Cystic fibrosis (CF) is a genetic condition characterised by recurrent lung infections, inflammation and progressive lung damage. Patients with CF and advanced lung disease are limited when exercising and performing activities of daily life, due to increased breathlessness and lower oxygen levels. Exercise is an important part of treatment in CF, having been shown to slow down the lung disease and improve quality of life. Patients with CF are encouraged to exercise both at home and during hospital admissions, even when the lung disease is advanced. Often, oxygen therapy is used in patients whose oxygen levels are otherwise too low during. This, however, does not improve their breathlessness. Recently, a device to deliver air at flows higher than what other device allow has become available. High flow nasal therapy (HFNT) provides patients with air or a blend of air and oxygen at flows up to 60 L/min. HFNT can improve oxygen levels and reduce shortness of breath in many situations both in the acute and chronic setting. HFNT was shown to improve the tolerance to exercise in patients with other respiratory conditions (chronic obstructive pulmonary disease). In CF, HFNT is routinely used for patients admitted with acute respiratory failure (inability to maintain adequate oxygenation) with positive results. In this study, the Investigators aim to understand if HFNT can improve the exercise tolerance in patients with CF and advanced lung disease, by reducing breathlessness and avoiding the drop in oxygenation observed during simple training. The Investigators propose a short study to assess if further large clinical trials are feasible and practical, and will therefore collect preliminary data to have some results to use for planning other studies. All patients who are admitted in the Leeds Regional Adult CF Unit will be considered for participation in the study.
Age range
18 Years
Sex
ALL
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6 minute walking distance
Timeframe: 6 minutes