Effects of CFTR-modulator triple therapy on sinunasal symptoms in children and adults with cystic fibrosis

Article Critical Care Medicine

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles

Simon Y. Graeber et al.

Summary: Treatment with ELX/TEZ/IVA leads to effective improvement of CFTR function in airway and intestinal epithelia in patients with CF and one or two F508del alleles.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Otorhinolaryngology

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

Adam J. Kimple et al.

Summary: Cystic fibrosis requires otolaryngology care, and although there is some consensus on symptoms, there is still uncertainty about optimal management.

INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY (2022)

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Review Otorhinolaryngology

The management of cystic fibrosis chronic rhinosinusitis: An evidenced-based review with recommendations

Daniel B. Spielman et al.

Summary: This review evaluates the diagnosis and treatment approaches to cystic fibrosis chronic rhinosinusitis (CF CRS) and provides evidence-based recommendations for its management. Based on the available evidence, nasal saline, endoscopic sinus surgery (ESS), and cystic fibrosis transmembrane conductance regulator (CFTR) modulators are recommended for the management of CF CRS.

INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY (2022)

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Article Otorhinolaryngology

Association of Quality of Life Measures and Otolaryngologic Care in Cystic Fibrosis Patients

Stephen Leong et al.

Summary: The appropriate management of chronic rhinosinusitis (CRS) is crucial for improving quality of life among cystic fibrosis (CF) patients. Otolaryngologists play a critical role in reducing CRS symptom burden. This study evaluated the role of patient-reported quality-of-life measures in guiding interventions for CF-related sinus disease, showing that CF patients, especially those with poorer quality-of-life assessments, were more likely to have established coordinated care with an otolaryngologist. Further validation of SNOT-22 and QOD-NS questionnaires as care coordination metrics is necessary in the CF population.

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY (2022)

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Review Otorhinolaryngology

Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

Joanna Krajewska et al.

Summary: Chronic rhinosinusitis (CRS) is commonly seen in cystic fibrosis (CF) patients, with current treatment lacking precise guidelines or recommended therapeutic agents. Targeting the underlying genetic defect, drug therapy, and surgery may be effective in treating CF-related CRS, but further studies are needed to establish consensus for therapy.

EUROPEAN ARCHIVES OF OTO-RHINO-LARYNGOLOGY (2022)

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Article Respiratory System

Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy

Daniel M. Beswick et al.

Summary: This study evaluated the impact of Elexacaftor-tezacaftor-ivacaftor (ETI) on olfaction in people with cystic fibrosis. The results showed that after 6 months of ETI treatment, there was no improvement in quantitative olfaction or olfactory cleft opacification. However, patients with cystic fibrosis did not report impairment in olfactory quality of life. Further research is needed to explore the mechanisms of olfactory dysfunction and whether olfaction improves with longer duration of ETI treatment or in younger age groups.

JOURNAL OF CYSTIC FIBROSIS (2022)

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Article Respiratory System

Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaf tor/tezacaf tor/ivacaf tor therapy in adults with cystic fibrosis

Lena Wucherpfennig et al.

Summary: This study found that therapy with Elexacaftor/Tezacaftor/Ivacaftor can improve chest and sinus abnormalities in adult cystic fibrosis patients, indicating the reversibility of structural lung and sinus abnormalities in patients with established disease.

JOURNAL OF CYSTIC FIBROSIS (2022)

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Article Respiratory System

Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis

Sufyan Shakir et al.

Summary: Upper gastrointestinal and upper airway diseases are common in cystic fibrosis (CF) and may contribute to lower airway infection and inflammation. A longitudinal cohort study showed that treatment with CFTR modulators can improve respiratory reflux symptoms and sinonasal symptoms, as well as improve lung function and weight in patients with advanced CF.

JOURNAL OF CYSTIC FIBROSIS (2022)

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Review Medicine, General & Internal

Medical interventions for chronic rhinosinusitis in cystic fibrosis

Tulasi Kota Karanth et al.

Summary: This review aimed to compare the effects of different medical interventions in patients with cystic fibrosis and chronic rhinosinusitis. However, no eligible trials were found. High-quality trials are needed to assess the efficacy of these interventions in managing chronic rhinosinusitis, preventing pulmonary exacerbations, and improving quality of life in cystic fibrosis patients.

COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2022)

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Article Respiratory System

Impact of Cystic Fibrosis Transmembrane Conductance Regulator Therapy on Chronic Rhinosinusitis and Health Status Deep Learning CT Analysis and Patient-reported Outcomes

Daniel M. Beswick et al.

Summary: ETI has substantial improvements in sinus CT opacification and productivity loss, as well as clinically meaningful improvements in sinonasal QOL and health utility. The improvements were rapid, robust, and durable over the study.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2022)

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Article Respiratory System

Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis

Emily DiMango et al.

Summary: Elexacaftor-tezacaftor-ivacaftor significantly improves sinonasal symptoms in cystic fibrosis patients, with greater benefits observed in those who had previously used CFTR modulators.

JOURNAL OF CYSTIC FIBROSIS (2021)

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Editorial Material Otorhinolaryngology

Impact of novel CFTR modulator on sinonasal quality of life in adult patients with cystic fibrosis

Jennifer E. Douglas et al.

INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY (2021)

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Article Psychiatry

Assessment of health-related quality-of-life in patients with chronic Rhinosinusitis - Validation of the German Sino-Nasal Outcome Test-22 (German-SNOT-22)

D. Riedl et al.

Summary: The SNOT-22 questionnaire is a validated tool for assessing symptoms, health-related quality-of-life, and treatment response in patients with chronic rhinosinusitis. The German version of SNOT-22 demonstrated good reliability, validity, and responsiveness in German patients with CRS.

JOURNAL OF PSYCHOSOMATIC RESEARCH (2021)

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Article Otorhinolaryngology