Gioya Noser has constant and life-threatening hunger due to a rare syndrome

The refrigerator and kitchen cabinets are locked with combination locks. There is only a jug of water for Gioya Noser on the dining table of the family home on the edge of the canton of Solothurn. No cookies. “I couldn’t control myself and ate everything,” says Gioya Noser, and her mother, sitting next to her, nods knowingly. Haven’t we all said this phrase while having fun with chips or nuts? But Gioya is deadly serious. Your life even depends on it. The 21-year-old has Prader-Willi syndrome.

“Something is broken in my brain,” says Gioya. You are always hungry. belly growling hunger. “I could eat five pizzas at once,” she shrugs. After eating, the feeling of satiety lasts only a short time. Then your brain asks for more, even though your stomach is full. In the worst case, Gioya has to break. The hunger returns a little later.

Some of the paternal genetic information is missing in people with Prader-Willi syndrome. The result is permanent damage to the hypothalamus in the brain. This is, as it were, the control center of our nervous system. It not only regulates hunger and satiety, but also body temperature, hormone balance, sleep and sexual behavior.

We know all about this thanks to three Zurich pediatricians – Andrea Prader, Heinrich Willi and Alexis Labhart – who researched the syndrome in 1956 and gave it its name. The doctor Urs Eiholzer was Prader’s last student and has been researching the syndrome for 30 years. He knows all about 200 affected people living in Switzerland. Also Gioya Noser.

About five children with disabilities are born in Switzerland each year. “Mothers notice during pregnancy that something is not right,” says Eiholzer. The embryo barely moves. The newborns looked floppy and weak. They usually just sleep, don’t cry and have trouble sucking milk. That’s because people with Prader-Willi syndrome also have muscle weakness.

With increasing age, new symptoms appear: short stature, lack of impulse control, tantrums, lack of puberty development – and also the irrepressible hunger. “He’s the biggest challenge,” says Eiholzer. Because lack of growth and lack of puberty development can be treated early today with hormones. Exercise also helps against muscle weakness. But the hunger remains.

To meet this need, some people with Prader-Willi can display a level of dexterity and creativity far beyond their cognitive abilities. This is because their IQ is only around 70, which is the same level as people with trisomy 21. But when they search for food, they can suddenly act like people with an IQ of 120. Searching for food becomes a survival instinct.

However, on the part of the authorities there is often little understanding of the living conditions and needs of those affected. Prader-Willi is one of the “rare diseases”. The family had to go to court several times so that the IV would cover the costs of Gioya Noser’s physical therapy or growth hormones. Urs Eiholzer guided them through the trials. With the revision of the law “Further development of the IV 2022” the situation was even worse. This was done only by “updating” the terminology in the legal text, forgetting that people with Prader-Willi would be excluded.

Due to the constant urge to eat, most of those affected die at a young age due to being overweight. So is Gioya Noser’s roommate. Until recently, the two lived together in a residential care center for people with Prader-Willi syndrome. There, Gioya receives eight carefully weighed meals a day. 1250 calories total. The 1.55 meter tall woman is no longer allowed to eat. This is the only way Gioya can maintain a healthy weight. “My body uses fewer calories than normal people,” she says.

Hunger is a daily struggle. Gioya puts it this way: “It feels like a little devil is sitting on one shoulder and whispering to me, ‘Come on, steal money from mommy’s purse and buy yourself something to eat.'” And on the other side a little devil sits Angel and says : ‘No you will not do that. Overeating is also bad for you!’» Meanwhile, the little angel often wins. But even the little devil can prevail. When that happens, Gioya feels incredibly bad. Moreover, the lost battle can hardly be hidden. People with Prader-Willi syndrome also gain weight faster than average.

At this point, Gioya’s mother, Janine Noser, intervenes: “From the beginning, everything was always a struggle with Gioya.” The hearing is not a reproach to the daughter, but rather a disillusionment. When Gioya was born, it was a struggle to get enough milk. Later on, it was a struggle to keep track of her weight, encourage her to exercise, find a daycare that wouldn’t feed her, and finally find a dorm where food was strictly locked.

Gioya notes that her mother is often referred to as a raven mother in private. “But my mom just makes sure I get the control I need.” She now always makes sure that all cupboards are locked and that all food is out of reach. “It helps me a lot,” says Gioya. Then she can focus on other things, ignore the desire. Also while cooking. Despite Prader-Willi syndrome, Gioya completed an apprenticeship as an assistant chef. Today she works 60 percent in a restaurant where people with disabilities work the stove.

But isn’t temptation greatest when cooking? “No, I always have something to do,” says Gioya. It’s only inconvenient when they’re bored, like when they’re waiting for guests in the kitchen. From time to time she becomes weak. But for the most part, she’s in control.

Despite all the daily struggles, mother and daughter stay together. Their relationship seems intimate but complex. They spend a lot of time together, playing games, doing sports, cooking. But the mother also reports many misfortunes she experienced with Gioya. She was lied to time and time again. Gioya kept finding new ways to trick her into getting food. “This loss of confidence is not nice,” says the mother. She knows that lying and cheating are linked to Prader-Willi syndrome. But she doesn’t want to take her daughter completely out of her responsibility either. Gioya looks down in embarrassment.

“Four years ago it was the worst,” says Janine Noser. Gioya nods and adds, “I kind of became a whore for cigarettes and food.” The mother shakes her head, her face twisted in pain. A much older man, also handicapped, abused her daughter for a long time. Later, a friend showered her with love, chocolate, and fast food, and she gained 20 pounds in just three months.

Gioya does not get direct access to money. She herself says that she would immediately spend it uncontrollably on food. Gioya keeps mentioning her mother’s structure and control or the Prader-Willi facility she lives in. This is what they need to live a healthy life.

But like any other 21-year-old woman, she dreams of living an independent life within her own four walls. When asked what she wishes for the future, Gioya’s eyes turn red and glassy. “I wish the scientists would finally find this drug that would help my hunger. They promised me that ten years ago.” Without this stupid hunger she wouldn’t need any control, she would be free like everyone else. She can handle everything else in her life on her own.

Urs Eiholzer says he never promised her a cure. Research has been fruitless for years. Drugs like Ozempic, which Hollywood stars are currently taking to suppress hunger, do not help people with Prader-Willi syndrome. And that’s how life currently works in the Prader-Willi-WG. (bzbasel.ch)