This study will explore the factors that influence screening behaviors of adults diagnosed
with hereditary hemorrhagic telangiectasia (HHT), an inherited condition in which blood
vessel defects called arteriovenous malformations (AVMs) result in direct connections between
arteries and veins. Patients most commonly have small AVMs called telangiectases on the
tongue, face, hands, mouth, and throat and the mucosal linings of the nose and
gastrointestinal tract. Recurrent nosebleeds are a hallmark of the disease. Large AVMs can
also occur in various organs, causing sudden and life-threatening complications.
The study will examine how patients think and feel about their condition and what actions
they take to screen for internal symptoms of the disease.
Men and women 18 years of age and older who have HHT may be eligible for this study.
Participants fill out a 30-minute questionnaire, available in print or online, that includes
questions about the participant s
- beliefs about HHT
- actions taken to screen for internal symptoms of HHT
- experience with HHT
- current health status, family history and demographic information
The proposed study aims to understand the factors that influence screening behaviors of
adults with hereditary hemorrhagic telangiectasia (HHT). HHT is a chronic condition, but with
early diagnosis followed by adherence to recommended screening guidelines, the major
complications of this disorder can be avoided and disability or even death can be prevented.
Yet, it has come to the attention of healthcare professionals that the recommended screening
is not commonly followed by individuals with HHT, even when the risk of serious complications
is known. Nonadherence to screening recommendations is not unique to HHT. It is rather common
across chronic conditions, and genetic diseases, such as HHT, are no exception. However, HHT
may have an added barrier to screening and treatment adherence in that it is a rare and
underdiagnosed condition. Inadequate knowledge of healthcare providers may be a serious
barrier to prevention, diagnosis, and treatment. The Health Belief Model (HBM) can be used to
frame this study of HHT screening. The HBM posits that preventive health behaviors will be
acted upon if individuals regard themselves as susceptible to the threat, they believe the
consequences to be severe, and the perceived benefits outweigh the perceived barriers. In
addition to the HBM constructs, this study will also consider the role of illness
representations which provide a more personal view of the lived experience of individuals
with HHT. A cosss-sectional design will be used to investigate the relationships among the
domains of illness representations, HBM constructs (perceived susceptibility, perceived
benefits, barriers, self-efficacy, response efficacy, cues to action), and HHT-specific
screening guidelines. Participants will be recruited from the HHT Foundation International,
Inc., an outline HHT Awareness social group, and HHT Clinics. Participants will be asked to
complete either a web-based or a paper survey. The main outcome measure is screening
behaviors and intentions to screen for complications associated with HHT.
- INCLUSION CRITERIA:
Men and women who self-report having a diagnosis of HHT.
To read and write English.
Individuals younger than 18.