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Cited by 18Cystic Fibrosis Research AdvancesChronic Obstructive Pulmonary Disease (COPD) ResearchTransplantation: Methods and Outcomes

Poor recovery from cystic fibrosis pulmonary exacerbations is associated with poor long‐term outcomes

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Sanders, Don B., Zhao, Qianqian, Li, Zhanhai, Farrell, Philip M.·2017·Pediatric Pulmonology
DOI 10.1002/ppul.23765PMC PMC5639928
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Abstract

Rationale: People with CF treated with IV antibiotics for a pulmonary exacerbation (PEx) frequently fail to recover to baseline FEV 1. The long‐term impact of these events has not been studied. Objectives: To determine if a patient's spirometric recovery after a PEx is associated with time to next PEx within 1 year, the spirometric recovery after the next PEx, and/or the number of PEx episodes in the next 3 years. Methods: We used data from the CF Foundation Patient Registry from 2004 to 2011. We randomly selected one PEx per patient that met inclusion/exclusion criteria. Patients were defined as Non‐Responders if their best FEV 1 (in liters) recorded in the 3 months after the PEx was <90% of the best FEV 1 (in liters) in the 6 months before the PEx. We compared Responders and Non‐Responders using multivariable regression models. Results: We randomly chose 13 954 PEx episodes that met inclusion/exclusion criteria. A total of 2 762 (19.8%) patients were classified as Non‐Responders. Non‐Responders had a shorter median time to the next PEx, 235 (95%CI 218, 252) days, versus >365 days for Responders. Thirty‐four percent of Non‐Responders at the initial PEx were also Non‐Reponders at the next PEx, versus 20% of Responders at the initial PEx. Non‐Responders had more PEx episodes over the next 3 years, 4.99 (95%CI 4.84, 5.13), than Responders, 3.46 (95%CI 3.41, 3.51). Conclusions: Poor recovery after a PEx is associated with a shorter time to the next PEx, increased risk of poor recovery at a second PEx, and more frequent subsequent PEx treatments.

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  • Pulmonary exacerbations are important clinical events associated with disease progression.

    “Pulmonary exacerbations are important clinical events associated with disease progression21-24; elexacaftor–tezacaftor–ivacaftor resulted in a lower rate of pulmonary exacerbations, including severe events leading to hospitalization or treatment with intravenous antibiotics, than placebo.”

    From Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele (2019) · cites this paper as [24]

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Cited by

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Authors
Sanders, D. B., Zhao, Q., Li, Z., & Farrell, P. M.
Affiliations
  • Riley Hospital for Children (US)
  • University of Wisconsin–Madison (US)
Corresponding author
Sanders, Don B.
ORCID
Venue
Pediatric Pulmonology
Publisher
Wiley
Published
2017-09-07
Volume
52
Issue
10
Pages
1268-1275
ISSN
  • 1099-0496 (ISSN-L)
  • 8755-6863
DOI
10.1002/ppul.23765
PMC
PMC5639928
OpenAlex
W2753466150
Cited by
18
References
1
Citations by year
2025: 1 · 2024: 1 · 2023: 2 · 2022: 1 · 2021: 1 · 2020: 3 · 2019: 5 · 2018: 4
FWCI
2.71
Type
journal article
Language
en
Open access
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Pediatric Pulmonology (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
  • IUScholarWorks (Indiana University) (submittedVersion)
  • PMC
Funders
  • National Center for Advancing Translational Sciences (KL2TR000428, UL1 TR000427)
  • Cystic Fibrosis Foundation (SANDERS11A0)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
  • Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
Keywords
cystic fibrosis, pulmonary exacerbations, long-term outcomes, exacerbation recurrence, treatment response, lung function
MeSH terms
Adolescent, Adult, Anti-Bacterial Agents, Child, Cystic Fibrosis, Female, Forced Expiratory Volume, Humans, Male, Prognosis, Spirometry, Disease Progression, Young Adult

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