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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