Kimple AJ, Senior, BA, Naureckas ET, et al. Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations. Int Forum Allergy Rhinol. 2022. Sep;12(9):1089-1103. doi: 10.1002/alr.22974. Epub 2022 Feb 22 Jan 28. doi: 10.1002/alr.22974.
Cystic fibrosis is a multisystemic disease that has significant otolaryngologic manifestations. The complete care of patients with CF should include screening and management by an otolaryngologist. However, there are no current guidelines for screening, management, or treatment of pediatric and adult people with CF who have sinonasal, otologic, or laryngologic manifestations of their disease. Chronic rhinosinusitis (CRS), hearing loss, and dysphonia (hoarseness) are three prevalent disease pathologies that that are typically evaluated and managed by an otolaryngologist. The goal of this work group was to create consensus statement recommendations to help guide optimal care for children and adults with CF with otolaryngologic disease.
The Cystic Fibrosis Foundation created a 29-person multidisciplinary task force 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 for children and adults with CF regarding their sinonasal, otologic, and laryngologic symptoms. The committee members developed and then voted on recommendation statements. Due to the limited evidence to support clinical management of otolaryngology symptoms, the committee agreed to issue recommendations:
- For an intervention.
- Against an intervention.
- As an option for select patients.
Agreement by 80% of all committee members was required via voting for approval of statements in the recommendation document.
For all recommendations and further detail, please see the published manuscript.
Screening and Diagnosis
|Recommendation||Evaluation of the Evidence|
1. The CFF recommends that CF infection prevention and control guidelines be followed for children and adults with CF being seen by an otolaryngology team.
|2.The CFF recommends otolaryngology consultation for children and adults with CF with persistent ear, nose, and throat symptoms.||100% Consensus|
|3.The CFF recommends the administration of a sinonasal quality-of-life tool to children and adults with CF (e.g., SN-5 for ages 6–12 years and SNOT-22 for ages 13 or older), to identify sinonasal symptoms.||93.1% Consensus|
|4. The CFF recommends baseline hearing study for ototoxic monitoring for all children and adults with CF in anticipation of receiving ototoxic therapies.||96.55% Consensus|
|5. The CFF recommends ototoxic monitoring annually for children and adults with CF who are exposed to ototoxic medications.||100% Consensus|
|6. The CFF recommends ototoxic monitoring following each course of intravenous ototoxic medications for children and adults with CF who already have any hearing loss.||89.66% Consensus|
|7. The CFF recommends voice evaluation and management for children and adults with CF and dysphonia.||100% Consensus|
|8. The CFF recommends nasal saline irrigation for children and adults with CF with signs or symptoms of CRS.||100% Consensus|
|9. The CFF recommends the treatment of allergic rhinitis, including topical nasal corticosteroids, to improve nasal symptoms in children and adults with CF and concomitant allergic rhinitis.||100% Consensus|
|10. The CFF recommends endoscopic sinus surgery for children and adults with CF who have symptomatic CRS refractory to appropriate medical therapy.||100% Consensus|
|11. The CFF recommends that perioperative airway clearance therapy be continued as tolerated in children and adults with CF who undergo endoscopic sinus surgery for CRS.||100% Consensus|
|12. The CFF recommends against the routine use of systemic corticosteroids for CF-CRS in children and adults.||100% Consensus|
|13. The CFF recommends against the routine use of intranasal corticosteroids administered by nebulizers in children and adults with CF-CRS.||96.55% Consensus|
|14. The CFF recommends against performing routine endoscopic sinus surgery for children and adults with CF for the sole indication of declining lung function.||100% Consensus|
|15. The CFF recommends against performing routine adenoidectomy alone for the treatment of CRS in children with CF.||89.66% Consensus|
|16. The CFF recommends against performing routine balloon sinuplasty for children and adults with CF-CRS.||100% Consensus|
Options for Management
When there was general agreement of the committee that the benefits of an intervention would outweigh the risks for select patients but there was limited or no evidence, the committee voted to “acknowledge as an option.” These interventions are considered reasonable clinical management for some patients and clinical scenarios but may not apply broadly to all children and adults with CF.
|Recommendation||Evaluation of Evidence|
|17. The CFF acknowledges surveillance visits with an otolaryngologist in the absence of ear, nose, and throat symptoms as an option.||86.21% consensus|
|18. The ear, nose, and throat guidelines committee acknowledges the use of CFTR modulator therapy for treatment of CF-CRS when appropriate modulator therapy is available for age and genotype as an option.||100% consensus|
|19. The CFF acknowledges the use of daily intranasal corticosteroid spray for children and adults with CF-CRS as an option||100% consensus|
|20. The CFF acknowledges extended sinus surgery in children and adults with CF and CRS refractory to appropriate medical therapy as an option.||100% consensus|
|21. The CFF acknowledges perioperative intravenous antibiotics for children and adults with CF who undergo endoscopic sinus surgery for CRS after discussion with the multidisciplinary CF care team as an option.||100% consensus|
|22. The CFF acknowledges the use of intranasal dornase alfa nebulization for treatment of CRS in children and adults with CF.||89.66% consensus|
|23. The CFF acknowledges the use of sinonasal topical antibiotic treatment in children and adults with CF who previously had endoscopic surgery as an option.||100% consensus|
|24. The CFF acknowledges ototoxic monitoring following each course of intravenous ototoxic medications as an option.||82.76% consensus|
- Determine the effects CFTR modulator therapy on CRS.
- Determine if topical medications delivered to the nose and sinuses, including antibiotics, steroids, or saline, provide benefit in individuals with CF.
- Further our understanding of how the upper airway influences the lower airway microbiologically and functionally.
- Determine if audiologic screening influences clinical practice and changes long-term hearing outcomes.
- Determine if audiologic screening results in patients getting earlier hearing rehabilitation.
- Determine if voice therapy help individuals with CF and dysphonia.
- Stapleton AL, Kimple AJ, Goralski JL, et al. Elexacaftor-Tezacaftor-Ivacaftor improves sinonasal outcomes in cystic fibrosis. J Cyst Fibros. 2022 Mar 14:S1569-1993(22)00051-0. doi: 10.1016/j.jcf.2022.03.002. Online ahead of print.
- Spielman DB, Beswick DM, Kimple AJ, et al. The management of cystic fibrosis chronic rhinosinusitis: An evidenced-based review with recommendations. Int Forum Allergy Rhinol. 2021 Dec 21. doi: 10.1002/alr.22953. Online ahead of print.
- Ji KSY, Frank-Ito D, Abi Hachem R, et al. Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes. Am J Rhinol Allergy. 2021 Nov 20:19458924211059606. doi: 10.1177/19458924211059606. Online ahead of print.
- Bacon DR, Stapleton A, Goralski JL, et al. Olfaction before and after initiation of elexacaftor-tezacaftor-ivacaftor in a cystic fibrosis cohort. Int Forum Allergy Rhinol. 2022 Feb;12(2):223-226. doi: 10.1002/alr.22891. Epub 2021 Oct 28.
This executive summary was prepared by:
Amanda L. Stapleton, MD (University of Pittsburgh), and Adam J. Kimple, MD, PhD (University of North Carolina)