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Cystic Fibrosis: an Update on Disease Pathophysiology, Management, and Novel Modalities of Therapy

Current Treatment Options in Allergy volume 6, pages 226–237 (2019)Cite this article

Abstract

Purpose of review

To explain the underlying pathophysiology of cystic fibrosis and to describe current treatment modalities for disease manifestations, including an introduction to novel therapies known as CFTR modulator drugs, which are now available to over 50% of people with cystic fibrosis.

Recent findings

CFTR modulator drugs, aimed at restoring CFTR protein function, have been shown to improve lung function and reduce pulmonary exacerbations, improve BMI and QOL, and are a new added treatment to the traditional therapies for cystic fibrosis.

Summary

With the current modalities of treatment available to patient with cystic fibrosis, the quality of life and the life expectancy continues to improve. Future directions for research include looking at novel anti-inflammatory agents, antimicrobial agents, mucociliary clearance agents, and newer generation CFTR modulator drugs/drugs that restore CFTR function.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  1. UCLA Pulmonary and Critical Care Fellow, University of California, Los Angeles, 10833 Le Conte Avenue Room 43-229 CHS, Los Angeles, CA, 90095-1690, USA

    Timothy J. Young

  2. UCLA Division of Pediatric Pulmonology, Mattel Children’s Hospital, University of California, Los Angeles, 10833 Le Conte Avenue 22-387 MDCC, Los Angeles, CA, 90095, USA

    Douglas A. Li

  3. UCLA Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles, 1223 16th Street, Suite 3400, Santa Monica, CA, 90404, USA

    Patricia H. Eshaghian

Authors
  1. Timothy J. Young
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  2. Douglas A. Li
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  3. Patricia H. Eshaghian
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Corresponding author

Correspondence to Patricia H. Eshaghian.

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Timothy Young, Douglas Li, and Patricia Eshaghian have no conflicts of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Pediatric Allergy

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Young, T.J., Li, D.A. & Eshaghian, P.H. Cystic Fibrosis: an Update on Disease Pathophysiology, Management, and Novel Modalities of Therapy. Curr Treat Options Allergy 6, 226–237 (2019). https://doi.org/10.1007/s40521-019-00211-4

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  • DOI: https://doi.org/10.1007/s40521-019-00211-4

Keywords

  • Cystic fibrosis
  • Bronchiectasis
  • Nasal polyps
  • Chronic rhinosinusitis
  • Pancreatic insufficiency
  • CFTR modulator