic fibrosis if attendance is not possible due to geographic isolation; AND

Must be treated in a centre with expertise in cystic fibrosis or in consultation with a centre with expertise in cystic fibrosis if attendance is not possible due to geographic isolation; AND

Patient must have at least one residual function (RF) mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to tezacaftor with ivacaftor; AND

The treatment must be the sole PBS-subsidised cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy for this condition; AND

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

Patient must have either chronic sinopulmonary disease or gastrointestinal and nutritional abnormalities; AND

Patient must be at least 12 years of age.

For the purposes of this restriction, the list of mutations considered to be responsive to tezacaftor with ivacaftor is defined in the TGA approved product information.

Dosage of tezacaftor with ivacaftor is tezacaftor 100 mg/ivacaftor 150 mg and ivacaftor 150 mg tablets on alternate days if the patient is concomitantly receiving one of the following moderate CYP3A4 drugs inhibitors: amprenavir, aprepitant, atazanavir, darunavir/ritonavir, diltiazem, erythromycin, fluconazole, fosamprenavir, imatinib, verapamil.

Dosage of tezacaftor with ivacaftor is tezacaftor 100 mg/ivacaftor 150 mg twice weekly (approximately 3 or 4 days apart) if the patient is concomitantly receiving one of the following strong CYP3A4 inhibitors: boceprevir, clarithromycin, conivaptan, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, voriconazole.

Tezacaftor with 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 in writing and must include:

(1) details of the proposed prescription; and

(2) a completed Cystic Fibrosis Authority Application Supporting Information Form; and

(3) details of the pathology report substantiating the patient having at least one RF mutation on the CFTR gene - quote each of the: (i) name of the pathology report provider, (ii) date of pathology report, (iii) unique identifying number/code that links the pathology result to the individual patient ; and

(4) CYP3A4 inhibitors, CYP3A4 inducers and IV antibiotics.

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

Cystic fibrosis - one residual function (RF) mutation

Continuing treatment

Must be treated by a specialist respiratory physician with expertise in cystic fibrosis or in consultation with a specialist respiratory physician with expertise in cystic fibrosis if attendance is not possible due to geographic isolation; AND

Must be treated in a centre with expertise in cystic fibrosis or in consultation with a centre with expertise in cystic fibrosis if attendance is not possible due to geographic isolation; AND

Patient must have previously received PBS-subsidised treatment with this drug for this condition; AND

The treatment must be the sole PBS-subsidised cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy for this condition; AND

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

Patient must be at least 12 years of age.

Dosage of tezacaftor with ivacaftor is tezacaftor 100 mg/ivacaftor 150 mg and ivacaftor 150 mg tablets on alternate days if the patient is concomitantly receiving one of the following moderate CYP3A4 drugs inhibitors: amprenavir, aprepitant, atazanavir, darunavir/ritonavir, diltiazem, erythromycin, fluconazole, fosamprenavir, imatinib, verapamil.

Dosage of tezacaftor with ivacaftor is tezacaftor 100 mg/ivacaftor 150 mg twice weekly (approximately 3 or 4 days apart) if the patient is concomitantly receiving one of the following strong CYP3A4 inhibitors: boceprevir, clarithromycin, conivaptan, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, voriconazole.

Tezacaftor with 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 in writing and must include:

(1) details of the proposed prescription; and

(2) a completed Cystic Fibrosis Continuing Authority Application Supporting Information Form; and

(3) current CYP3A4 inhibitors, CYP3A4 inducers and IV antibiotics.