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 2 years and older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene'.
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'.
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'.
IVA–TEZ–ELX is available as tablets or sachets. The dosage schedules are available in the following:
The dosage schedule for TEZ–IVA is available in the following:
LUM–IVA is available as tablets or sachets. The dosage schedules are available in the following:
The list price for IVA–TEZ–ELX tablets is £8,346.30 (excluding VAT; BNF online, accessed April 2024) 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.
The list price for IVA–TEZ–ELX sachets is £8,346.30 (excluding VAT; dictionary of medicines and devices, accessed April 2024) per 28‑sachet pack of:
IVA 60 mg, TEZ 40 mg and ELX 80 mg, or
IVA 75 mg, TEZ 50 mg and ELX 100 mg
The list price for TEZ–IVA is £6,293.91 (excluding VAT; BNF online, accessed April 2024) per 28‑tablet pack of:
TEZ 50 mg and IVA 75 mg, or
TEZ 100 mg and IVA 150 mg.
The list price for LUM–IVA tablets is £8,000 (excluding VAT; BNF online, accessed April 2024) per 112‑tablet pack of:
LUM 100 mg and IVA 125 mg, or
LUM 200 mg and IVA 125 mg.
The list price for LUM–IVA sachets is £8,000 (excluding VAT; dictionary of medicines and devices, accessed April 2024) 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.
The list price for IVA tablets is:
£7,000 (excluding VAT; BNF online, accessed April 2024) per 28‑tablet pack of:
IVA 75 mg, or
IVA 150 mg
£14,000 (excluding VAT; BNF online, accessed April 2024) per 56‑tablet pack of IVA 150 mg.
The list price for IVA sachets is:
£7,000 (excluding VAT; BNF for children online, accessed April 2024) per 28‑sachet pack of:
IVA 59.5 mg, or
IVA 75 mg
£14,000 (excluding VAT; BNF for children online, accessed April 2024) per 56‑sachet pack of:
IVA 25 mg, or
IVA 50 mg, or
IVA 75 mg.
The company has a commercial arrangement for IVA–TEZ–ELX in combination with IVA, TEZ–IVA in combination with IVA, and LUM–IVA. These make the treatments available to the NHS with a discount. The sizes of the discounts are commercial in confidence.