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Rhinology/Allergy

Treating Patients With Cystic Fibrosis: Best Practices, Strategies, Updates 2023

Tuesday, October 3, 2023
1:00 PM - 2:00 PM East Coast USA Time
Location: 102AB

    Moderator(s)

  • Amanda L. Stapleton, MD

    Associate Professor of Otolaryngology
    Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center
    Pittsburgh, PA, United States

    • Panelist(s)

  • SL

    Stella E. Lee, MD

    Section Chief—Rhinology
    Division of Otolaryngology—Head & Neck Surgery, Brigham and Women’s Hospital, Harvard Medical School
    Weston, MA, United States

  • Brent A. Senior, MD

    Harold C. Pillsbury, III Distinguished Professor
    Department of Otolaryngology-Head & Neck Surgery
    Chapel Hill, NC, United States

  • Adam J. Kimple, MD/PhD

    Associate Professor
    UNC School of Medicine
    Chapel Hill, North Carolina, United States

Disclosure(s):
Amanda L. Stapleton, MD: No relevant relationships to disclose.
Stella E. Lee, MD: No relevant relationships to disclose.
Brent A. Senior, MD: No relevant relationships to disclose.
Adam J. Kimple, MD/PhD: No relevant relationships to disclose.
PROGRAM DESCRIPTION: Sixty percent of pediatric and adult patients with Cystic Fibrosis (CF) report chronic rhinosinusitis (CRS) symptoms when questioned and endoscopic and radiographic evidence of sinonasal inflammation is nearly universal. Frontal headaches, anosmia, obstruction, discharge, and congestion are all common to the CF population.
This panel of pediatric and adult rhinology experts will discuss the evaluation, medical management, imaging indications, surgical options, and long-term care of the pediatric and adult cystic fibrosis patient. New Cystic Fibrosis Foundation Otolaryngology Guidelines for Care will be presented and discussed.
The introduction of new Cystic Fibrosis Transmembrane Receptor (CFTR) corrector/modulator medical therapy will also be reviewed, outcome studies discussed, and clinical cases showcased.
Current treatments for Cystic Fibrosis Chronic Rhinosinusitis focus on surgeries to relieve mechanical obstruction, debride infected tissue, and improve drainage followed by topical antibiotic and/or steroid treatments. Repeated surgical procedures are commonly performed, and systemic antibiotics are unable to clear infections in this protected space. Nasal saline irrigations, topical steroids, and topical nasal antibiotic rinses are all in use, but have little data. The introduction of CFTR corrector/modulators has provided an additional medical treatment option for Cystic Fibrosis sinus disease. The combination of elexacaftor/tezacaftor/ivacaftor and ivacaftor is hoped to have significant improvement in CFTR function. With the 2019 FDA approval of this drug, members of the panel are currently prospectively following patients with Cystic FIbrosis Chronic Rhinosinusitis to determine if the new medication improves sinus disease while also improving lung function. Outcomes will be discussed and representative clinical cases showcased.

OUTCOME OBJECTIVE 1: Evaluate and treat patients with Cystic Fibrosis

OUTCOME OBJECTIVE 2: Understand how Cystic Fibrosis Transmembrane Receptor corrector/modulators work.

OUTCOME OBJECTIVE 3: Determine how to incorporate surgical intervention into Cystic Fibrosis care.

BACKGROUND STATEMENT: This panel contains expert Cystic Fibrosis Rhinologists. Their combined experience and knowledge of the treatment and care of Cystic Fibrosis patients will allow them to share the most up-to-date medical knowledge, surgical skills, and patient outcomes with the audience members.