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Bedfordshire and Luton Joint Formulary
Bedfordshire Hospitals NHS Foundation Trust
Bedfordshire, Luton and Milton Keynes ICB
Formulary Chapter: 3 - Respiratory system 
Notes:

Any drug not listed on the Formulary should be considered Non-Formulary - Not recommended for prescribing
Links:
BLMK ICB Guidance: Chronic obstructive pulmonary disease guidelines for primary care BLMK ICB Guidance: Review of Inhaled Corticosteroids (ICS) in patients with COPD BLMK Paediatric Wheeze and Asthma Guidelines (Children Age 16 and under), Oct 2024 NICE NG115: COPD guideline NICE NG78: Cystic fibrosis: diagnosis and management. October 2017 Right Breathe Inhaler Prescribing Information
03.07 Mucolytics

Acetylcysteine
(Tablets)

GREEN
Formulary

Nacsys 600mg Effervescent tablets ONCE a day (lower pill burden than carbocisteine).

Brand prescribe on FP10. Most cost-effective brand is NACSYS.

BNF BNFc SPC

Acetylcysteine 2g/10mL
(nebulised)

RED
Formulary

Off-label use of the injection.

Most commonly use undiluted injection (20% solution) 3 to 5ml 3 to 4 times daily alternatively 400mg (2ml) mixed with 2mls sodium chloride 0.9% three or four times a day can be used.

BNF BNFc SPC

Carbocisteine

GREEN
Formulary
375mg Capsules
250mg in 5mL Syrup
BNF BNFc SPC

Ivacaftor tablets (Kalydeco®)

RED
Formulary

 For prescribing by specialists working within NHS England commissioned CF services only - No prescribing in Primary Care

CFTR modulator drugs are supplied via homecare and any admitted hospital inpatients requiring this should bring their own stock previously supplied to them through homecare to the hospital.

Entry reviewed: July 2025

BNF BNFc SPC
NICE TA988 Evidence-based recommendations on ivacaftor–tezacaftor–elexacaftor (Kaftrio) plus ivacaftor (Kalydeco), tezacaftor–ivacaftor (Symkevi) plus ivacaftor, and lumacaftor–ivacaftor (Orkambi) for treating cystic fibrosis. MHRA DSU Feb 2022: Ivacaftor, tezacaftor, elexacaftor (Kaftrio▼) in combination with ivacaftor (Kalydeco): risk of serious liver injury; updated advice on liver function testing

Ivacaftor, tezacaftor, elexacaftor 75 mg/50 mg/100 mg film-coated tablets (Kaftrio®)

RED
Formulary

 For prescribing by specialists working within NHS England commissioned CF services only - No prescribing in Primary Care

CFTR modulator drugs are supplied via homecare and any admitted hospital inpatients requiring this should bring their own stock previously supplied to them through homecare to the hospital.

Entry reviewed: July 2025

BNF BNFc SPC
NICE TA988 Evidence-based recommendations on ivacaftor–tezacaftor–elexacaftor (Kaftrio) plus ivacaftor (Kalydeco), tezacaftor–ivacaftor (Symkevi) plus ivacaftor, and lumacaftor–ivacaftor (Orkambi) for treating cystic fibrosis. MHRA DSU Feb 2022: Ivacaftor, tezacaftor, elexacaftor (Kaftrio▼) in combination with ivacaftor (Kalydeco): risk of serious liver injury; updated advice on liver function testing

lumacaftor–ivacaftor tablets (Orkambi®)

RED
Formulary

 

Ivacaftor 125 mg with Lumacaftor 100 mg

Ivacaftor 125 mg with Lumacaftor 200 mg

For prescribing by specialists working within NHS England commissioned CF services only - No prescribing in Primary Care

CFTR modulator drugs are supplied via homecare and any admitted hospital inpatients requiring this should bring their own stock previously supplied to them through homecare to the hospital.

Entry reviewed: July 2025

BNF BNFc SPC
NICE TA988 Evidence-based recommendations on ivacaftor–tezacaftor–elexacaftor (Kaftrio) plus ivacaftor (Kalydeco), tezacaftor–ivacaftor (Symkevi) plus ivacaftor, and lumacaftor–ivacaftor (Orkambi) for treating cystic fibrosis.

Mannitol Challenge Test (Osmohale®)

RED
Restricted Drug Restricted

For use by Bedford Hospital Respiratory Physiology department

BNF BNFc SPC

Methacholine solutions various strengths (Methylcholine, Acetyl-β-methylcholine)

RED
Formulary

unlicensed unlicensed

Respiratory department for breath challenge testing

tezacaftor–ivacaftor tablets (Symkevi®)

RED
Formulary

Ivacaftor 75 mg with Tezacaftor 50 mg

Ivacaftor 150 mg with Tezacaftor 100 mg

 

For prescribing by specialists working within NHS England commissioned CF services only - No prescribing in Primary Care

CFTR modulator drugs are supplied via homecare and any admitted hospital inpatients requiring this should bring their own stock previously supplied to them through homecare to the hospital.

Entry reviewed: July 2025

BNF BNFc SPC
NICE TA988 Evidence-based recommendations on ivacaftor–tezacaftor–elexacaftor (Kaftrio) plus ivacaftor (Kalydeco), tezacaftor–ivacaftor (Symkevi) plus ivacaftor, and lumacaftor–ivacaftor (Orkambi) for treating cystic fibrosis.

Vanzacaftor-tezacaftor-deutivacaftor (Alyftrek®)

RED
Restricted Drug Restricted

50mg deutivacaftor / 20mg tezacaftor / 4mg vanzacaftor film-coated tablets

125mg deutivacaftor / 50mg tezacaftor / 10 mg vanzacaftor film-coated tablets

 For prescribing by specialists working within NHS England commissioned CF services only - No prescribing in Primary Care

CFTR modulator drugs are supplied via homecare and any admitted hospital inpatients requiring this should bring their own stock previously supplied to them through homecare to the hospital.

Entry reviewed: July 2025

BNF BNFc SPC
NICE TA1085: Vanzacaftor-tezacaftor-deutivacaftor for treating cystic fibrosis with 1 or more F508del mutations in the CFTR gene in people 6 years and over
03.07 Dornase alfa
03.07 Hypertonic Sodium Chloride
Classifications
GREEN
May be initiated in any care setting
Amber SPA
Specialist to advise therapy and provide first 28 days supply, continuation in Primary Care
Amber SPIS
Specialist to initiate and stabilise medicine prior to continuation in Primary Care
SCG
To be prescribed as per Shared Care Guidance. If no SCG in place status reverts to red.
RED
Red medicines are designated as specialist only medicines which should only be prescribed by a specialist, usually within secondary care (either due to the requirement for specialist knowledge, long-term monitoring requirements, or restrictions that mean medicine supplies are only available to hospitals).
DNP
A decision has been made either locally and/or nationally not to routinely commission this preparation. Do not prescribe.
SELF
To be purchased over the counter. May be prescribed for chronic, long term conditions or on admission to hospital if essential.