Humidified high flow air for children and young people with Cystic Fibrosis (CF): A pilot study

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Cystic fibrosis (CF) is a common genetic disorder affecting approximately 1 in 3000 people in New Zealand. CF results in thickened airway mucus which causes infection, inflammation and loss of lung function with gradual onset of respiratory failure. Individuals with CF have poor growth and failure to thrive occuring through malabsorption of fat and fat-soluble nutrients, but also through increased energy expenditure with increased work of breathing. Unlike healthy controls, persons with CF have an increased respiratory rate and work of breathing during sleep. Reducing this may have long term benefits in improving respiratory health, weight gain and overall quality of life. Humidified high flow air via nasal prongs (HHF) is a novel but increasingly utilized treatment to decrease work of breathing. It is currently used in the hospital setting, but is a relatively easy treatment to deliver in the home. HHF delivers flows up to 70 L/min via nasal prongs and if delivered through sleep may benefit persons with CF by : i) Reducing the work of breathing through reduced upper airway resistance and dead space ii) Improving oxygenation iii) Increasing hydration of airway secretionsimproving airway secretion clearance As well as short term benefits – these could result in more substantial long term benefits with improved weight gain and reduced respiratory infections – both directly associated with disease prognosis. The Starship Respiratory Department is the national centre for children with CF, and the national centre for sleep and home respiratory support providing outreach to other hospitals around New Zealand. In this - initial randomised controlled cross-over study, we will determine the effect of HHF compared to a usual night of ‘unsupported’ sleep in 15 young persons with CF on respiratory rate, work of breathing, and other physiological parameters as well as measuring sleep quality. We will also determine the acceptability of having this support in the home. If effective and acceptable this will inform the development of a larger multi-centre study measuring the effect of HHF on longerterm outcomes such as weight gain, respiratory infections , ability to exercise, quality of life, and general well-being. HHF is potentially an effective new treatment to be used in the home to prevent disease progression and improve longevity.

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