Rendu-Osler-Weber-Syndrom: Aspekte der Lebensqualität bei Patienten mit chronisch-rezidivierender Epistaxis

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited vasculopathy, which can cause multiple symptoms. Leading symptom is chronic recurrent epistaxis. It was investigated, if the chronic recurrent epistaxis can be understood as an isolated symptom or if it has an effect on the health related quality of life (HrQoL). Furthermore we tried to determine to which extent HHT patients are impaired in their HrQoL compared to other chronic diseases. METHODS: This prospective cross-sectional study uses the German „Profil der LQ chronisch Kranker“ (PLC) – questionnaire plus a newly generated questionnaire dealing with HHT-specific symptoms (HHT-SFB). As a comparison normative PLC data of healthy Germans as well as PLC data of diverse chronic diseases were used. RESULTS: Results show, that 90% of the HHT-patients consider their recurrent epistaxis as the most compromising symptom. This results in an interference with hobbies and leisure time in 63% of the patients. Their professional life is negatively affected in 53% of the cases. HHT patients show worse results in 5 of 6 PLC scales compared to other chronic diseases like cardiomyopathy and rheumatism. Seventythree percent of the patients would appreciate new treatment concepts for epistaxis. CONCLUSION: Optimizing the treatment of chronic recurrent nosebleeds could help to improve HrQoL in HHT-patients. Presumed that enduring reduction of epistaxis in these patients can not be achieved in the near future, other aspects should be considered. The development of a specific psychological support would be favourable. Another approach could be psychosomatic care, which should convey relaxation techniques - which in return would reduce stress and consecutively epistaxis.