Critical Care of the Adult Patient With Cystic Fibrosis

Christopher S. King, A. Whitney Brown, Shambhu Aryal, Kareem Ahmad, Scott H Donaldson

Research output: Contribution to journalReview article

Abstract

Cystic fibrosis (CF) is a multiorgan, genetic disease resulting from dysfunction of the CF transmembrane conductance regulator. The primary clinical manifestations are bronchiectasis, chronic pulmonary infections with recurrent exacerbations, and pancreatic insufficiency. Advances in CF care have led to improved survival, with more than one-half of those affected being adults. As a result, adult pulmonary and critical care physicians increasingly will be involved in the care of these patients. Patients with CF are at risk for numerous conditions that require ICU admission, including respiratory failure, massive hemoptysis, pneumothorax, hepatic failure, and bowel obstruction. Multiple aspects of the care of patients with CF benefit from specialized knowledge, including pancreatic enzyme replacement and nutritional support; airway clearance modalities; treatment of multiply resistant, polymicrobial infections, and unique drug metabolism. In extreme cases, patients may benefit from advanced therapies, including extracorporeal support and organ transplant. Optimal care of patients with CF requires a multidisciplinary care team that includes respiratory therapists, dieticians, social workers, psychologists, pharmacists, and physicians who have expertise in the treatment of this complex, multisystem disorder.

LanguageEnglish (US)
Pages202-214
Number of pages13
JournalChest
Volume155
Issue number1
DOIs
StatePublished - Jan 1 2019

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Critical Care
Cystic Fibrosis
Patient Care
Physicians
Exocrine Pancreatic Insufficiency
Cystic Fibrosis Transmembrane Conductance Regulator
Lung
Inborn Genetic Diseases
Bronchiectasis
Nutritionists
Nutritional Support
Hemoptysis
Liver Failure
Pneumothorax
Coinfection
Pharmacists
Respiratory Insufficiency
Therapeutics
Psychology
Transplants

Keywords

  • ECMO and cystic fibrosis
  • cystic fibrosis
  • intensive care
  • lung transplant
  • mechanical ventilation

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Critical Care of the Adult Patient With Cystic Fibrosis. / King, Christopher S.; Brown, A. Whitney; Aryal, Shambhu; Ahmad, Kareem; Donaldson, Scott H.

In: Chest, Vol. 155, No. 1, 01.01.2019, p. 202-214.

Research output: Contribution to journalReview article

King, CS, Brown, AW, Aryal, S, Ahmad, K & Donaldson, SH 2019, 'Critical Care of the Adult Patient With Cystic Fibrosis', Chest, vol. 155, no. 1, pp. 202-214. https://doi.org/10.1016/j.chest.2018.07.025
King, Christopher S. ; Brown, A. Whitney ; Aryal, Shambhu ; Ahmad, Kareem ; Donaldson, Scott H. / Critical Care of the Adult Patient With Cystic Fibrosis. In: Chest. 2019 ; Vol. 155, No. 1. pp. 202-214.
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