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Maintenance of Pulmonary Therapies

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Cystic Fibrosis

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

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Abstract

The treatment of cystic fibrosis (CF) pulmonary disease is aimed to optimize pulmonary function and prevent disease progression and other complications through (1) management of chronic infection; (2) clearance of mucous secretions; and (3) reduction of airway inflammation. Central to the concepts of precision medicine and coproduction among the individual, family, and clinical care team, the management of CF pulmonary disease should be tailored to an individual’s respiratory needs and personal preferences. Perhaps the most recognizable precision therapies are cystic fibrosis transmembrane conductance regulator (CFTR) modulators in adults and children with CF as young as 6 months of age. Most of the currently used therapeutic interventions in CF, such as selection of antibiotics for specific respiratory infections and modalities for effective airway clearance, constitute personalized treatment regimens. In addition, clinical practice guidelines have been developed worldwide to standardize the treatment of CF pulmonary disease and updated to include the CFTR modulator therapies. How the CFTR modulator therapies might alter the present recommendations for maintenance pulmonary therapies in CF pulmonary disease is a focus for future investigations. Therefore, it is critical for CF care teams to collaborate with individuals and families to develop a therapeutic regimen and optimize individual care. This chapter will review maintenance pulmonary therapies in CF and includes a family’s perspectives on chronic daily treatments.

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Authors and Affiliations

  1. Johns Hopkins School of Medicine, Eudowood Division of Pediatric Respiratory Sciences, Baltimore, MD, USA

    Shruti M. Paranjape

  2. The Johns Hopkins Medical Institutions, Department of Pediatrics, Baltimore, MD, USA

    Peter J. Mogayzel Jr

Authors
  1. Shruti M. Paranjape
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  2. Peter J. Mogayzel Jr
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Corresponding author

Correspondence to Shruti M. Paranjape .

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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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Paranjape, S.M., Mogayzel, P.J. (2020). Maintenance of Pulmonary Therapies. In: Davis, S., Rosenfeld, M., Chmiel, J. (eds) Cystic Fibrosis. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-42382-7_10

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  • DOI: https://doi.org/10.1007/978-3-030-42382-7_10

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