title: Health screening behaviors among adults with hereditary hemorrhagic telangiectasia in North America.
abstract: This study aimed to identify factors that influence screening behaviors of adults with hereditary hemorrhagic telangiectasia (HHT).
  
  
  
  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.
  
  
  
  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 that 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 health-care providers (HCPs) familiar with and/or knowledgeable about HHT.
  
  
  
  Our results reveal suboptimal 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.Genet Med advance online publication 13 October 2016.
authors.affiliation: Social and Behavioral Research Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.|Current address: GeneDx, Elmwood Park, New Jersey, USA.
authors.forename: Melanie
authors.identifier: 0000-0002-0752-1656
authors.initials: M
authors.lastname: Baxter
authors.affiliation: Social and Behavioral Research Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Lori
authors.identifier: 0000-0001-6200-9033
authors.initials: L
authors.lastname: Erby
authors.affiliation: Health, Behavior and Society, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA.
authors.forename: Debra
authors.initials: D
authors.lastname: Roter
authors.affiliation: Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Barbara A
authors.identifier: 0000-0002-9096-8765
authors.initials: BA
authors.lastname: Bernhardt
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Terry
authors.affiliation: The Permanent Fund for Vermont's Children, Burlington, Vermont, USA.
authors.forename: Alan
authors.identifier: 0000-0002-9746-3145
authors.initials: A
authors.lastname: Guttmacher
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003430:Cross-Sectional Studies; D005260:Female; D007722:Health Knowledge, Attitudes, Practice; D006801:Humans; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D010342:Patient Acceptance of Health Care; D010349:Patient Compliance; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis* / Q000523:psychology; D055815:Young Adult