Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

Adam J Kimple¹, Brent A Senior¹, Edward T Naureckas², David A Gudis³, Ted Meyer^{1

4}, Sarah E Hempstead⁵, Helaine E Resnick⁶, Dana Albon⁷, Wayne Barfield⁸, Margo McKenna Benoit⁹, Daniel M Beswick¹⁰, Eliza Callard¹¹, Shelagh Cofer¹², Veronica Downer¹³, E Claire Elson¹⁴, Angela Garinis¹⁵, Ashleigh Halderman¹⁶, Lisa Hamburger¹¹, Meagan Helmick¹¹, Michael McCown¹⁷, Cameron J McKinzie¹⁸, Hanna Phan¹⁹, Kenneth Rodriguez²⁰, Ronald C Rubenstein²¹, Ashley Severin²², Gopi Shah¹⁶, Ambika Shenoy²³, Brittney Sprouse^{23

24}, Frank Virgin²⁵, Bradford A Woodworth²⁶, Stella E Lee²⁷

Affiliations

¹ Department of Otolaryngology/Head & Neck Surgery, The University of North Carolina, Chapel Hill, North Carolina, USA.
² Department of Pulmonary Medicine, Critical Care Medicine, University of Chicago Medicine, Chicago, Illinois, USA.
³ Department of Otolaryngology - Head and Neck Surgery, Columbia University Irving Medical Center/New York-Presbyterian Hospital, New York, New York, USA.
⁴ Department of Otolaryngology - Head and Neck Surgery, Medical University of South Carolina, South Carolina, USA.
⁵ Cystic Fibrosis Foundation, Bethesda, Maryland, USA.
⁶ Resnick, Chodorow & Associates, USA.
⁷ Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Virginia, Charlottesville, Virginia, USA.
⁸ Pediatric and Adult CF Center, Medical University of South Carolina, South Carolina, USA.
⁹ Department of Otolaryngology-Head and Neck Surgery, University of Rochester Medical Center, Rochester, New York, USA.
¹⁰ Department of Head and Neck Surgery, University of California, Los Angeles, California, USA.
¹¹ Community Advisor to the Cystic Fibrosis Foundation, Bethesda, Maryland, USA.
¹² Mayo Clinic-Otolaryngology (ENT)/Head and Neck Surgery, Rochester, Minnesota, USA.
¹³ Michigan Medicine, University of Michigan, Michigan, USA.
¹⁴ Department of Pharmacy, Children's Mercy Kansas City, Kansas City, Missouri, USA.
¹⁵ Oregon Hearing Research Center, Oregon Health & Science University, Portland, Oregon, USA.
¹⁶ Department of Otolaryngology/Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
¹⁷ Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.
¹⁸ Department of Pharmacy, University of North Carolina Medical Center, Chapel Hill, North Carolina, USA.
¹⁹ College of Pharmacy, Department of Clinical Pharmacy, The University of Michigan, C.S. Mott Children's Hospital, Michigan Medicine, Michigan, USA.
²⁰ Department of Otolaryngology, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA.
²¹ Allergy and Pulmonary Medicine, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
²² Department of Social Work, Children's Mercy Kansas City, Kansas City, Missouri, USA.
²³ Department of Pediatrics, Division of Pediatric Pulmonology, Nemours Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.
²⁴ University of Chicago Medicine, Chicago, Illinois, USA.
²⁵ Department of Otolaryngology - Head and Neck Surgery, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee, USA.
²⁶ Department of Otolaryngology - Head and Neck Surgery, University of Alabama at Birmingham, Birmingham, Alabama, USA.
²⁷ Brigham and Women's Hospital, Division of Otolaryngology-Head & Neck Surgery, Harvard Medical School, Boston, Massachusetts, USA.

PMID: 35089650
PMCID: PMC9545592
DOI: 10.1002/alr.22974

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

Adam J Kimple et al. Int Forum Allergy Rhinol. 2022 Sep.

. 2022 Sep;12(9):1089-1103.

doi: 10.1002/alr.22974. Epub 2022 Feb 22.

