Cystic fibrosis (CF) is a multisystem disorder with a significantly shortened life expectancy with the major cause of mortality related to lung disease. Inflammation is seen in the CF airways from a very early age and contributes significantly to symptoms and disease progression. As the condition worsens over time, lung function declines, usually measured by Forced Expiratory Volume in 1 second (FEV1)% predicted, and extra-pulmonary complications often manifest. While the life expectancy in CF is still short, the median age of death and predicted survival age are continually increasing. Therapeutic interventions for CF have improved significantly in the last 20 years and now there are targeted therapies towards specific elements in CF that may impact upon exacerbation frequency, symptoms, and eventually mortality due to lung disease.
Part of the book: Cystic Fibrosis in the Light of New Research
In classical teaching, the lungs were thought of as a sterile environment with the isolation of bacteria on sputum or bronchoalveolar lavage sampling felt to represent pathogenic colonisation in disease states. This teaching has been over-turned with the discovery of a rich microbiome in the respiratory tract. The respiratory microbiome is a huge target for novel research in many fields, most notable in that of airway diseases such as asthma and chronic obstructive pulmonary disease (COPD). Next-generation sequencing is a culture-independent method for microbial sampling which has transformed the accuracy and speed at which whole microbial communities can be described in studies. This has led to an explosion of knowledge regarding the human respiratory microbiome. COPD is a common, chronic disease of the respiratory system involving an irreversible airway obstruction which places huge burden on patients and healthcare systems alike. The respiratory microbiome is different in those who suffer from COPD than in those without the disease, but little is known as to the role of the microbiome in disease pathogenesis or manifestation. This chapter aims to outline the advances in sequencing methods in relation to the microbiome and establish a description of the respiratory microbiome in health and in COPD. We will describe the existing literature on the topic and discuss potential key areas for future research.
Part of the book: COPD