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