Cystic Fibrosis Pulmonary Exacerbation

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 treatment of an exacerbation, the investigators will track symptoms and lung function during recovery, and collect blood, sputum and stool samples to allow us to explore the biological mechanisms of exacerbations and how they relate to different treatment responses. The study is event driven and will complete recruitment once 125 participants have completed treatment and follow-up for a severe exacerbation event. This study is funded by the Cystic Fibrosis Trust. This study is part of a wider programme of research, led by the PULSE-CF Innovation Hub (and hosted by the University of Manchester). The aim of the Hub is that the data from the UNIFIED-CF study will ultimately support the design of a platform clinical trial to test exacerbation-prevention interventions in CF.

The CF-Tracker study is a community surveillance study, designed to understand the causes of exacerbations in people with cystic fibrosis (CF) (pwCF). These are episodes when pwCF become more unwell, typically characterised by increased cough, sputum, and breathlessness, and requiring prolonged courses of oral or intravenous antibiotics. This observational study applies a two-tiered approach over 12 months. It will recruit 200 pwCF to Group A, and an additional 100 pwCF to Group B, which follows the same format but includes additional in-clinic sampling. Participants will provide longitudinal clinical data and biological samples. Group B will be offered at 5 specialist CF centres (Manchester, Cardiff, Newcastle, Leeds, Liverpool), will include additional sampling methods at clinic visits, and additional scheduled clinic visits at 1 month and 6 months. Group B participants will be offered an in-person visit if they become unwell, so that samples can be collected before they start antibiotics. In Group B, those attending the Manchester clinic will have the option of taking part in a 12 month home environmental and pollution monitoring, and sleep monitoring (both optional arms). A pilot study will test the practicalities of running the same protocol in a paediatric population. This will consist of up to 25 children with CF (5-15 years) attending a paediatric clinic in one of the four core centres. Up to 40 healthy volunteers will be recruited to provide samples on a single occasion as controls. This study is funded by the Cystic Fibrosis Trust. This study is part of a wider programme of research, led by the PULSE-CF Innovation Hub (and hosted by the University of Manchester, www.pulse-cf.com). The aim of the Hub is that the data from CF-Tracker will support the delivery of a platform clinical trial to test exacerbation-prevention interventions in CF.

The purpose of this study is to look at pulmonary exacerbations in people with cystic fibrosis (CF) that need to be treated with antibiotics given through a tube inserted into a vein (intravenous or IV). A pulmonary exacerbation is a worsening of respiratory symptoms in people with CF that needs medical intervention. Both doctors and CF patients are trying to understand the best way to treat pulmonary exacerbations. This study is trying to answer the following questions about treating a pulmonary exacerbation: * Do participants have the same improvement in lung function and symptoms if they are treated with one type of antibiotic (called beta-lactams or β-lactams) versus taking two different types of antibiotics (tobramycin and β-lactams)? * Is taking one type of antibiotic just as good as taking two types?

The goal of this study is to investigate the prevalence of dysglycemia with continuous glucose monitoring (CGM) obtained during pulmonary exacerbations, both outpatient and inpatient, in youth with cystic fibrosis (CF).