Guidelines for the Care of Individuals With Advanced CF Lung Disease

These guidelines provide recommendations to the cystic fibrosis community regarding the management of advanced cystic fibrosis lung disease (ACFLD), including reducing practice variability, improving the quality of life and survival of those with ACFLD, and identifying future research directions.

7 min read
In this article
Summary
  • The goal of these consensus guidelines is to provide guidance for management of advanced cystic fibrosis lung disease (ACFLD).
  • Recognizing that care must be customized to each individual, these recommendations aim to reduce practice variability, improve the quality of life and survival of those with ACFLD, and identify gaps in clinical knowledge where future research is needed.

Guidelines for the Care of Individuals With Advanced CF Lung Disease: Executive Summary

S.G. Kapnadak, E. Dimango and D. Hadjiliadis et al. 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 https://doi.org/10.1016/j.jcf.2020.02.015. Epub 2020 Feb 27. 

Purpose and Background

While the quality of life and survival of individuals with cystic fibrosis are improving, advanced cystic fibrosis lung disease (ACFLD) remains common and the most frequent cause of death. ACFLD is associated with increased physical symptoms and emotional stress, pulmonary exacerbations, and health care utilization. Despite its importance, there is limited literature directed specifically at the unique challenges facing individuals with ACFLD. Furthermore, clinical experience suggests variability in practice patterns pertaining to ACFLD management with some aspects of recommended care shown to be inconsistently applied. The goal of these consensus guidelines is to provide guidance for management of ACFLD. Recognizing that care must be customized to each individual, these recommendations aim to reduce practice variability, improve the quality of life and survival of those with ACFLD, and identify gaps in clinical knowledge where future research is needed.

Methodology

The Cystic Fibrosis Foundation assembled a multidisciplinary team that utilized PICO (Population, Intervention, Control, Outcome) methodology to guide systematic searches of the medical literature. Recommendations were developed, voted on, and accepted if 80 percent of the committee agreed, after which feedback from public comment was incorporated.

Recommendations

Diagnosis

Recommendations Evaluation of the Evidence
1. When individuals with CF meet criteria for ACFLD, the CF Foundation recommends routine advance care planning conversations with them and their caregiver(s), including communication about prognosis and goals of care, documentation of advance directives, and decision-making surrounding lung transplantation. Consensus
2. The CF Foundation recommends that individuals with ACFLD undergo screening for hypoxemia on exertion and sleep, hypercarbia, and pulmonary hypertension. Consensus
3. The CF Foundation recommends supplemental oxygen for individuals with ACFLD and exercise-induced or nocturnal hypoxemia. Consensus
4. The CF Foundation recommends consideration of nocturnal noninvasive ventilation for individuals with ACFLD and chronic hypercarbia. Consensus
5. The CF Foundation found insufficient evidence to make a recommendation regarding the use of pulmonary vasodilator therapy in individuals with ACFLD and pulmonary hypertension. Consensus
6. The CF Foundation recommends lung transplantation as a treatment option for individuals with ACFLD if congruent with goals of care. Consensus
7. The CF Foundation recommends that individuals with ACFLD and acute respiratory failure be considered eligible for intensive care unit management regardless of transplant status if congruent with goals of care. Consensus
8. The CF Foundation recommends that individuals with ACFLD and acute respiratory failure be considered for a trial of high flow nasal cannula oxygen and/or noninvasive ventilation. Consensus
9. For individuals with ACFLD and acute respiratory failure requiring invasive mechanical ventilation, the CF Foundation recommends consideration of early tracheostomy when anticipated need for mechanical ventilation is more than 5–7 days and support remains congruent with goals of care. Consensus
10. The CF Foundation recommends that individuals with ACFLD who develop refractory respiratory failure requiring invasive mechanical ventilation be considered for early transition to extracorporeal life support if congruent with goals of care. Consensus
11. For individuals with ACFLD, the CF Foundation recommends a trial of continuous alternating inhaled antibiotics as dictated by bacterial pathogens identified in respiratory culture. Consensus
12. The CF Foundation recommends that individuals with progressive ACFLD undergo screening for fungal pathogens in addition to standard microbiological screening. Consensus
13. The CF Foundation recommends that individuals with ACFLD participate in a pulmonary rehabilitation program. Consensus
14. The CF Foundation found insufficient evidence to make a recommendation regarding the use of systemic corticosteroids in individuals with ACFLD. Consensus
15. The CF Foundation found insufficient evidence to make a recommendation regarding routine screening for gastroesophageal reflux (GER) in individuals with ACFLD. Consensus
16. The CF Foundation recommends the use of enteral tube feeds for individuals with ACFLD and malnutrition after consideration of procedural risks versus benefits. Consensus
17. For individuals with ACFLD with frequent prior and continuing exposure to nephrotoxic and ototoxic agents, the CF Foundation recommends increased monitoring for accumulating toxicity. Consensus
18. The CF Foundation recommends that women with ACFLD contemplating pregnancy carefully consider the risks in consultation with high-risk obstetrics and CF providers. Consensus
19. For individuals with ACFLD with indications for opioids, the CF Foundation recommends treatment in accordance with established Center for Disease Control guidelines; this should include monitoring for adverse effects, and consultation with pain and/or palliative care specialists as appropriate. Consensus
20. For individuals with ACFLD and anxiety, the CF Foundation recommends management in accordance with the International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety, reserving benzodiazepines for refractory symptoms or end of life symptom palliation. Consensus
21. When individuals with CF meet criteria for ACFLD, and with subsequent changes in clinical or social status, the CF Foundation recommends a formal care conference involving caregiver(s) and selected team members to develop a plan for ongoing psychosocial support. Consensus
22. In individuals with ACFLD, the CF Foundation recommends assessing the adequacy of financial resources at least biannually, and with changes in clinical or social status. Consensus
23. For pediatric patients with ACFLD nearing the age of transition to an adult CF care program, the CF Foundation recommends formally outlining a transition plan that provides flexibility in timing and coordination of transfer. Consensus

Unanswered Questions

  • What is the optimal dissolved carbon dioxide (pCO2) threshold and modality of noninvasive ventilation in individuals with ACFLD and hypercapnia?
  • Is there a role for pulmonary vasodilators in ACFLD?
  • What are the implications of fungal pathogens when found in the sputum in ACFLD?
  • Is there a role for systemic corticosteroids in ACFLD?
  • Is there a role for screening for GER in ACFLD?
  • How will the advent of highly effective CFTR modulators impact ACFLD demographics, symptoms, and clinical trajectory?

Further Reading

Kavalieratos D, Georgiopoulos AM, Dhingra L, et al. Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines. J Palliat Med. 2020 Sep 16. doi: 10.1089/jpm.2020.0311. Online ahead of print.

Burgel P-R, Durieu I, Chiron R, et al. Rapid Improvement After Starting Elexacaftor-tezacaftor-ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease. Am J Respir Crit Care Med. 2021 Jul 1;204(1):64-73. doi: 10.1164/rccm.202011-4153OC

The guidelines were published in February 2020, they were reviewed in July 2021 and it was determined that no update is needed at this time.

This executive summary was prepared by:

Marc A. Sala, MD (Northwestern University Feinberg School of Medicine); Siddhartha G. Kapnadak, MD (University of Washington School of Medicine)

 

Share this article
Topics
Clinical Care Guidelines | Clinician Resources
Have questions? We’re here to help. Call us at 1-800-FIGHT CF

(1-800-344-4823)
Mon - Thu, 9 am - 7 pm ET
Fri, 9 am - 3 pm ET

 

More Ways To Get Help