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

Cystic fibrosis - homozygous for the F508del 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 be homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene; 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.

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 being homozygous for the F508del 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) current CYP3A4 inhibitors, CYP3A4 inducers and IV antibiotics.

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

Cystic fibrosis - homozygous for the F508del 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.