Non Invasive Ventilation for Acute Exacerbations in Adult CF (NIVinCF)

Cystic Fibrosis (CF) is a life limiting illness. Median predicted UK survival is 41.4 years (UK CF Registry 2011). The commonest cause of death is respiratory failure.

Non invasive ventilation (NIV) is a system which delivers a preset pressure to supplement the size and depth of each breath. It is introduced in CF to manage established respiratory failure. A nose or a mask which covers both the nose and mouth allows flexible ventilation, is used just at night, or for part of the day in addition or for 24 hours as clinical status indicates. It is introduced within a normal ward environment and then continued longterm at home.Once respiratory failure is established longterm noninvasive ventilation is introduced throughout 24 hours and multidisciplinary assessment concludes that the timing is appropriate for the individual.

This study aims to evaluate a potential development of current practice: the use of non invasive ventilation during hospital admission only to enhance recovery from an acute exacerbation which has caused respiratory failure in those individuals where long term non invasive ventilation is not yet indicated. A mixed methods design will allow description of the experience of noninvasive ventilation during a semistructured interview to add to understanding of the results from an experiment designed to measure the differences between noninvasive ventilation and standard care.

Aim: To compare the clinical efficacy with the patient experience of NIV on recovery from an acute respiratory exacerbation complicated by respiratory failure in adult Cystic Fibrosis.