Health Screening Behaviors among Adults with Hereditary Hemorrhagic Telangiectasia in North America

PURPOSE: This study aimed to identify factors that influence screening behaviors of adults with hereditary hemorrhagic telangiectasia (HHT). METHODS: Participants with a self-reported diagnosis of HHT were recruited from the HHT Foundation International, Inc., the “HHT Awareness” Facebook group, and six HHT clinics. A cross-sectional mixed methods survey was administered to investigate the relationships among the Health Belief model constructs, the domains of illness representations, and HHT-specific screening behaviors consistent with recommended guidelines. RESULTS: A total of 320 participants reported rates of cerebral arteriovenous malformation (AVM) screenings, pulmonary AVM screenings, and HHT annual checkups that were 82.0, 67.1, and 56.5%, respectively. Logistical regression analysis showed perceived barriers (β= -0.114, p<0.001), perceived susceptibility (β= 0.117, p<0.05), treatment control (β=0.078, p<0.05), and emotional representations (β= 0.067, p<0.05) were significant predictors of HHT screening. Open-ended responses revealed perceived barriers to screening, including a lack of healthcare providers (HCPs) familiar with and/or knowledgeable about HHT. CONCLUSION: Our results reveal sub-optimal screening rates among adults with HHT, and identify several factors influencing these behaviors. We suggest that there is a need for increased provider education regarding HHT as well as approaches that providers can use to improve screening adherence.