Preface

Cystic fibrosis (CF) is one of the most common fatal inherited diseases. The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene 30 years ago set the cornerstone for unravelling the pathogenesis of CF lung disease, continuous development of disease-modifying agents, and the inception of mutation-specific therapies, which are now becoming available for a subgroup of patients.

This book provides an update on aspects of CF lung disease. Frost et al. describe the use of novel culture-independent techniques such as next-generation sequencing to analyse the lung microbiome, allowing us to further understand the diversity, complexity, and effects of acute exacerbations, and the use of antibiotics on bacterial communities. The understanding of the CF microbiome presents new opportunities for disease management in CF and has the potential to explore the impact of CFTR modulators. Abdelbary also provides an overview of the microbiology in CF including the impact of bacterial colonisation, fungi and respiratory viruses.

The overview of CFTR modulators and gene therapy provides understanding of personalized therapeutic options with a focus on an individual's specific mutations. Rang et al. provide a succinct summary of these exciting treatment modalities in CF and how these treatment modalities for forever alter the care of these patients.

Szczesniak et al. discuss the use of CF registries that have the potential to improve on CF clinical management effectiveness and the development of treatment policies, although missing and confounding data can be huge challenges in the evolution of these policies.

Around 40 percent of patients with CF have some form of liver abnormalities. In recent years, an increase in CF-related liver disease (CFLD) has been noted. This is most likely related to patients with CF who are being screened more stringently for liver disease starting from a young age. Wiecek et al. provide an overview of the risk factors, clinical symptoms, diagnostic methods and treatment of liver changes in the course of CF.

In light of COVID-19, many health-governing bodies are turning to remote consultations in order to minimise the risk of infection for staff and patients. Fogazzi et al. describe the use of video education to improve the adherence of airway clearance and inhaled therapies. This is an important stress test depicting the usefulness of technology in this specific patient cohort.

Finally, Vagg et al. describe the design and development of a patient passport mHealth application for adults with CF. This application allows patients access to their own data. It will be of benefit when travelling abroad and between CF centres. This technology also has the potential to improve the quality and safety of care.

This book is invaluable for clinicians, scientists and researchers involved in the care of patients with CF.
