Individuals Patterns of Disclosure About Huntington s Disease (HD) and the Association With Adaptation to HD

This study will examine the ways in which people reveal their status as a carrier of
Huntington s disease (HD) or of being at risk for the disease. It will explore factors that
influence decisions about disclosure and how disclosure is made to family members, partners,
and close friends.

HD is an inherited, progressive disease. It causes nerve degeneration, motor disturbance,
loss of awareness, and psychiatric symptoms. Currently, no effective treatment is available
to prevent or delay HD progression. The mean age of onset is 35 to 44 years, and the median
survival rate after onset is 15 to 18 years. HD affects about 1 in 10,000 people in the
United States, so about 30,000 have HD and more than 200,000 are at risk. Predictive testing
for HD has been available since 1993. It can be a life-changing event to learn of being at
risk for HD. Disclosure has been studied among people with HD and other diseases, but
knowledge about the extent of nondisclosure and disclosure is limited. There is evidence that
a person s psychological adaptation to AD may be a factor. Adaptation involves processes that
help a person search for meaning in what has happened, attempt to gain control of his or her
life, and improve self-esteem in light of the threatening situation.

Participants ages 18 and older who have had a positive genetic test result more than 6 months
earlier regarding HD or who have a family history of HD but no predictive testing and who do
not have symptoms of HD may be eligible for this study. Recruitment is done through HD
clinics, support groups, and online websites and mailing listservs. About 260 people will be
in the study. Participants will complete a survey taking 30 to 40 minutes to do. Two survey
versions are available: for those who are gene carriers and for those at risk. Participants
are asked to complete the version applying to them. The survey can be done online or through
a hard copy to complete at home and send to NIH. This survey is anonymous.

Participants will list the adults with whom they have a relationship and up to 10 people they
interact with. They will indicate those who know about the HD gene or risk status. They will
also list those to whom they have personally made disclosure. The goal is to distinguish if
knowing the status or the act of disclosure is more important. Questions also involve
discussing the inheritance and features of HD, and participants feelings or concerns about HD
gene or risk status. Participants will be asked about their first disclosure experience, most
recent experience of it, and timing of disclosure the time between learning of HD status and
telling another person about it. There are also questions on decisions of nondisclosure,
negative and positive aspects of disclosure for participants, and what health care
professionals can do to help participants disclosure decisions.