Interviews reveal problems with hunger and anxiety in people with PWS
Adolescents and adults with Prader-Willi syndrome (PWS) say they constantly struggle with hunger and the urge to forage, according to an interview-based study.
Participants experienced difficulties with anxiety, behavioral outbursts, changes in schedule and education, many trying to distance themselves from the syndrome.
They also expressed the need for new drugs that reduce hunger and anxiety, or that can cure PWS.
The study, ““The cure for us is many things”: how do young people with Prader-Willi syndrome perceive themselves and do they perceive future clinical trials, ”Was published in the Journal of Applied Research in Intellectual Disability.
PWS is caused by the loss or defects of paternal genes that control metabolism, appetite, growth, sleep, intellectual ability, and social behavior.
Characteristic symptoms include an insatiable appetite, known as binge eating, which can lead to obesity. Behavioral issues such as temper tantrums, stubbornness, and obsessive-compulsive tendencies can also occur.
Although studies have focused on the self-perception of people with developmental disabilities, such as Down syndrome, few studies have explored how people with PWS perceive themselves. In addition, research is also needed on how patients perceive new therapies to treat their disease.
To gain a better understanding, researchers at Vanderbilt University in Tennessee interviewed 21 people with PWS, ages 14 to 38, asking how they understood their syndrome and its impact on their lives, and their views on the symptoms. new treatments.
All of the participants lived at home in the United States, 57% were female, and 52% were in high school. Of these, 35% were classified as normal weight, 25% as overweight and 40% as obese. A total of 10% had average intellectual abilities, 30% were below average, 40% were borderline intellectually disabled, 5% had mild intellectual disabilities, and 15% were moderately disabled.
Participants were first asked how they felt PWS affected them in their daily lives. Depending on the responses, they were asked questions about weight, anxiety, or behavioral seizures. People were then asked about the new medications and any other ideas about living with PWS that others should know about.
Most of the participants (71%) responded and engaged with the interviewers, while six had difficulty understanding the questions or expressing their thoughts.
All respondents stated that they lived with persistent hunger and frequent urges to forage and eat.
“Food calls out to me, I can’t think of anything else until I eat it,” said participant Emily.
Another, Kody, added, “Then in the morning the food is like waking me up, that’s the problem with me.”
Participants expressed their hunger and need for food in negative and emotionally charged language, using terms such as ‘really difficult’, ‘horrible’ and ‘upsetting’, as well as ‘life threatening’, “Like cancer” and “food is poison.”
“Like the food around me, it’s like a drug that kills me. Food is a poisonous drug that kills me, ”Thomas said.
Unlike the need for food, fewer participants (47%) said their weight was an issue. Although it is difficult to maintain or lose weight, the responses were less negative about hunger, “suggesting that weight doesn’t matter in relation to chronic hunger,” the researchers wrote.
In 40% of responses, people with PWS said their family helped them lose weight by making food inaccessible at home.
“Like my parents lock down the pantry and stuff and that’s for me,” Taylor said.
“Parents are telling their kids about PWS at the right age,” Kyle said. “Then you can help them manage the food. “
A majority (60%) said it was difficult to go out in the community.
Kaylee said, “You can’t go where there is food. “
And “there will usually be food around,” Gabe said.
James said, “Fast food is everywhere. I am surrounded by food that I shouldn’t be eating.
Participants also said that their hunger limited their relationships with friends and family, as well as their employment or education goals.
“The SPW affects the jobs you get and the way things are done in the community, you can’t go where there is food,” Kaylee said.
In total, 61% of those surveyed had strategies to manage their hunger and desire to forage for food, including getting help from friends, using social limitations against food theft, and developing eating methods.
“Sometimes I put notes on my hands,” Gabe said. “To remember things. So tomorrow there is a girls athletics competition and there will be cookies. So the note warned me not to go.
Most (84%) said PWS signified persistent difficulty controlling anxiety, anger, and stress. Participants used a variety of terms, including “stressed”, “worried”, “anxious”, “angry”, “crazy”, “angry”, “bad” and “occasionally sad”.
“I get nervous all day,” Sophie said.
“Sometimes I just lose my temper over something or I get emotional,” Rose said. “It’s like crazy the way I talk or anything.”
Some participants said their worries and seizures were due to feeling hungry, refusing food, parenting provocations and thinking problems, as well as unforeseen changes in their routines or plans.
“Anytime there’s a change it just pisses me off,” Blake said. “When they change it to me and don’t tell me. It makes me angry. When they tell me ahead of time, I’m fine.
Most said attending school was stressful, triggered anxiety, and was a negative experience. Difficulties included problems with learning, homework, testing and being teased. Those out of school made most of the comments, “suggesting that schooling remains emotionally important to them even as young adults,” the scientists wrote.
“When I’m in school and working on something I’m not sure how to do, PWS affects the way I think or get things done,” Rose said.
“The kids laughed at me and called me fat,” James said. “It made me sad, brought me down.”
A majority (61%) said they distanced themselves from PWS, with some denying they had PWS, while others wanted it to move away or downplayed the impact of being overweight. Some have suggested that PWS is not what they are, but it is the syndrome that takes them over and is responsible for overeating and temper tantrums.
“It’s like I’m getting too out of control, I can do bad things,” Max said. “Yes, and it’s not myself, it’s the PWS. “
“I won’t be hungry, but my Prader-Willi will tell my brain that I’m still hungry,” Anna said.
All respondents supported the need for new therapies to treat PWS, with 80% saying they wanted medications that control their hunger and facilitate weight loss.
“Life would be different because you don’t think about food all the time,” Kody said. Or as Gabe noted, “You can do things without fighting yourself. “
A total of 67% said new drugs would make them happier, less stressed and anxious, while 47% said how much reducing hunger or anxiety would help relationships. Likewise, 60% described the potential positive outcomes of the new drugs, such as meeting their life goals, living independently, having more employment opportunities, and attending college or vocational training programs.
“It would help people like us to be more independent in the long run,” Katlyn said. “Like working in a job where there is food. I could go to school myself. I want to go to college. “
Although the interviews did not use the word “cure,” 47% saw the newer drugs as a cure for PWS. “Cure hunger and completely cure the urge to forage,” said Jacob.
Several participants (47%) expressed the need for greater awareness among the public and companies developing therapies for PWS. This, the researchers wrote, indicated that “they understand that their disease is rare and that increased knowledge is an important step towards the development of new drugs.”
One study participant, Thomas, said, “I want them to know about SPW and get it all over the news! “
“I want the drug companies to allow everyone to study,” Jacob said. “I can’t do a lot of them because I’m not in that age group.”
Finally, the researchers carried out a statistical analysis and found no significant correlation between the topics covered and age, genetics of PWS, body weight or cognitive abilities. However, men were about four times more likely to stray from PWS than women (68% vs. 17%).
“This study is the first to give a voice to people with PWS, and how they perceive their condition and the need for new pharmacological treatments,” the investigators wrote. “Participants explained how hard they work each day to deal with the many challenges of their syndrome. “
“At the same time, they also envision future goals and a better life for themselves and their families, reminding us that people with disabilities are more than their limitations or chromosomal abnormalities,” they added.