Evaluating FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis

Bouzek, Drake C.; Ren, Clement L.; Thompson, Misty; Slaven, James E.; Sanders, D.B.

doi:10.1002/ppul.25925

Cited by 16 publications

(13 citation statements)

References 43 publications

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“…The odds of a clinician-diagnosed RTE have recently been shown to increase when associated with reported symptoms and a drop in forced expiratory volume (FEV 1 ). However, evaluating FEV 1 decline and subsequent RTE management in non-cooperative children can be challenging ( 27 ).…”

Section: Physical Examinationmentioning

confidence: 99%

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Landini

Ciet²,

Janssens³

et al. 2023

Front. Pediatr.

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Respiratory tract exacerbations play a crucial role in progressive lung damage of people with cystic fibrosis, representing a major determinant in the loss of functional lung tissue, quality of life and patient survival. Detection and monitoring of respiratory tract exacerbations are challenging for clinicians, since under- and over-treatment convey several risks for the patient. Although various diagnostic and monitoring tools are available, their implementation is hampered by the current definition of respiratory tract exacerbation, which lacks objective “cut-offs” for clinical and lung function parameters. In particular, the latter shows a large variability, making the current 10% change in spirometry outcomes an unreliable threshold to detect exacerbation. Moreover, spirometry cannot be reliably performed in preschool children and new emerging tools, such as the forced oscillation technique, are still complementary and need more validation. Therefore, lung imaging is a key in providing respiratory tract exacerbation-related structural and functional information. However, imaging encompasses several diagnostic options, each with different advantages and limitations; for instance, conventional chest radiography, the most used radiological technique, may lack sensitivity and specificity in respiratory tract exacerbations diagnosis. Other methods, including computed tomography, positron emission tomography and magnetic resonance imaging, are limited by either radiation safety issues or the need for anesthesia in uncooperative patients. Finally, lung ultrasound has been proposed as a safe bedside option but it is highly operator-dependent and there is no strong evidence of its possible use during respiratory tract exacerbation. This review summarizes the clinical challenges of respiratory tract exacerbations in patients with cystic fibrosis with a special focus on imaging. Firstly, the definition of respiratory tract exacerbation is examined, while diagnostic and monitoring tools are briefly described to set the scene. This is followed by advantages and disadvantages of each imaging technique, concluding with a diagnostic imaging algorithm for disease monitoring during respiratory tract exacerbation in the cystic fibrosis patient.

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Section: Physical Examinationmentioning

confidence: 99%

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Landini

Ciet²,

Janssens³

et al. 2023

Front. Pediatr.

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“…Lung function is routinely undertaken to assess clinical stability and the impact of pulmonary exacerbations. 36 In pwCF, variation in lung function may also be a useful marker of adherence to both oral and inhaled medication. 11 In the prepandemic era, uptake of home monitoring systems was low, due to limited patients' engagement, lack of equipment, and inconsistency between home and clinic spirometry values.…”

Section: Lung Functionmentioning

confidence: 99%

Impact of Digital Technologies on Clinical Care for Adults with Cystic Fibrosis

Peckham

Spoletini

2022

Semin Respir Crit Care Med

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The coronavirus disease 2019 pandemic accelerated the implementation of digital technologies, which have now become embedded as essential tools for the management of chronic disease, including cystic fibrosis (CF). Despite subsequent easing of restrictions and because of improved clinical stability resulting from the introduction of highly effective modulator therapy, digital technologies including video and telephone consultations and remote monitoring are likely to remain integral to the future delivery of CF health care. In this article, we explore some of the key developments in digital technologies, barriers to their adoption, and how the CF community is likely to embrace lessons learned from the recent pandemic to help modernize and reshape the future of CF care.

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“…However, it is also under discussion whether the modified Fuchs BEx score is truly more applicable to the real world [ 19 , 20 ]. Recently, a decline in FEV1 (ΔFEV1) as modest as 5% or more could be associated with increased cough and/or sputum and clinician-diagnosed BEx in children with CF [ 21 ], questioning the requirement for ΔFEV1 ≥10%. This is particularly important in the HEMT era with lower sputum volumes and potentially milder FEV1 changes, where ΔFEV1 ≥10% may no longer prove sensitive enough.…”

Section: Introductionmentioning

confidence: 99%

Antibiotic Therapy for Pulmonary Exacerbations in Cystic Fibrosis—A Single-Centre Prospective Observational Study

Schwarz¹,

Wimmer

Holz

et al. 2023

Antibiotics

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People with cystic fibrosis experience bronchopulmonary exacerbations, leading to lung damage, lung function decline, increased mortality, and a poor health-related quality of life. To date, there are still open questions regarding the rationale for antibiotic use and the optimal duration of antibiotic therapy. This prospective single-center study (DRKS00012924) analyzes exacerbation treatment over 28 days in 96 pediatric and adult people with cystic fibrosis who started oral and/or intravenous antibiotic therapy in an inpatient or outpatient setting after clinician diagnosis of bronchopulmonary exacerbation. Biomarkers of exacerbation were examined in terms of their ability to predict response to treatment and the need for antibiotic therapy. The mean duration of antibiotic therapy was 14 days. Inpatient treatment was associated with a poorer health status, but no significant difference was found in the modified Fuchs exacerbation score between inpatients and outpatients. A significant increase of in-hospital FEV1, home spirometry FEV1, and body-mass index and a significant decrease of the modified Fuchs symptom score, C-reactive protein, and 8 out of the 12 domain scores of the revised cystic fibrosis questionnaire were demonstrated after 28 days. However, a trend towards a FEV1 decline in the inpatient group on day 28 could be demonstrated, while FEV1 was maintained in the outpatient group. Correlation analyses of changes between baseline and day 28 show a strong positive correlation between home spirometry and in-hospital FEV1, strong negative correlations between FEV1 and the modified Fuchs exacerbation score and between FEV1 and C-reactive protein, and a moderately negative correlation between FEV1 and the three domains of the revised cystic fibrosis questionnaire. Responders and non-responders to antibiotic therapy were defined in terms of FEV1 improvement after therapy. A higher baseline C-reactive protein, a greater decrease in C-reactive protein, a higher baseline modified Fuchs exacerbation score, and a greater decrease in the score after 28 days could be found in the responder group, while other baseline and follow-up parameters like FEV1 showed no significant differences. Our data show that the modified Fuchs exacerbation score is applicable in a clinical setting and can detect acute exacerbations regardless of health status. Home spirometry is a useful tool for outpatient exacerbation management. A change in C-reactive protein and a modified Fuchs score change are suitable follow-up markers of exacerbation due to their strong correlation with FEV1. Further studies are needed to assess which patients would benefit from a longer duration of antibiotic therapy. C-reactive protein at exacerbation onset and C-reactive protein decline during and after therapy better predict antibiotic therapy success than FEV1 at therapy onset, while the modified Fuchs score indicates exacerbation regardless of the need for antibiotic therapy, suggesting that antibiotic therapy is only part of exacerbation management.

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Evaluating FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis

Cited by 16 publications

References 43 publications

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging

Impact of Digital Technologies on Clinical Care for Adults with Cystic Fibrosis

Antibiotic Therapy for Pulmonary Exacerbations in Cystic Fibrosis—A Single-Centre Prospective Observational Study

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