AI Article Synopsis

  • The study investigates whether adding nebulized hypertonic saline (HS) to dornase alfa (DA) treatment improves lung function in cystic fibrosis (CF) patients compared to those receiving DA alone, specifically focusing on F508del homozygous patients.
  • A retrospective analysis of the Cystic Fibrosis Foundation Patient Registry from 2006-2014 included data from 1,241 patients who were treated for 1 to 5 years, analyzing changes in FEV % predicted between the two treatment groups.
  • Results indicated that while patients on DA and HS initially showed lower lung function compared to those on DA only, after adjusting for various factors, their lung function was comparable over time, suggesting that the HS treatment

Article Abstract

Background: Introduction of novel therapies for cystic fibrosis (CF) raises the question of whether traditional treatments can be withdrawn. Nebulized hypertonic saline (HS) potentially could be discontinued in patients receiving dornase alfa (DA).

Research Question: In the era before modulators, did people with CF who are F508del homozygous (CF) and who received DA and HS have better preserved lung function than those treated with DA only?

Study Design And Methods: Retrospective analysis of the Cystic Fibrosis Foundation Patient Registry data (2006-2014). Among 13,406 CF with data for at least 2 consecutive years, 1,241 CF had spirometry results and were treated with DA for 1 to 5 years without DA or HS during the preceding (baseline) year. Absolute FEV % predicted change while receiving DA and HS, relative to treatment with DA only, was the main outcome. A marginal structural model was applied to assess the effect of 1 to 5 years of HS treatment while controlling for time-dependent confounding.

Results: Of 1,241 CF, 619 patients (median baseline age, 14.6 years; interquartile range, 6-53 years) received DA only and 622 patients (median baseline age, 14.55 years; interquartile range, 6-48.1 years) were treated with DA and HS for 1 to 5 years. After 1 year, patients receiving DA and HS showed FEV % predicted that averaged 6.60% lower than that in patients treated with DA only (95% CI, -8.54% to -4.66%; P < .001). Lower lung function in the former relative to the latter persisted throughout follow-up, highlighting confounding by indication. After accounting for baseline age, sex, race, DA use duration, baseline and previous year's FEV % predicted, and time-varying clinical characteristics, patients treated with DA and HS for 1 to 5 years were similar to those treated with DA only regarding FEV % predicted (year 1: mean FEV % predicted change, +0.53% [95% CI, -0.66% to +1.71%; P = .38]; year 5: mean FEV % predicted change, -1.82% [95% CI, -4.01% to +0.36%; P = .10]).

Interpretation: In the era before modulators, CF showed no significant difference in lung function when nebulized HS was added to DA for 1 to 5 years.

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http://dx.doi.org/10.1016/j.chest.2023.05.024DOI Listing

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