Indicators of pulmonary exacerbation in cystic fibrosis: A Delphi survey of patients and health professionals

McCourt, Fiona; O’Neill, Brenda; Logan, Ian; Abbott, Janice; Plant, Barry J.; McCrum-Gardner, Evie; McKeown, Stephanie; Elborn, J. Stuart; Bradley, Judy

doi:10.1016/j.jcf.2014.06.007

Cited by 12 publications

(8 citation statements)

References 26 publications

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“…At the time of AE, the mean variation of FEV1 relative to the best personal value was −0.26 ± 0.22 L, similar to several studies that reported decrease in FEV1 in a context of AE (McCourt et al., 2015; Sanders et al., 2011). Improvement of FEV1 value after AE treatment is also reported (Hoppe et al., 2018; Ren et al., 2006; Wagener et al., 2020).…”

Section: Discussionsupporting

confidence: 87%

Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults

Blin

Flament

Mankikian

et al. 2020

Clin Physio Funct Imaging

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Background Diagnosis of acute exacerbation (AE) of cystic fibrosis (CF) must be precise because both under‐ and over‐prescription of antibiotics may be detrimental. How lung function tests contribute to diagnose AE is unclear. We aimed to describe variation of spirometry and oscillometry measurements, at Stable state and at AE in adults with CF. Methods Patients were included in a retrospective single‐centre study when both spirometry (FEV1, FVC) and oscillometry (X5, R5, R5−R20 and AX) data were available for at least one Stable and one AE visit between December 2016 and July 2019. For each visit, we calculated variation (Δ) in spirometry and oscillometry indices in comparison with personal best values. Measurements were expressed as % of predicted values and Z‐scores when applicable. Areas under ROC curves (AUC) were computed. Results Forty‐two patients (28 ± 9 years, FEV1 64 ± 21%) were included; 80 AE and 104 Stable visits were analysed. FEV1 (L, %pred and Z‐score) and FVC (%pred and Z‐score) varied significantly between AE and Stable visits (p < .05), although differences were small (80 ml/2.7%pred for FEV1). Among oscillometry indices, X5 (kPa.s.L−1), R5–R20 (kPa.s.L−1) and AX (kPa/L) varied significantly. The AUCs for the variation in spirometry indices ranged from 0.601 (ΔFVC L) to 0.635 (ΔFEV1%pred). They were not significantly different from the AUCs for ΔX5 (0.589), ΔR5−R20 (0.649) and ΔAX (0.598). Conclusions Performance of both spirometry and oscillometry to discriminate AE from Stable state was poor. Variation of oscillometry indices (X5, R5−R20, AX) may be helpful when spirometry is unreliable or uncomfortable.

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Section: Discussionsupporting

confidence: 87%

Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults

Blin

Flament

Mankikian

et al. 2020

Clin Physio Funct Imaging

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“…The lung disease of CF is characterized by chronic persistent infection and inflammation that is punctuated by intermittent episodes of worsening signs and symptoms that are labeled as pulmonary exacerbations and typically treated with systemic antibiotics [9]. While it has long been appreciated that pulmonary exacerbations are a significant driver of the longterm loss of lung function in patients with CF [20,21], there is lack of consensus regarding their definition and treatment [9,18,22,23], leading to inconsistencies in both [6,10,18,24]. Registry analyses have documented the surprising proportion of exacerbations that are left untreated [6,25,26], especially in patients with relatively preserved baseline lung function [10], as well as the unsurprising fact that treatment mitigates a subsequent deterioration in ppFEV1 and the development of worsening signs and symptoms of lung disease [10,11].…”

Section: Discussionmentioning

confidence: 99%

Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis

Schechter

Schmidt

Williams

et al. 2018

Journal of Cystic Fibrosis

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Improvements in pulmonary outcomes can be accomplished rapidly using basic quality improvement principles, including interdisciplinary team goal setting, standardized and proactive approaches that ensure consistent recognition and treatment of pulmonary exacerbations, and the use of data to follow the effectiveness of the process. We believe that the steps involved would be easy for other CF Centers to adapt to their own settings.

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“…signs and symptoms) or administration of antibiotics as clinical endpoints 21 . For this study, an unvalidated modification of the Fuchs exacerbation endpoint was used.…”

Section: Discussionmentioning

confidence: 99%

A phase 3, multi-center, multinational, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of levofloxacin inhalation solution (APT-1026) in stable cystic fibrosis patients

Flume

VanDevanter

Morgan

et al. 2016

Journal of Cystic Fibrosis

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Indicators of pulmonary exacerbation in cystic fibrosis: A Delphi survey of patients and health professionals

Cited by 12 publications

References 26 publications

Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults

Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults

Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis

A phase 3, multi-center, multinational, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of levofloxacin inhalation solution (APT-1026) in stable cystic fibrosis patients

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