Understanding Inflammation, InFection and Interventions in Severe Exacerbations of Cystic Fibrosis

The UNIFIED-CF study is an observational study designed to investigate the impacts of treatment given for severe pulmonary exacerbations in people living with cystic fibrosis (pwCF). Exacerbations are episodes when pwCF become more unwell, typically characterised by increased cough, sputum, and breathlessness and treated with a combination of oral and/or intravenous antibiotics. Severe exacerbations require treatment with intravenous antibiotics and impart considerable morbidity on pwCF. In this study, the investigators will recruit people at risk of severe CF exacerbations when they are well and if/when they are subsequently admitted for…

• : 1. Confirmed diagnosis of cystic fibrosis (CF), defined as presence of two pathogenic CF-causing CFTR mutations AND clinical features consistent with a diagnosis of CF, OR presence of at least one pathogenic CF-causing CFTR mutation AND sweat chloride (before use of CFTR modulators) >60mmol/L AND clinical features consistent with a diagnosis of CF. 2. Receiving care from a UK Adult Cystic Fibrosis Centre taking part in the study. 3. EITHER: • Have had at least 1 previous exacerbation of CF lung disease, treated with intravenous antibiotics, in the previous 12 months. OR • Enrolled in the CF-Tracker study (IRAS ID 338539) within the last 24 months (dated from date of completion of baseline Tracker visit) 4. In case of treatment for an exacerbation, likely to be treated with a ß-lactam or an anti-pseudomonal penicillin, combined with tobramycin or colistin, per CF Trust and NICE guidelines for 1st-line CF therapies. 5. Able to produce sputum (spontaneous or induced) at baseline visit. 6. Able to understand the patient information sheet, willing to consent to study protocol.

Use the source registry for the full inclusion and exclusion criteria before discussing referral or enrolment.