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Review
. 2021 Jan;159(1):93-102.
doi: 10.1016/j.chest.2020.09.084. Epub 2020 Sep 20.

Pulmonary Exacerbations in Adults With Cystic Fibrosis: A Grown-up Issue in a Changing Cystic Fibrosis Landscape

Gemma E Stanford  1 , Kavita Dave  2 , Nicholas J Simmonds  3
Affiliations
Review

Pulmonary Exacerbations in Adults With Cystic Fibrosis: A Grown-up Issue in a Changing Cystic Fibrosis Landscape

Gemma E Stanford et al. Chest. 2021 Jan.

Abstract

Pulmonary exacerbations (PExs) are significant life events in people with cystic fibrosis (CF), associated with declining lung function, reduced quality of life, hospitalizations, and decreased survival. The adult CF population is increasing worldwide, with many patients surviving prolonged periods with severe multimorbid disease. In many countries, the number of adults with CF exceeds the number of children, and PExs are particularly burdensome for adults as they tend to require longer courses and more IV treatment than children. The approach to managing PExs is multifactorial and needs to evolve to reflect this changing adult population. This review discusses PEx definitions, precipitants, treatments, and the wider implications to health-care resources. It reviews current management strategies, their relevance in particular to adults with CF, and highlights 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 nonantimicrobial agents alongside antibiotics. An overview of these issues is provided, concluding that with the changing landscape of adult CF care, the definitions and management of PExs may need to evolve to enable continued improvements in outcomes across the age spectrum of CF.

Keywords: cystic fibrosis; infection; review.

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Figures

Figure 1
Figure 1
IV antibiotic treatment for people with cystic fibrosis between 2004 and 2018, showing a higher prevalence in adults than children. (Reprinted with permission from the Cystic Fibrosis Foundation Patient Registry 2018 Annual Data Report.1)
Figure 2
Figure 2
The increasing percentage of adults with CF. CF = cystic fibrosis. (Reprinted with permission from the Cystic Fibrosis Foundation Patient Registry 2018 Annual Data Report.1)
Figure 3
Figure 3
Oxygen and NIV usage in the UK population with cystic fibrosis, showing a higher prevalence in adults than children. NIV = noninvasive ventilation. (Reprinted with permission from the UK CF Trust Registry 2018.70)
See this image and copyright information in PMC

References

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