Oxygen and Non-Invasive Ventilation Pathways in an Adult Cystic Fibrosis Centre


Cystic Fibrosis (CF) is a genetically inherited condition affecting more than 10,000 people in the United Kingdom. A progressive cycle of infection and lung damage occurs. Worsening lung function results in hypoventilation and ultimately leads to respiratory failure that may require supplementary oxygen and/or mechanical support such as Non-Invasive Ventilation (NIV). Guidelines support the use of NIV for nocturnal hypoventilation, hypercapnic respiratory failure and as a bridge to transplant. At the time of development, there were no published guidelines on the use of oxygen therapy in CF and no published pathways on the set up and management of supplementary oxygen or NIV in CF. This special interest report documents the development of separate oxygen and NIV pathways through interdisciplinary working in an adult CF centre.

To develop pathways for supplementary oxygen and the set up and management of NIV in an adult CF centre.

4 elective NIV set ups
since implementing the pathway


An evaluation of the CF centre's NIV and oxygen services, patient demographics and clinical needs was conducted alongside a review of current guidelines. An absence of formal standardised multidisciplinary team (MDT) processes was found. An opportunity to utilise clinical specialist knowledge across various teams was identified.

Interdisciplinary working groups were created and produced the pathways. Standard operating procedures were created for the transfer of CF patients on acute NIV between wards and the management of CF patients admitted on home NIV. A formal doctor-to-doctor referral procedure was created with weekly meetings between the NIV and CF specialist physiotherapists. The pathways were presented to CF, NIV and various ward multidisciplinary teams and successfully implemented in 2016. The NIV pathway was used during meetings, helping identify patients who potentially would need NIV set up and troubleshooting those already established. Cases were then presented to medical consultants for consideration and further planning or referral for NIV set up.


NIV Pathway
Since implementation of the NIV pathway, there have been 4 elective NIV set ups. This had not occurred previously. No emergency NIV set ups have occurred.

Oxygen Pathway
Prior to the implementation of the oxygen pathway, patients were assessed once signs of a need for long term supplementary oxygen were apparent. The Oxygen pathway is now used for all patients attending clinic or being admitted to the ward, to enable timely oxygen assessments to take place


Collaborative working utilising clinicians' specialist knowledge and skills in a team approach enabled the development of the pathways the production of specific supplementary oxygen and NIV pathways providing a number of patient benefits.

The oxygen pathway facilitated a more systematic and proactive approach to consideration of oxygen assessment and treatment planning. The NIV pathway has promoted a culture of elective rather than emergency NIV set ups. The proactive team approach resulted in more set ups being conducted electively and facilitated team and patient involvement in the process. Future work on the pathway will include an audit and examination against the latest published guidelines.

Top three learning points

  1. Initiating and facilitating interdisciplinary working can be time consuming and challenging. It requires patients, resilience and persistence.
  2. Don’t be afraid to question your own work. Improvement needs critical appraisal.
  3. Clinical practice changes while projects are underway. Be flexible in the approach to the project and adapt to changes as they occur.

Funding acknowledgements

This work was not funded.

Additional notes

This work was presented at Physiotherapy UK 2018.