CFTR modulators (Formulary)

TAM (Treatments and Medicines) NHS Highland
NHS Highland

CFTR: Cystic fibrosis transmembrane conductance regulator

IVACAFTOR WITH TEZACAFTOR AND ELEXACAFTOR

Therapy notes

  • EMC
  • MHRA Alert: Ivacaftor, tezacaftor, elexacaftor (Kaftrio▼) in combination with ivacaftor (Kalydeco): risk of serious liver injury; updated advice on liver function testing (February 2022) (www.gov.uk).

Formulation and dosage details

Formulation:

Tablets (Kaftrio®) 37.5mg/25mg/50mg, 75mg/50mg/100mg (Restricted: Hospital use only)

Dosage:

As per SMC2713: 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.

Homecare is the preferred option - contact nhsh.homecare@nhs.scot

Notes:

  • For primary care: please note that this drug has multiple interactions therefore please check when prescribing other medication.
  • Interacts with grapefruit juice.

Formulation and dosage details

Formulation:

Granules in sachet (Kaftrio®) 60mg/40mg/80mg, 75mg/50mg/100mg (Restricted: Hospital use only)

Dosage:

As per SMC2713: 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.

Homecare is the preferred option - contact nhsh.homecare@nhs.scot

Notes:

  • For primary care: please note that this drug has multiple interactions therefore please check when prescribing other medication.
  • Interacts with grapefruit juice.

LUMACAFTOR WITH IVACAFTOR

Therapy notes

Formulation and dosage details

Formulation:

Tablets (Orkambi®) 100mg/125mg, 200mg/125mg (Restricted: Hospital use only)

Dosage:

As per SMC2712: 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.

Homecare is the preferred option - contact nhsh.homecare@nhs.scot

Notes:

  • For primary care: please note that this drug has multiple interactions therefore check when prescribing other medication.

Formulation and dosage details

Formulation:

Granules in sachet (Orkambi®) 75mg/94mg, 100mg/125mg, 150mg/188mg (Restricted: Hospital use only)

Dosage:

As per SMC2712: 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.

Homecare is the preferred option - contact nhsh.homecare@nhs.scot

Notes:

  • For primary care: please note that this drug has multiple interactions therefore check when prescribing other medication.

TEZACAFTOR WITH IVACAFTOR

Therapy notes

Formulation and dosage details

Formulation:

Tablets (Symkevi®) 50mg/75mg, 100mg/150mg (Restricted: Hospital use only)

Dosage:

As per SMC2711: 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.

Homecare is the preferred option - contact nhsh.homecare@nhs.scot

Notes:

  • For primary care: please note that this drug has multiple interactions therefore please check when prescribing other medication.
  • Interacts with grapefruit juice.

Editorial Information

Document Id: F387