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The aim of this study is to identify the demographics and epistaxis burden of hereditary hemorrhagic telangiectasia (HHT). A questionnaire was sent to participants with HHT who were recruited from a prospectively maintained respiratory clinic data base in a tertiary hospital. Details on demographics, HHT symptoms, family history, epistaxis severity, and treatment received were recorded. There were 34 of 60 responses (57%). Two responses were from families of the deceased. Of the 32 evaluable patients (men, 14; women 18), the average age was 51 years (range, 23-78 years). The average age of HHT diagnosis was 31 years (range, 3-61 years). The diagnosis of HHT was made by the respiratory team in 13 patients; neurologist (2); ear, nose, and throat (ENT) specialist (4); general practitioner (5); hematologist (4); gastroenterologist (1); and not mentioned in two patients. Twenty-seven of 32 patients (84%) had a positive family history of HHT. Only 13 patients had formal genetic testing (4 endoglin, 1 activin receptor-like kinase, 8 unknown gene). All patients who presented to the respiratory clinic had a background of epistaxis, which was noted on presentation. The average age at initial epistaxis was 14 years (range, 2-50 years). The frequency of epistaxis was daily 63% (n = 20), weekly 9% (3), monthly 16% (5), and a few times a year 10% (3), and unstated in one patient. Nine of 32 patients (28%) required a transfusion. Six patients thought that they were unable to perform daily activities due to epistaxis. Only 15 of 32 patients (47%) were under the care of an ENT specialist. The treatment plan for epistaxis management was deemed good by 7 patients, adequate in 8, poor in 6, and not stated by 11 patients. In conclusion, this survey is the first to quantify the epistaxis burden within the northeast of England. The management of epistaxis needs specific education and treatment to optimize the quality of life among these patients.

A Postal Survey of Hereditary Hemorrhagic Telangectasia in the Northeast of England