3.7 Mucolytics

Carbocisteine
  • Capsules 375mg (£7.27 = 2.25g daily)
  • Sachets 750mg/10ml sugar-free oral solution (£21.56 = 2.25g daily)

Indications

  • Reduction of sputum viscosity

Dose

  • Adult: Initially 2.25g daily in divided doses, then 1.5g daily in divided doses as condition improves. Treatment should only be continued if there is symptomatic improvement following a 4-week trial

Notes

  1. Carbocisteine sachets are licensed for adults and children over 15 years for the adjunctive therapy of respiratory tract disorders characterised by excessive, viscous mucus, including chronic obstructive airways disease (COPD)
Dornase alfa
  • Nebuliser solution 2500 units in 2.5ml

Indication

  • Management of cystic fibrosis patients with a forced vital capacity (FVC) of greater than 40% of predicted to improve pulmonary function

Notes

  1. A Jet Nebuliser used exclusively for dornase alfa is required for administration
Kalydeco®

(Ivacaftor)

  • Tablets 150mg

Notes

  1. See NHS England Clinical Commissioning Policy: Ivacaftor for Cystic Fibrosis (March 2012)
  2. NHS England has confirmed that ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor will be made available as treatment options for people with cystic fibrosis who have one of an expanded range of cystic fibrosis transmembrane conductance regulator (CFTR) mutations within the criteria set out in this commissioning statement.
  3. NHS England and NHS Improvement and Vertex Pharmaceuticals have concluded an access agreement to enable eligible patients in England access to treatment with ivacaftor via the NHS. A new agreement has commenced, which fully incorporates and expands the terms of a previous agreement (dated 11th November 2019)
    1. The purpose of this Agreement is to describe the arrangements intended to capture the data that may address the clinical uncertainties in the evidence base concerning ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor to inform a future health technology appraisal by NICE
    Orkambi®

    (Lumacaftor / Ivacaftor)

    • Tablets 100mg / 125mg, 200mg / 125mg

    Notes

    1. Whilst NICE TA398 (July 2016) states that Lumacaftor-ivacaftor is not recommended for treating cystic fibrosis in people 12 years and older who are homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, NHS England has confirmed that ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor will be made available as treatment options for people with cystic fibrosis who have one of an expanded range of cystic fibrosis transmembrane conductance regulator (CFTR) mutations within the criteria set out in this commissioning statement.
    2. NHS England and NHS Improvement and Vertex Pharmaceuticals have concluded an access agreement to enable eligible patients in England access to treatment with lumacaftor / ivacaftor via the NHS. A new agreement has commenced, which fully incorporates and expands the terms of a previous agreement (dated 11th November 2019)
      1. The purpose of this Agreement is to describe the arrangements intended to capture the data that may address the clinical uncertainties in the evidence base concerning ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor to inform a future health technology appraisal by NICE
    Symkevi®

    (Tezacaftor / Ivacaftor)

    • Tablets 100mg / 150mg

    Notes

    1. NHS England has confirmed that ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor will be made available as treatment options for people with cystic fibrosis who have one of an expanded range of cystic fibrosis transmembrane conductance regulator (CFTR) mutations within the criteria set out in this commissioning statement.
    2. NHS England and NHS Improvement and Vertex Pharmaceuticals have concluded an access agreement to enable eligible patients in England access to treatment with tezacaftor / ivacaftor via the NHS. A new agreement has commenced, which fully incorporates and expands the terms of a previous agreement (dated 11th November 2019)
      1. The purpose of this Agreement is to describe the arrangements intended to capture the data that may address the clinical uncertainties in the evidence base concerning ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor to inform a future health technology appraisal by NICE
    Kaftrio®

    (Elexacaftor / Tezacaftor / Ivacaftor)

    • Tablets 100mg / 50mg / 75mg

    Notes

    1. NHS England has confirmed that ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor will be made available as treatment options for people with cystic fibrosis who have one of an expanded range of cystic fibrosis transmembrane conductance regulator (CFTR) mutations within the criteria set out in this commissioning statement.
    2. NHS England and NHS Improvement and Vertex Pharmaceuticals have concluded an access agreement to enable eligible patients in England access to treatment with elexacaftor / tezacaftor / ivacaftor via the NHS. A new agreement has commenced, which fully incorporates and expands the terms of a previous agreement (dated 11th November 2019)
      1. The purpose of this Agreement is to describe the arrangements intended to capture the data that may address the clinical uncertainties in the evidence base concerning ivacaftor, lumacaftor / ivacaftor, tezacaftor / ivacaftor, and elexacaftor / tezacaftor / ivacaftor to inform a future health technology appraisal by NICE
    Mannitol
    • Inhalation powder capsules with device 40mg

    Notes

    1. NICE TA266: Mannitol dry powder for inhalation (Bronchitol) is recommended as an option for treating cystic fibrosis in adults (November 2012):
      1. who cannot use rhDNase because of ineligibility, intolerance or inadequate response to rhDNase and
      2. whose lung function is rapidly declining (forced expiratory volume in 1 second [FEV1] decline greater than 2% annually) and
      3. for whom other osmotic agents are not considered appropriate
    Last updated: 15-04-2021

     

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