Non Invasive Ventilation for Acute Exacerbations in Adult CF

Design: The study will use a mixed methodology and it will be in 2 phases. Phase I A group randomised controlled trial The following measurements will be performed on days 1, 3, 7, 10, 14 and twice weekly until discharge: The exact timing of measurements will be planned by the participant and the researcher. All of the outcome measures form part of routine clinical practice except the symptom score; the Cystic Fibrosis Respiratory Symptoms Diary (CFRSD V2.0) (Goss et al., 2009) 1. Early morning and day time carbon dioxide (CO2) level. 2. Day time oxygen levels 3. Lung function Forced Expiratory Volume in 1 second (FEV1) and Forced Vital Capacity (FVC) 4. Day time resting Respiratory Rate (RR), Heart Rate (HR) 5. Symptom score CFRSD Phase II A qualitative exploration of the experience of using NIV for adults with Cystic Fibrosis during an acute exacerbation Methods: Prior to discharge a semistructured interview designed to explore the experience of using noninvasive ventilation will be undertaken by a clinical psychologist from the Cystic Fibrosis multidisciplinary team. The interviews will be undertaken purely for the research project, recorded and transcribed verbatim by the lead investigator. Thematic analysis will be undertaken to interpret and explore the individual's experience. Credibility checking of interpretation will be undertaken by the clinical psychologists and within academic supervision sessions.