Key to symbols
The medicine should be initiated by, or on the advice of a specialist, but is suitable for continuation by a GP
The medicine should only be used and prescribed by a specialist
Indicates the preferred choice within a class or group of medicines
3. Respiratory system
3.12. Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies
Total Formulary
IVACAFTOR, LUMACAFTOR (ORKAMBI)
Restrictions:
Restricted to specialist use for the treatment of the following indication:
- Treatment of cystic fibrosis (CF) in patients aged 1 year and older (granules in sachet) or 6 years and older (film-coated tablets) who are homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
IVACAFTOR, TEZACAFTOR (SYMKEVI)
Restrictions:
Restricted to specialist use for the treatment of the following indication:
- 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.
IVACAFTOR, TEZACAFTOR, ELEXACAFTOR (KAFTRIO)
Restrictions:
Restricted to specialist use for the treatment of the following indication:
- In a combination regimen with ivacaftor for the treatment of cystic fibrosis (CF) in patients aged 2 years to less than 6 years (granules in sachet) and 6 years and older (film-coated tablets) who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.