» Articles » PMID: 38154821

Improved Recognition of Lung Function Decline As Signal of Cystic Fibrosis Pulmonary Exacerbation: a Cystic Fibrosis Learning Network Innovation Laboratory Quality Improvement Initiative

Overview
Journal BMJ Open Qual
Specialty Health Services
Date 2023 Dec 28
PMID 38154821
Authors
Affiliations
Soon will be listed here.
Abstract

Introduction: Cystic fibrosis (CF) is a systemic autosomal recessive condition characterised by progressive lung disease. CF pulmonary exacerbations (PEx) are episodes of worsening respiratory status, and frequent PEx are a risk factor for accelerated lung function decline, yet many people with CF (PwCF) go untreated at the time of decline. The goal of this quality improvement (QI) initiative was to improve recognition, treatment and follow-up of PEx in PwCF.

Methods: Using the Model for Improvement, the Cystic Fibrosis Learning Network (CFLN) initiated a QI innovation laboratory (iLab) with a global aim to decrease the rate of lung function decline in PwCF. The iLab standardised definitions for signals of PEx using a threshold for decline in forced expiratory volume in one second (FEV) and/or changes in symptoms. The FEV decline signal was termed FIES (FEV-indicated exacerbation signal). Processes for screening and recognition of FIES and/or symptom changes, a treatment algorithm and follow-up in the presence of a signal were tested concurrently in multiple settings.

Specific Aims: The specific aim is to increase the per cent of PwCF assessed for a PEx signal at ambulatory encounters and to increase the per cent of recommendations to follow-up within 6 weeks for PwCF experiencing a PEx signal.

Results: FIES recognition increased from 18.6% to 73.4% across all teams during the iLab, and every team showed an improvement. Of PwCF assessed, 15.8% experienced an FIES event (>10% decline in FEV per cent predicted (FEVpp)). Follow-up within 6 weeks was recommended for an average of 70.5% of those assessed for FIES and had an FEVpp decline greater than 5%.

Conclusion: The CFLN iLab successfully defined and implemented a process to recognise and follow-up PEx signals. This process has the potential to be spread to the larger CF community. Further studies are needed to assess the impact of these processes on PwCF outcomes.

Citing Articles

Evaluating precision medicine tools in cystic fibrosis for racial and ethnic fairness.

Colegate S, Palipana A, Gecili E, Szczesniak R, Brokamp C J Clin Transl Sci. 2024; 8(1):e94.

PMID: 39220818 PMC: 11362628. DOI: 10.1017/cts.2024.532.


Interventions to improve system-level coproduction in the Cystic Fibrosis Learning Network.

Gamel B, Albon D, Bandla S, Davison D, Flath J, Sabadosa K BMJ Open Qual. 2024; 13(3).

PMID: 39067867 PMC: 11287073. DOI: 10.1136/bmjoq-2024-002860.

References
1.
Harris P, Taylor R, Thielke R, Payne J, Gonzalez N, Conde J . Research electronic data capture (REDCap)--a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform. 2008; 42(2):377-81. PMC: 2700030. DOI: 10.1016/j.jbi.2008.08.010. View

2.
Sanders D, Bittner R, Rosenfeld M, Hoffman L, Redding G, Goss C . Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation. Am J Respir Crit Care Med. 2010; 182(5):627-32. PMC: 5450763. DOI: 10.1164/rccm.200909-1421OC. View

3.
Kraynack N, David Gothard M, Falletta L, McBride J . Approach to treating cystic fibrosis pulmonary exacerbations varies widely across US CF care centers. Pediatr Pulmonol. 2011; 46(9):870-81. DOI: 10.1002/ppul.21442. View

4.
Ong T, Van Citters A, Dowd C, Fullmer J, List R, Pai S . Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network. J Cyst Fibros. 2021; 20 Suppl 3:57-63. DOI: 10.1016/j.jcf.2021.08.035. View

5.
Albon D, Thomas L, Hoberg L, Stamper S, Somerville L, Varghese P . Cystic fibrosis learning network telehealth innovation lab during the COVID-19 pandemic: a success QI story for interdisciplinary care and agenda setting. BMJ Open Qual. 2022; 11(2. PMC: 9121114. DOI: 10.1136/bmjoq-2022-001844. View