Intensive Care Management of the Patient With Cystic Fibrosis

Author:

Kremer Ted M.1,Zwerdling Robert G.2,Michelson Peter H.3,O'Sullivan Brian P.2

Affiliation:

1. Department of Pediatrics, University of Massachusetts Medical Center, Worcester, Massachusetts,

2. Department of Pediatrics, University of Massachusetts Medical Center, Worcester, Massachusetts

3. Division of Pulmonary Medicine, Children's Hospital of Pittsburgh, Pennsylvania

Abstract

Cystic fibrosis was previously thought to be a disease of childhood. With a better understanding of this condition along with improvements in therapy, patients with cystic fibrosis are now living well into adulthood. The aim of this article is to familiarize the intensive care unit physician with cystic fibrosis care, to discuss complications associated with cystic fibrosis specifically related to the intensive care unit, and to detail the current recommendations for the clinical management of the patient with cystic fibrosis. With advancing disease, the most severely affected organs are the lungs. Obstruction, infection, and inflammation contribute to the decline of pulmonary function, ultimately leading to death. Some patients may be eligible for lung transplantation, but choosing wisely will affect posttransplant survival. Because other organs are affected by the genetic defect and associated treatments, serious complications related to the liver, pancreas, intestines, and kidneys must be considered by the intensivist faced with a patient with cystic fibrosis. As practitioners, the fact that not all patients will survive and help our patients and families gracefully through the end-of-life process should be accepted.

Publisher

SAGE Publications

Subject

Critical Care and Intensive Care Medicine

Cited by 15 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. What Are Special Considerations for Patients With Cystic Fibrosis?;Evidence-Based Practice of Palliative Medicine;2023

2. Outcomes of Children With Cystic Fibrosis Admitted to PICUs*;Pediatric Critical Care Medicine;2020-04-20

3. Pediatric lung transplantation and end of life care in cystic fibrosis: Barriers and successful strategies;Pediatric Pulmonology;2017-08-08

4. FARMACOLOGÍA DEL PACIENTE PEDIÁTRICO;Revista Médica Clínica Las Condes;2016-09

5. Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients;The Journal of Heart and Lung Transplantation;2013-06

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