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Advanced Stage Lung Disease

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Cystic Fibrosis

Part of the book series: Respiratory Medicine ((RM))

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Abstract

The pathogenesis of cystic fibrosis (CF) lung disease is the downstream result of multiple factors including chronic airways infection and unabated pulmonary and systemic inflammation resulting in progressive loss of lung function. Therapeutic advancements including airway clearance therapies, antibiotics, and highly effective CFTR modulators have resulted in improved health outcomes and improved survival; however, the predicted life expectancy for CF patients remains well below that of the rest of the population, and respiratory failure is the most common cause of death. As such, the patients, their families, and CF care providers must be prepared to manage the challenges of advanced stage lung disease. The focus of this chapter is the care of CF patients with advanced stage lung disease and includes defining advanced CF lung disease, risk factors associated with the development of advanced lung disease, the pulmonary complications associated with advanced CF lung disease and their corresponding management, and, finally, referral for transplantation and palliative care.

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Author information

Authors and Affiliations

  1. Department of Medicine, Medical University of South Carolina, Charleston, SC, USA

    Bryan Garcia & Patrick A. Flume

  2. Person with CF2, Bluffton, SC, USA

    Jessica Mattson

  3. Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA

    Patrick A. Flume

Authors
  1. Bryan Garcia
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  2. Jessica Mattson
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  3. Patrick A. Flume
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Corresponding author

Correspondence to Bryan Garcia .

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Editors and Affiliations

  1. The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Children’s Hospital, Chapel Hill, NC, USA

    Stephanie Duggins Davis

  2. Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, WA, USA

    Margaret Rosenfeld

  3. Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, IN, USA

    James Chmiel

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Garcia, B., Mattson, J., Flume, P.A. (2020). Advanced Stage Lung Disease. In: Davis, S., Rosenfeld, M., Chmiel, J. (eds) Cystic Fibrosis. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-42382-7_11

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  • DOI: https://doi.org/10.1007/978-3-030-42382-7_11

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