Pulmonary Exacerbations in Adults With Cystic Fibrosis

Abstract

Pulmonary exacerbations (PEx) are significant life events in people with cystic fibrosis (CF), associated with declining lung function, reduced quality of life (QoL), hospitalisations and decreased survival. The adult CF population is increasing worldwide, with many patients surviving prolonged periods with severe multi-morbid disease. In many countries the number of adults with CF exceeds the number of children, and PEx are particularly burdensome for adults as they tend to require longer courses and more intravenous treatment than children. The approach to managing PEx is multifactorial and needs to evolve to reflect this changing adult population. In this review, we discuss PEx definitions, precipitants, treatments and the wider implications to healthcare resources. We review current management strategies, their relevance in particular to adults with CF, and highlight some of the gaps in our knowledge. A number of studies are underway to try to answer some of the unmet needs, such as the optimal length of treatment and the use of non-antimicrobial agents alongside antibiotics. We provide an overview of these issues, concluding that with the changing landscape of adult CF care, the definitions and management of PEx may need to evolve to enable continued improvements in outcomes across the age spectrum of CF.

Cystic Fibrosis: Recent Insights into Inhaled Antibiotic Treatment and Future Perspectives

“…Comparing these sequential treatments with inhaled aztreonam and tobramycin to tobramycin monotherapies, Estrella Rojo-Molinero and co-authors found that alternating antibiotics produced an increase in antibiofilm activity against the laboratory and clinical strains of P. aeruginosa [ 99 ]. Moreover, combinations of antibiotics against P. aeruginosa are commonly used to generate synergistic antibacterial activity and reduce drug resistance [ 100 ]. Finally, while CF patients may benefit from prolonged courses of inhaled antibiotics, there is currently little evidence to support the benefits of chronic maintenance therapy for bacteria other than P. aeruginosa [ 14 ].…”

Lung Microbiome in Cystic Fibrosis

“…Lung function measured by FEV (forced expiratory volume) decreases with age and correlates with less microbiome diversity and dominance of these two pathogens, which, when present, decrease lung function in young CF subjects as well [ 19 ]. Another important aspect to consider is the episodes of exacerbation with an increase in respiratory symptoms, which are very common and persistent during the lives of CF patients [ 22 ]. The most logical explanation would be that these episodes are due to an increase in CF pathogens, causing a consequent reduction in microbial diversity.…”

Allergic Reactions to Serine Protease-Like Proteins of Staphylococcus aureus

“…In the long term, the pathogen adapts to the host, gradually reducing its virulence during airway infection (5). Nevertheless, recurrent pulmonary exacerbations gradually worsen the lung function and clinical condition of CF patients (6).…”

“…Prevention and therapy of chronic bacterial and fungal inflammation are therefore key in the treatment regimen of CF (6)(7)(8).…”

“…In the long term, the pathogen adapts to the host, gradually reducing its virulence during airway infection ( 5 ). Nevertheless, recurrent pulmonary exacerbations gradually worsen the lung function and clinical condition of CF patients ( 6 ). Prevention and therapy of chronic bacterial and fungal inflammation are therefore key in the treatment regimen of CF ( 6 – 8 ).…”