Sinus Symptoms in Cystic Fibrosis Patients and the Role of Interdisciplinary Care

Chronic rhinosinusitis (CRS) is known to occur in most cystic fibrosis (CF) patients. This phenomenon occurs because the genetic mutations in CF cause impaired chloride ion transport, which affects water transport and thus leads to thick mucus that cannot be cleared easily in both the lower and upper airways. While improvements continue to be made in treatment modalities for CF patients in terms of modulator therapies, the effect on sinus disease symptoms and the role of the otolaryngologist (ENT) continues to evolve. This study investigates the sinus symptoms of CF patients with comparison to ENT involvement in their care, the histories of polyps and sinus surgeries of these CF patients, and how their initiation to modulator treatment has affected ENT involvement in their care. A retrospective health records review was conducted including patients from Augusta University Adult Cystic Fibrosis Center with stratification based on demographics, mutations, modulators, ENT clinic visits, history of polyps or nasal surgery, and reported symptoms of headaches, olfactory dysfunction, and sleep problems. 98 patients of the Augusta University Adult Cystic Fibrosis Center met the inclusion criteria of this study. Most of the patients with reported ENT visits in the past 10 years were white and between the ages of 20-29. The most common modulator therapy of the CF patients was elexacaftor-tezacaftor-ivacaftor at 72.22%. More than half of the patients had a history of sinonasal surgery, and almost half had a history of polyps. In comparing ENT visits to the initiation of modulator therapy, 24% of the patients with reported ENT visits saw an ENT after starting modulator therapy. CRS can contribute to morbidity in CF patients with most patients requiring surgical intervention in their lifetime. Additionally, persistent sinonasal symptoms can reduce the quality of life in CF patients. Despite treatment with modulator therapies, otolaryngologists continue to have a role in interdisciplinary CF management.CF- Cystic Fibrosis; CFTR- Cystic Fibrosis Transmembrane Regulator; CRS- Chronic Rhinosinusitis; EMR- Electronic Medical Record; ENT- Otolaryngologist