The Impact of COVID-19 in Cystic Fibrosis

Flume, Patrick A.; Saiman, Lisa; Marshall, Bruce C.

doi:10.1016/j.arbres.2021.12.003

Cited by 12 publications

(16 citation statements)

References 33 publications

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“… 17 Moreover, the COVID‐19 pandemic potentially affected the exacerbation rate of the patients, but a drop in exacerbation rate has previously been shown before the COVID‐19 pandemic. 18 This study also lacks data on medical adherence to assess the effect on lung function; patients were only interviewed about their use of ETI treatment. Another limitation to this study is the lack of control group; this was not feasible due to ethical considerations.…”

Section: Discussionmentioning

confidence: 99%

“…For some patients, home monitoring of lung function was possible to reduce missing values; however, this may have led to underestimation of ppFEV 1 due to the lack of support from the lung function technician 17 . Moreover, the COVID‐19 pandemic potentially affected the exacerbation rate of the patients, but a drop in exacerbation rate has previously been shown before the COVID‐19 pandemic 18 . This study also lacks data on medical adherence to assess the effect on lung function; patients were only interviewed about their use of ETI treatment.…”

Section: Discussionmentioning

confidence: 99%

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Real‐life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients

Neerincx

Fenn

et al. 2022

Pharmacology Res & Perspec

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Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane conductance regulator modulator, which has shown efficacy in CF patients (≥6 years) with ≥1 Phe508del mutation and a minimal function mutation. In October 2019, ETI became available on compassionate use basis for Dutch CF patients with severe lung disease. Our objective was to investigate safety and efficacy of ETI in this patient group in a real‐life setting. A multicenter longitudinal observational study was conducted to examine changes in FEV 1 , BMI, and adverse events at initiation and 1, 3, 6, and 12 months after starting ETI. The number of exacerbations was recorded in the 12 months before and the 12 months after ETI treatment. Patients eligible for compassionate use had a FEV 1 <40% predicted. Wilcoxon signed‐rank test analyzed changes over time. Twenty subjects were included and followed up for up to 12 months after starting ETI. Treatment was well tolerated with mild side effects reported, namely, rash (15%) and stomach ache (20%) with 80% resolving within 1 month. Mean absolute increase of FEV 1 was 11.8/13.7% ( p ≤ .001) and BMI was 0.49/1.87 kg/m 2 ( p < .001–0.02) after 1/12 months, respectively. In comparison to the number of exacerbations pretrial, there was a marked reduction in exacerbations after initiation. Our findings show long‐term effects of treatment with ETI in patients with severe CF lung disease in a real‐life setting. Treatment with ETI is associated with increased lung function and BMI, less exacerbations, and only mild side effects.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Real‐life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients

Neerincx

Fenn

et al. 2022

Pharmacology Res & Perspec

View full text Add to dashboard Cite

show abstract

“…Procedures such as respiratory function tests have been drastically reduced and remote attention such as telehealth, 120 , 121 , 122 , 123 home spirometry, 108 home collection of sputum samples, 124 home delivery of medication from the hospital pharmacy, and long-distance electronic monitoring 123 were required. This provided an opportunity to take new approaches to improving CF patients care with new and effective models, with durable changes in the future CF health care ( Fig.…”

Section: Cystic Fibrosismentioning

confidence: 99%

“…It is also important to highlight that fewer transplant 125 , 126 were performed although, in CF patients, the use of triple modulatory therapy just at the beginning of the pandemic was able to minimize the impact and severity of these patients. 123 , 127 …”

Section: Cystic Fibrosismentioning

confidence: 99%

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Short and Long-Term Impact of COVID-19 Infection on Previous Respiratory Diseases

Chiner-Vives

Cordovilla-Pérez

Rosa-Carrillo

et al. 2022

Archivos de Bronconeumología

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SARS‐CoV‐2 infection in children with cystic fibrosis: A cross‐sectional multicenter study in Spain. New waves, new knowledge

Mondejar‐Lopez,

Moreno‐Galarraga,

de Manuel‐Gomez

et al. 2023

Pediatric Pulmonology

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IntroductionThe association between viral infections and pulmonary exacerbations in children with cystic fibrosis (cwCF) is well established. However, the question of whether cwCF are at a higher risk of COVID‐19 or its adverse consequences remains controversial.MethodsWe conducted an observational, multicenter, cross‐sectional study of cwCF infected by severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV‐2) between March 2020 and June 2022, (first to sixth COVID‐19 pandemic waves) in Spain. The study aimed to describe patients' basal characteristics, SARS‐CoV‐2 clinical manifestations and outcomes, and whether there were differences across the pandemic waves.ResultsDuring study time, 351 SARS‐CoV2 infections were reported among 341 cwCF. Median age was 8.5 years (range 0–17) and 51% were female. Cases were unevenly distributed across the pandemic, with most cases (82%) clustered between November 2021 and June 2022 (sixth wave, also known as Omicron Wave due to the higher prevalence of this strain in that period in Spain). Most cwCF were asymptomatic (24.8%) or presented with mild Covid‐19 symptoms (72.9%). Among symptomatic, most prevalent symptoms were fever (62%) and increased cough (53%). Infection occurring along the sixth wave was the only independent risk factor for being symptomatic. Just eight cwCF needed hospital admission. No multisystem inflammatory syndrome, persisting symptoms, long‐term sequelae, or deaths were reported.ConclusionsSpanish current data indicate that cwCF do not experience higher risks of SARS‐CoV‐2 infection nor worse health outcomes or sequelae. Changes in patients' basal characteristics, clinical courses, and outcomes were detected across waves. While the pandemic continues, a worldwide monitoring of COVID‐19 in pediatric CF patients is needed.

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The Impact of COVID-19 in Cystic Fibrosis

Cited by 12 publications

References 33 publications

Real‐life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients

Real‐life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients

Short and Long-Term Impact of COVID-19 Infection on Previous Respiratory Diseases

SARS‐CoV‐2 infection in children with cystic fibrosis: A cross‐sectional multicenter study in Spain. New waves, new knowledge

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