Description

Cystic Fibrosis (CF) is a rare hereditary disease with autosomal recessive transmission, affecting 1 in 4700 births in France. It is characterized by a mutation in the CFTR gene (Cystic Fibrosis Transmembrane conductance Regulator), which codes for a channel regulating chloride ion transport on the surface of epithelial cells in exocrine glands. While its primary impact is pulmonary, various systemic effects, including oral manifestations, are documented.

Several studies have explored the links between oral health and CF. They reveal the existence of hyposalivation associated with an increased risk of dental erosion, an elevated prevalence of enamel structural defects, and a reduced incidence of dental caries in CF children. Studies on the relationship between periodontal disease (periodontitis) and CF have shown no significant difference between CF patients and the general population. The oral quality of life in CF patients, measured by the OHIP-14 (Oral Health Impact Profile), a 14-item questionnaire, is also of interest, with only one study to date assessing this dimension in a population of minors.

These studies, often limited in scope, primarily focus on a CF population consisting mainly of children and young adults. In recent years, improved CF management has increased the median lifespan, exceeding 50 years (maximum age recorded in 2022: 85 years). New challenges arise due to the growing adult CF population. Additionally, the oral cavity acts as a bacterial reservoir at the entrance of airways, impacting chronic pulmonary conditions such as Chronic Obstructive Pulmonary Disease (COPD). Bacteria found in the oral environment (e.g. P. aeruginosa) are also identified in CF patients' lungs. The study of oral bacterial diseases in CF patients is of particular interest.

Therefore, to enhance knowledge on the links between oral health and CF and improve dental care for CF patients, updating the specific dental profile of this population with specific needs is necessary.

Objective and Methods : The proposed research is a questionnaire-based health data study aiming to improve knowledge of the links between oral health and CF. Due to early CF diagnosis, improved patient care, and innovative therapies in recent years, the median lifespan of CF patients has increased. While the disease traditionally affected only children, the adult population became the majority in France, representing nearly 60% in 2020. Yet, there is limited data on the oral health status of these patients, despite the importance of oral health for overall health and patient quality of life. The questionnaire seeks to collect data on socio-professional categories, medical history, dental care, periodontal/tooth wear/caries risk, mouth dryness, and oral quality of life, to identify real-life oral care needs for CF patients. The questionnaire, developed in collaboration with the "Vaincre La Mucoviscidose" association, uses reference tools from international literature :

Periodontal Risk Screening Score Tooth Decay/Tooth Wear Risk Score Oral Quality of Life OHIP-14 Mouth Dryness Shortened version of Xerostomia Inventory

Additionally, the study aims to assess whether CF patients follow the recommendations of the French Union for Oral Health 2022 for check-ups (2/year for chronic diseases) and good oral hygiene practices (2 brushings of 2 minutes/day with a soft toothbrush, daily interdental brushing, and fluoridated toothpaste). This study encompasses all adult CF patients in France, numbering over 4000 (4577 adult CF patients according to the French CF registry data report 2022 .The research is based on a Lime Survey questionnaire that will be distributed to all cystic fibrosis patients in France, via the association's newsletter and the Vaincre La Mucoviscidose social networks, as well as posters in the waiting rooms of these patients' care centers (Filière MucoCFTR). The research is monocentric, carried out by Prof. Gosset in the oral medicine department of Hôpital Charles Foix AP-HP.