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2 Information about ivacaftor–tezacaftor–elexacaftor, tezacaftor–ivacaftor and lumacaftor–ivacaftor

Marketing authorisations

2.1

Ivacaftor–tezacaftor–elexacaftor (IVA–TEZ–ELX, Kaftrio, Vertex) is indicated 'in a combination regimen with ivacaftor for the treatment of cystic fibrosis (CF) in patients aged 6 years and older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene'.

2.2

Tezacaftor–ivacaftor (TEZ–IVA, Symkevi, Vertex) is indicated 'in a combination regimen with ivacaftor tablets for the treatment of patients with cystic fibrosis (CF) aged 6 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'.

2.3

Lumacaftor–ivacaftor (LUM–IVA, Orkambi, Vertex) is indicated 'for the treatment of cystic fibrosis (CF) in patients aged 1 year and older who are homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene'.

Dosage in the marketing authorisation

Price

2.7

The list price for IVA–TEZ–ELX is £8,346.30 (excluding VAT; BNF online, accessed October 2023) per 56-tablet pack of:

  • IVA 37.5 mg, TEZ 25 mg and ELX 50 mg or

  • IVA 75 mg, TEZ 50 mg and ELX 100 mg.

2.8

The list price for TEZ–IVA is £6,293.91 (excluding VAT; BNF online, accessed October 2023) per 28-tablet pack of:

  • TEZ 50 mg and IVA 75 mg or

  • TEZ 100 mg and IVA 150 mg.

2.9

The list price for LUM–IVA is £8,000 (excluding VAT; BNF online, accessed October 2023) per 112-tablet pack of:

  • LUM 100 mg and IVA 125 mg or

  • LUM 200 mg and IVA 125 mg.

2.10

LUM–IVA is also available in 56-sachet packs. The list price is £8,000 (excluding VAT; BNFc online, accessed October 2023) per 56-sachet pack of:

  • LUM 75 mg and IVA 94 mg or

  • LUM 100 mg and IVA 125 mg or

  • LUM 150 mg and IVA 188 mg.

2.11

The list price for a 28-tablet pack of ivacaftor (IVA) 75 mg or IVA 150 mg is £7,000 (excluding VAT; BNF online accessed October 2023). IVA is also available in 28-sachet packs of 25 mg, 50 mg or 75 mg, but the price is commercial in confidence and cannot be reported here.

2.12

The company has a commercial arrangement, which would have applied if IVA–TEZ–ELX, TEZ–IVA or LUM–IVA had been recommended.