Dental care course and oral quality of life in patients with X-linked hypophosphatemia: a qualitative exploration
Abstract Background. X-linked hypophosphatemia (XLH) is a rare, hereditary, progressive, and lifelong phosphate wasting disorder characterized by pathological elevations in fibroblast growth factor (FGF) 23 serum concentration and activity. In the oral cavity, spontaneous abscesses can occur often without any clinical signs of alteration of the causal tooth. The objective of our study was to evaluate the readability of the oral care pathway and the oral quality of life of patients followed in an expert oral medicine department located within a Parisian hospital and working in close collaboration with an endocrinology department expert in this pathology. Methods. This study employed a qualitative descriptive design including semi-structured interviews using guiding themes. Results. Twenty-one patients were included in the study, with an average age of 28 years. The topics discussed exceeded the initial objectives as the patients largely addressed the alteration of their oral and general quality of life, a very chaotic oral health care pathway with oral health professionals not aware of their pathology, consequences on their social, professional, and school integration and access to care complicated by financial factors. Patients reported the importance of having a multidisciplinary team around them, including medical and dental professionals.Conclusions. The variety of manifestations in patients with XLH necessitates a high coordination of multidisciplinary patient care to optimize quality of life and reduce disease burden. Education of oral health professionals on this disease is essential to enable patients to receive the best possible care as early as possible.