Sinusitis and Olfaction Improve in Young Children with Cystic Fibrosis Treated with ETI

Janice J Chung1, Eugene Oh2, Michelle J Lee1

  • 1Department of Otolaryngology-Head and Neck Surgery, University of California, Los Angeles, Los Angeles, CA, USA.

Insights

Early therapy in children with cystic fibrosis improved smell function and reduced sinus issues. Quality of life remained stable, suggesting current tools may not fully capture treatment response in young patients.

Area of Science:

  • Pediatric Otolaryngology
  • Pulmonology
  • Medical Imaging

Background:

  • Cystic Fibrosis (CF) often involves sinonasal disease, potentially impacting olfactory function.
  • Early intervention in pediatric chronic rhinosinusitis (CRS) is crucial for long-term outcomes.
  • Olfactory dysfunction is a common but often overlooked comorbidity in pediatric CF.

Purpose of the Study:

  • To evaluate the efficacy of early therapeutic initiation in ameliorating olfactory dysfunction and CRS in young children with CF.
  • To assess the impact of treatment on sinonasal structure and olfactory bulb volume.
  • To determine changes in quality of life metrics following therapeutic intervention.

Main Methods:

  • A prospective study enrolled 31 participants aged 10 years or younger across six U.S. CF centers.
  • Data collection included baseline and one-year follow-up assessments of sinonasal imaging, olfactory psychophysical testing, and quality of life questionnaires.
  • Mixed-effects models were utilized for data analysis, adjusting for age, sex, and nasal steroid use.

Main Results:

  • Therapeutic initiation led to significant improvements in odor identification scores (p=0.03) and olfactory bulb volume (p=0.02).
  • Olfactory cleft opacification (p=0.004), sinus opacification (p=0.001), Lund-Mackay scores (p=0.002), and CRS-MRI scores (p=0.005) all showed significant improvement.
  • Quality of life scores remained stable and near-normal throughout the study period.

Conclusions:

  • Early therapy in young children with CF positively impacts olfactory function and sinonasal disease.
  • Objective measures show structural and functional improvements in the olfactory system and sinuses.
  • Current quality of life instruments may be insufficient to fully assess sinonasal disease and treatment response in this pediatric population.
Abstract

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