Sinus Symptoms in Cystic Fibrosis Patients on Modulator Therapy

Abstract

Background: Cystic fibrosis (CF) is a progressive, genetic disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that disables the body's ability to move chloride to the cell surface. This subsequently causes patients to have chronic sinusitis and a plethora of symptoms. With the advent of modulator therapies for many CF patients, pulmonary function and nutritional status have improved. Their effects on sinus disease as well as the changing role of the otolaryngologist (ENT) is not yet clearly defined. Methods: Adult cystic fibrosis patients from Augusta University Adult Cystic Fibrosis Center were included in this study, with stratification of patients based on their demographics, specific gene mutation, modulator therapy, ENT clinic visits, history of polyps, history of nasal surgery, headaches, olfactory dysfunction, and sleep problems. Data was abstracted from the Port CF database and the electronic medical record after obtaining local IRB approval. Results: Out of the 98 adult CF patients, 54 were seen by an ENT after 2012. Sixty-one of them had a history of nasal or sinus surgery (62.24%) and 45 had a history of polyps (45.92%). 52% of the total patients were seen between the age ranges 20-29 years, with an equal distribution of male and female patients (27 of each). The most common race was white (83.33%). 42.6% had the commonest mutation F508del/F508del. The most common modulator treatment was elexacaftor/tezacaftor/ivacaftor (72.22%). 24% of patients saw ENT after having started elexacaftor/tezacaftor/ivacaftor therapy. Discussion: Sinus disease can contribute to substantial morbidity in CF patients even on modulator therapy with the majority of patients requiring surgical intervention at some point in their lifetime. Almost half of the patients have polyp history, which are known to recur and are difficult to treat adequately. Additionally, many patients have sinonasal symptoms that are untreated. An otolaryngologist, therefore, remains an integral part of the CF team. Significance to the audience: This data will help us understand the role of ENT in the standard care of CF patients for the purpose of planning evaluations, referrals, and interventions in the near future.