Care of Individuals with Advanced Cystic Fibrosis Lung Disease

Patient Guideline Summary

Publication Date: April 30, 2020

Last Updated: March 3, 2023

Objective

This patient summary means to discuss key recommendations from the Cystic Fibrosis Foundation (CFF) for care of individuals with advanced cystic fibrosis lung disease.

Overview

Cystic fibrosis is a hereditary disease of the lungs, digestive system, pancreas, and other organs. It is present at birth but may not cause symptoms until adulthood.

We will use the abbreviation CF throughout this summary to refer to cystic fibrosis and CFF to refer to the Cystic Fibrosis Foundation.

Symptoms are primarily digestive and respiratory – constipation, greasy stools, wheezing, lung infections, and productive cough.

This patient summary focuses on the management of advanced CF.

Diagnosis

- The CFF lists 8 criteria, any one of which defines an advanced state of disease.

Treatment

Patients and their caregivers should engage in advance care planning with a full discussion of care goals, disease progression, and lung transplantation at the initial visit and with significant changes in status.
The CFF recommends:
- Assessment of oxygen need during activities, rest, and sleep.
- Supplemental oxygen at night.
- Screening for infectious agents including fungi.
- Monitoring for accumulating toxicity from medications.
- Expert risk assessment if considering pregnancy.
- Expert consultation with pain specialists if opioid treatment is under consideration.
- Mental health screening and expertise if indicated.
- Assessing financial resources at least biannually.
- Coordinated transfer from pediatric to adult care.
If consistent with treatment goals, CFF recommends consideration of:
- Lung transplantation
- Intensive care
- A trial of high flow nasal cannula oxygen and/or noninvasive ventilation
- Early tracheostomy when anticipated need for mechanical ventilation is more than 5–7 days
- Early transition to extracorporeal life support (life-sustaining treatment methods requiring devices to secure oxygenation and blood flow).
- A trial of continuous alternating inhaled antibiotics
- Pulmonary rehabilitation
- Tube feeding

Abbreviations

CF: Cystic Fibrosis
CFF: Cystic Fibrosis Foundation

Source Citation

Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, Faro A, Albright J, Benden C, Blair S, Dellon EP, Gochenour D, Michelson P, Moshiree B, Neuringer I, Riedy C, Schindler T, Singer LG, Young D, Vignola L, Zukosky J, Simon RH. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020 May;19(3):344-354. doi: 10.1016/j.jcf.2020.02.015. Epub 2020 Feb 27. PMID: 32115388.

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.