This pilot study is to determine the safety and efficacy of oral sirolimus (blood trough
level 6-10ng/ml) in patients with HHT that are experiencing moderate or severe epistaxis. The
effect of oral sirolimus on epistaxis will be compared to baseline using the Patient-Reported
Outcome of cumulative weekly nose Bleeding Duration (PRO-CB). The PRO-CB association with
biomarker variability over the duration of the study will be investigated. In the pilot study
subjects will be treated with 2mg of sirolimus once daily to obtain a trough level of
6-10ng/ml for 3 months.