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Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients
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Real-world association between ivacaftor initiation and lung function variability: A registry study.

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This summary is machine-generated.

Ivacaftor treatment significantly reduced lung function variability in cystic fibrosis (CF) patients. This reduction in forced expiratory volume in 1 second % predicted (FEV1pp) variability may indicate treatment effectiveness.

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Area of Science:

  • Pulmonary Medicine
  • Pharmacology
  • Genetics

Background:

  • Increased lung function variability, specifically in forced expiratory volume in 1 second % predicted (FEV1pp), is linked to faster decline in cystic fibrosis (CF).
  • The impact of ivacaftor on FEV1pp variability has not been previously characterized.

Purpose of the Study:

  • To investigate the association between ivacaftor initiation and FEV1pp variability in individuals with CF.
  • To identify risk factors associated with increased FEV1pp variability.

Main Methods:

  • Analysis of the Cystic Fibrosis Foundation Patient Registry (2008-2020) data.
  • Utilized linear mixed effects models to compare pre- and post-ivacaftor FEV1pp variability using measures like maximum/median deviations and trendline standard deviations.
  • Included 527 individuals in the analysis cohort.

Main Results:

  • Ivacaftor initiation was associated with a reduction in FEV1pp variability (median reduction: 1.85 % predicted).
  • Reductions were most pronounced using maximum deviation from best FEV1pp, while trendline measures, particularly median deviation, showed consistent findings.
  • Risk factors for variability differed between pediatric and adult patients but were consistent across G551D and R117H subgroups. F508del homozygous patients showed minimal change.

Conclusions:

  • Evidence suggests ivacaftor initiation effectively reduces FEV1pp variability in people with CF.
  • Measuring FEV1pp variability could serve as a valuable marker for assessing ivacaftor's therapeutic efficacy.