A telehealth intervention has been developed and is being tested that is aimed at reducing social isolation and loneliness in young adults with Prader-Willi syndrome (PWS). This Vanderbilt Kennedy Center pilot study is the first to address a major risk factor in health and mental health problems experienced by persons with PWS.
PWS is a rare, complex genetic disorder affecting appetite, growth, metabolism, cognitive function, and behavior. It is typically characterized by low muscle tone, short stature (when not treated with growth hormone), incomplete sexual development, cognitive disabilities, and behavioral problems. The specific features of PWS vary from person to person, but nearly all people with this condition have a voracious appetite over which they have little control and a slowed metabolism, which can lead to life-threatening obesity.
“We know from research that adults with intellectual and developmental disabilities who are socially isolated and lonely are at increased risk for a whole range of negative health and mental health consequences, including those with Prader-Willi syndrome,” said Elisabeth Dykens, Ph.D., who is leading the study. Dykens is professor of Psychology, Psychiatry & Behavioral Sciences, and Pediatrics, and is a national expert on PWS and other genetic syndromes.
“Individuals with PWS or other developmental disabilities typically have fewer friendships and smaller social networks, often limited to immediate or extended family or paid caregivers. Moreover, they may experience rejection, bullying, and even abuse,” Dykens said.
The first aim of this pilot study, which is funded by the Foundation for Prader-Willi Research, is to create, adapt, and revise, as needed, a manualized intervention aimed at reducing social isolation and loneliness; and improving social interactions, skills, cognitions, and friendships in adolescents and young adults with PWS. The curriculum is called BOSS—Building Our Social Skills. The 10-week intervention, with three 30-minute sessions per week, is done with 5 to 8 young adults in a group format using telehealth technology. The group is led by two trained facilitators experienced in working with individuals with PWS and trained graduate students.
The second aim of the study is to assess the effectiveness of the BOSS intervention before, during, and after treatment, and in a 4-month follow-up period.
The intervention study began in February 2017 and will extend through the first half of 2018.
“Participants range in age from 16 to 26 years, the time when they move out of school and into the adult service system,” said Elizabeth Roof, PWS research coordinator and a group facilitator. “Young adults with intellectual and developmental disabilities—not just those with Prader-Willi syndrome—lose the services and supports and social networks that schools provide. After they leave school, they may have little to do during the day, and families often have difficulty navigating diffuse adult service systems. So these young adults are at high risk for social isolation and effects like depression.”
Since PWS is a rare genetic syndrome, telehealth allows the research team to have participants nationwide take part in the social group intervention.
“It seems paradoxical, perhaps, to use technology as a way to improve social interaction, but we are seeing it work well,” Roof said.
Initially, the sessions are led by the facilitators, but in later sessions, participants lead the session themselves, with facilitators redirecting as needed.
“We provide topics, such as reading facial cues or expressions to understand emotions, identifying one’s own feelings, how emotions color our experiences—which is a challenge, since it is not uncommon for persons with PWS mistakenly to perceive others as being negative. We aim to build basic skills like how to have conversations, initiating invitations to others, coming up with appropriate responses to questions or comments.”
Some sessions involve role-playing scenarios. First, facilitators act out the scenario; then participants act it out. Then a new scenario is provided for participants, without facilitators prompting in advance.
Participants use a social media platform that allows them to be in contact with one another outside the group sessions. The platform is curated by the facilitators and access is controlled, so that it is safe for participants.
Although data analysis has not yet begun, facilitators have observed improvements in how group members interact and connect.
“Ultimately, our goal is to show the effectiveness of a manualized intervention that improves quality of life, which would then be made available to the broader PWS community to implement on a larger scale,” Dykens said. “Moreover, it’s possible that the intervention curriculum could be adapted for other genetic syndromes to promote social skills using distance technology and to reduce social isolation.”
For additional information, contact email@example.com, PWS research coordinator.
Jan Rosemergy is VKC director of Communications and Dissemination.
Pictured top of page: Video conferencing allows VKC facilitators to connect small groups of young adults with Prader-Willi syndrome across the country as they learn social skills together.