Improvement in epistaxis management: the experience of a dedicated hereditary haemorrhagic telangiectasia clinic.

Abstract

Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic vascular disorder which is characterised by the development of arteriovenous malformations and telangiectasias. A key clinical manifestation is recurrent epistaxis. This study examined the impact of a dedicated HHT clinic in a major Australian tertiary hospital on epistaxis symptoms and subjective quality of life. A multidisciplinary HHT clinic was established in 2015. All patients satisfied either genetic diagnosis or fulfilled Curacao criteria. A protocol based clinical assessment was performed and a standardised treatment regimen was implemented. Patients completed quality of life and epistaxis severity score (ESS) questionnaires at each review. Participants of the dedicated clinic included 21 females (58.3%) and 15 males (41.7%), with a mean age of 49.0 ± 24.0 years. The ACVRL1 variant was the most common (n= 20, 55.6%). A statistically significant reduction in epistaxis severity was noted between the baseline and second review (P= 0.02) and was maintained to the third visit (P= 0.015). Patients older than 50 years demonstrate a consistently higher ESS than those less than 50 years old (P= 0.03). This trend is noted throughout the follow up period with the dedicated clinic. The introduction of a multidisciplinary, dedicated HHT clinic to provide enhanced assessment, monitored treatment regimen and greater access to care, resulted in improvement in the management of epistaxis and quality of life in these patients.