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BNSSG Paediatric Joint Formulary

3.3 Conditions affecting sputum viscosity

Last edited: 24-10-2024

3.3.1 Cystic fibrosis

First line drugs Second line drugs Specialist drugs Secondary care drugs

 

Dornase alfa (TLS Red)

Ivacaftor (TLS Red)

Elexacaftor / Tezacaftor / Ivacaftor (TLS Red)

  • NHSE SSC 2587 Changes in licensed access to elexacaftor/tezacaftor/ivacaftor for patients aged 2- 5 years with cystic fibrosis and homozygous for F508del combined with any other mutation
  • As per NICE TA988 Ivacaftor–tezacaftor–elexacaftor, tezacaftor–ivacaftor and lumacaftor–ivacaftor for treating cystic fibrosis

Tezacaftor / Ivacaftor (TLS Red)

  • As per NICE TA988 Ivacaftor–tezacaftor–elexacaftor, tezacaftor–ivacaftor and lumacaftor–ivacaftor for treating cystic fibrosis

Lumacaftor / Ivacaftor (TLS Red)

  • As per NICE TA988 Ivacaftor–tezacaftor–elexacaftor, tezacaftor–ivacaftor and lumacaftor–ivacaftor for treating cystic fibrosis

Hypertonic Sodium chloride 6% (Mucoclear®) or 7% (Nebusal®) (TLS Amber)

  • To be started on Specialist respiratory advice only