Authors

Affiliations

¹ Department of Otolaryngology/Head & Neck Surgery, The University of North Carolina, Chapel Hill, North Carolina, USA.
² Department of Pulmonary Medicine, Critical Care Medicine, University of Chicago Medicine, Chicago, Illinois, USA.
³ Department of Otolaryngology - Head and Neck Surgery, Columbia University Irving Medical Center/New York-Presbyterian Hospital, New York, New York, USA.
⁴ Department of Otolaryngology - Head and Neck Surgery, Medical University of South Carolina, South Carolina, USA.
⁵ Cystic Fibrosis Foundation, Bethesda, Maryland, USA.
⁶ Resnick, Chodorow & Associates, USA.
⁷ Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Virginia, Charlottesville, Virginia, USA.
⁸ Pediatric and Adult CF Center, Medical University of South Carolina, South Carolina, USA.
⁹ Department of Otolaryngology-Head and Neck Surgery, University of Rochester Medical Center, Rochester, New York, USA.
¹⁰ Department of Head and Neck Surgery, University of California, Los Angeles, California, USA.
¹¹ Community Advisor to the Cystic Fibrosis Foundation, Bethesda, Maryland, USA.
¹² Mayo Clinic-Otolaryngology (ENT)/Head and Neck Surgery, Rochester, Minnesota, USA.
¹³ Michigan Medicine, University of Michigan, Michigan, USA.
¹⁴ Department of Pharmacy, Children's Mercy Kansas City, Kansas City, Missouri, USA.
¹⁵ Oregon Hearing Research Center, Oregon Health & Science University, Portland, Oregon, USA.
¹⁶ Department of Otolaryngology/Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
¹⁷ Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.
¹⁸ Department of Pharmacy, University of North Carolina Medical Center, Chapel Hill, North Carolina, USA.
¹⁹ College of Pharmacy, Department of Clinical Pharmacy, The University of Michigan, C.S. Mott Children's Hospital, Michigan Medicine, Michigan, USA.
²⁰ Department of Otolaryngology, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA.
²¹ Allergy and Pulmonary Medicine, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
²² Department of Social Work, Children's Mercy Kansas City, Kansas City, Missouri, USA.
²³ Department of Pediatrics, Division of Pediatric Pulmonology, Nemours Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.
²⁴ University of Chicago Medicine, Chicago, Illinois, USA.
²⁵ Department of Otolaryngology - Head and Neck Surgery, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee, USA.
²⁶ Department of Otolaryngology - Head and Neck Surgery, University of Alabama at Birmingham, Birmingham, Alabama, USA.
²⁷ Brigham and Women's Hospital, Division of Otolaryngology-Head & Neck Surgery, Harvard Medical School, Boston, Massachusetts, USA.

PMID: 35089650
PMCID: PMC9545592
DOI: 10.1002/alr.22974

Abstract

Background: Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing loss and dysphonia. Given these manifestations of CF, otolaryngologists are frequently involved in the care of patients with CF; however, there is limited consensus on optimal management of sinonasal, otologic, and laryngologic symptoms.

Methods: The Cystic Fibrosis Foundation convened a multidisciplinary team of otolaryngologists, pulmonologists, audiologists, pharmacists, a social worker, a nurse coordinator, a respiratory therapist, two adults with CF, and a caregiver of a child with CF to develop consensus recommendations. Workgroups developed draft recommendation statements based on a systematic literature review, and a ≥80% consensus was required for acceptance of each recommendation statement.

Results: The committee voted on 25 statements. Eleven statements were adopted recommending a treatment or intervention, while five statements were formulated recommending against a specific treatment or intervention. The committee recommended eight statements as an option for select patients in certain circumstances, and one statement did not reach consensus.

Conclusion: These multidisciplinary consensus recommendations will help providers navigate decisions related to otolaryngology consultation, medical and surgical management of CF-CRS, hearing, and voice in individuals with CF. A collaborative and multidisciplinary approach is advocated to best care for our patients with CF. Future clinical research is needed utilizing standardized, validated outcomes with comprehensive reporting of patient outcome, effects of modulator therapies, and genetic characteristics to help continue to advance care, decrease morbidity, and improve the quality of life for individuals with CF.

Keywords: FESS; chronic rhinosinusitis; cystic fibrosis; dysphonia; evidence-based medicine; sinus; voice and allergy.

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References

1. Crawford I, Maloney PC, Zeitlin PL, et al. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci USA. 1991;88(20):9262–9266. - PMC - PubMed
1. Denning GM, Ostedgaard LS, Cheng SH, Smith AE, Welsh MJ. Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia. J Clin Invest. 1992;89(1):339–349. - PMC - PubMed
1. Trezise AE, Buchwald M. In vivo cell‐specific expression of the cystic fibrosis transmembrane conductance regulator. Nature. 1991;353(6343):434–437. - PubMed
1. Tipirneni KE, Woodworth BA. Medical and surgical advancements in the management of cystic fibrosis chronic rhinosinusitis. Curr Otorhinolaryngol Rep. 2017;5(1):24–34. - PMC - PubMed
1. Fokkens WJ, Lund VJ, Hopkins C, et al. European Position Paper on rhinosinusitis and nasal polyps 2020. Rhinology. 2020;58(Suppl S29):1–464. - PubMed

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Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

Affiliations

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

Authors

Affiliations

Abstract

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical