A deadly hunger: Rare condition causes insatiable desire for food
In the Grundy family's Groton home, the kitchen remains locked at all times. A four-digit code unlocks the specially installed door, and at night, a second code disarms the loud alarm that blares if anyone tries to get into the kitchen without it.
Everyone in the family knows the two codes, except Cami.
The system was installed to keep the 21-year-old, who has the rare genetic condition Prader-Willi syndrome, out of the room. Her condition causes insatiable hunger, and if she were to get in unsupervised, she may very well eat herself to death.
Green, yellow, red
On a January evening, Larry Grundy typed in the code that unlocks the kitchen door to start preparing a meal out of sight of his daughter, Cami, who was curled up on the living room couch waiting for dinner time.
Meals in the Grundy household are a strict, structured affair. They have to be, to keep Cami safe and healthy on a diet especially designed for her PWS symptoms: insatiable hunger, compulsive thoughts, slow digestion, low muscle tone, low energy levels and limited cognitive function.
She eats three meals per day, at the same time every day. Her foods are color coordinated; fruits and vegetables are green, starches are yellow, and sweets and snacks are red. She rarely eats anything red.
She is limited to 1,000 calories per day on a diet designed by a specialist at the Children's Institute in Pittsburgh, where Cami went for a 60-day program in 2012. Though that number may seem low, it's the healthiest amount for her: PWS causes short stature — she is fully grown at just 4 foot 6, despite a decade of growth hormones — and low muscle tone and easy fatigue, making exercise difficult. When she first visited the institute, Cami was overweight and her parents were struggling to keep up with the increasing symptoms that emerged as she aged.
On the chilly evening in January, Cami abided by her usual diet, eating chicken, peas, watermelon and two cups of red Jell-O. She drank coffee, made in the Keurig she got for Christmas, with sugar-free creamer from a pink and purple mug. She rarely begs for sweets or snacks but loves her coffee, her parents said.
During every meal, Cami sits on a piano bench in the living room near the kitchen doorway, eating off of a TV dinner tray. Despite being ravenously hungry all day and thinking of this moment constantly since her lunch, she eats calmly and quietly, complimenting her dad on the chicken and chatting about which Jell-O flavor she likes best.
Cami eats there so that she is outside the kitchen and dining area, where she might try to grab additional food, or fixate on the idea. And her mom can safely lock the kitchen door and get a few things done around the house while still keeping an eye on Cami.
Though PWS causes cognitive disabilities, Cami understands why her parents are strict with her. "I would eat all the food in the kitchen, so that's why we have the kitchen door," said Cami, who remembers that before the code lock, she would always find the key to their old door. "It's for my safety."
As each meal is prepared, her parents measure out each ingredient — in measurements they have memorized by now — and serve Cami her dinner at the same time with the same utensils. To keep her from fixating on sweets, her parents only give her dessert on special occasions — for her birthday, a small piece of cake; for Thanksgiving, a small slice of pie. Any other time she asks, she's reminded that "it's not a red food day."
Her parents and siblings, older brother Cody and younger brother Cavan, limit their snacking and sweets in front of her. Often, if Cami sees someone eating something that isn't part of her diet, or outside one of her structured mealtimes, she will fixate on it for hours. Perseverating, or repeating things, her family said, is a common symptom of PWS and one that majorly affects Cami.
To keep her safe and ease her anxiety, they have to be strict with Cami and with themselves.
"Someone with Prader-Willi syndrome is requiring their network, their support, to provide 100% food security," her dad said.
"A person with this syndrome cannot control their hunger feelings and if left to their own desires will basically eat themselves to death," he said. "It's impossible for a person with Prader-Willi syndrome to not seek after food and not eat it when it's there."
'The Stealth Ninja'
PWS is caused by a mutation of chromosome 15 before birth. In Cami's case, both of her chromosome 15s are from her mother, rather than one from her mother and one from her father.
The syndrome affects about 1 in 15,000 and causes developmental delays, compulsive behaviors, low muscle tone, slow digestion and short stature in addition to the insatiable hunger.
When Larry Grundy asks his daughter "What do you think about all the time?"
She instantly replies: "food."
Her desire often leads her to get her hands on food outside her diet. And when she does, her parents blame themselves. "When she gets that added calories and that food that's outside her regular diet, it's a great feeling of remorse and guilt that comes over us because it's not her fault, we're the ones that failed her," said Larry, breaking down in tears.
In the Grundy home, Cami is known as "the stealth ninja" — if she has just a few seconds where no one's eyes are on her, she'll steal as much food as she can grab. Usually, her tactic is to hoard the food in her pockets or under her fleece purple bathrobe until bedtime, when she'll binge everything she managed to get away with and hide the wrappers.
"If I'm in the kitchen, I am very quiet and fast," Cami said. "I would take something out of the kitchen and I would bring it into my room, anything like that Mom and Dad have to watch out for."
Her mother said that although Cami's IQ is low, she is extremely observant in the kitchen and is incredibly creative in her schemes to steal food. Her mother said it's likely because she is thinking about food all the time. "She watches everything," Kim said. "If you take her into the kitchen, she's not going to look at you, she's going to look everywhere else, so that if she gets the chance, she is going to grab something and run."
Her older brother, Cody, said that Cami's mind is fascinating when she's focused on food. "It's interesting because she doesn't know 2 plus 2," said Cody, "but she'll think of the most intricate way to get from here to there without you thinking she ever had time to get food."
When her parents find evidence that she's been binging, such as an empty box of her younger brother Cavan's Cliff bars and several wrappers behind her bed, they are consumed with guilt.
"It's amazing how emotional you get when you see the food wrappers behind the bed, especially when it's a lot and you're not expecting it," said her mother. "I feel frustration, fatigue and I feel guilty that maybe one of us did something."
Breaking her diet also has a physical reaction for Cami, often causing her stomach aches and discomfort while she's in school that may require adjustments in her medication or require a trip to the emergency room, her mom said.
Navigating the hunger and the risks taken to get food can be difficult, and risky, for both the person with PWS and their caregivers.
In addition to trying to break into the kitchen, Cami has often snuck out of the house in the middle of the night to find food. Her usual plan, said her parents, is to sneak out the front door to re-enter the home through the back door to get to the kitchen.
Now, the alarm system is also triggered by access to the front or back doors without the code, and Cami's parents are often woken up in the middle of the night to find their daughter out of bed and out of the house. In fact, they said they haven't slept through the night since she was born.
Cami said that even though she knows doing those things is wrong, she can't help it. In those moments, she is always thinking about food.
Stacy Ward, director of family and medical support and special projects for the Prader-Willi Syndrome Association in Florida, said this is common behavior for a person with PWS. "Even though individuals know that their behavior is harmful, in the moment they can't process that thought," she said, "because the drive to eat is truly a physical, psychological drive to eat."
Ward said one patient described the sensation as an itch inside their body that was only satisfied when they ate. Another broke down in tears when she described her thought process. "She knew she shouldn't do certain things but she knew she couldn't not do them either," the woman told her.
"It's just really heartbreaking," Ward said.
Larry Grundy said there is a saying in the PWS community: if everyone is watching, no one is watching.
The family, he said, can't simply agree to keep an eye on Cami as a whole, someone always has to be dedicated to watching her at every moment. "We have to be on our game at all times and can't really let our guard down," he said.
Her parents said that there are usually a few hours in the evening, following Cami's 7 p.m. bedtime, that she stays asleep and they are able to let their guard down. "There are nights when it's a screaming, yelling, door-slamming night," said Kim, "but most nights, that's the time that we can finally let go."
The family will relax on their couch and pull out some ice cream or a bag of chips, but even then, they have to be careful. If they get up for a moment or leave the room, they have to make sure the snack gets put away.
"I'm very fast," Cami said.
An unexpected diagnosis
Cami was the couple's second child and the pregnancy was normal, Kim said. The birth was, too.
The first sign that something was wrong, ironically, was that she wasn't eating. A few hours after Cami was born, nurses took her away for observation after noticing she wouldn't latch on to her mother's breast or a bottle. Nurses told the parents that the baby was "floppy." It's a word commonly used to describe babies with PWS, according to Ward, because of their low muscle tone, which also makes it difficult for them to latch on to a nipple or bottle.
Children with PWS usually start occupational therapy at about 18 months old and their developmental milestones, like walking and talking, are delayed, Ward said. There is some sort of development disability in every person with the syndrome, but the spectrum is huge: some people are nonverbal, while others go on to graduate high school.
However, most have trouble holding a job, because sudden changes in job expectations can be difficult for them to process, they tire easily and they can't be in areas with food without being supervised.
According to her parents, Cami's cognitive function is on the lower end of the spectrum. She can read and solve puzzles, which people with PWS are often good at, but she has difficulty with comprehension and cannot do simple math. She has limited skills with money and has no concept of time.
She needs constant routine and has tendencies to hoard things or develop obsessions. She struggles with compulsive behaviors, such as picking up hundreds of pine needles under the Christmas tree. She sometimes tries to hurt herself or others, tackling her mom and throwing things at walls.
According to Ward, people with PWS can most seriously harm themselves through their food seeking. Since they require fewer calories than a person without the syndrome, Ward said a food binge can harm them more quickly and more seriously, and "the consequence could be, and has been, death."
Ward said that people with PWS have a high chance of stomach perforation after a food binge, but they might not even know when it happens. People with the syndrome, she said, have an extremely high pain tolerance, so by the time they show symptoms or complain of pain, they will likely already be "in a true medical crisis" that could lead to death.
Outside of those risks, people with PWS usually live into their 60s. The oldest person known to have the syndrome just turned 70, Ward said.
A new normal
The Grundys have had to adjust their lives to meet Cami's needs. Leaving the house for errands like grocery shopping or dining at a restaurant requires constant planning and preparing.
Kim and Larry said they try to avoid bringing Cami into stores with lots of food. And they rarely have a chance to socialize. "We don't go out with a lot of friends, we don't have a lot of friends, just because our lives are so complicated," Kim said.
Despite all that, the family has found ways to get out of the house in comfortable, safe ways. They are very involved in their church, Groton Bible Chapel, and Larry is very active with the music boosters at Robert E. Fitch Senior High School, where he was once in the marching band.
One local restaurant, Pizza Palace, even worked with Larry to create "The Cami Pizza" that she can order when they go there for dinner. The pizza has half the dough of a normal pizza, very little sauce, very little cheese and lots of vegetables, Larry said.
When they're going to a place they've never been before, they pack her a whole meal in case there's nothing that she can have.
Cami's parents both work full-time jobs, Larry running a three-generation family landscaping business, and Kim working as an operations assistant at LEARN Regional Education Service Center. Larry's parents help care for Cami a great deal, but after working all day, the parents come home to the exhaustive role of keeping a constant eye on their daughter, while raising their teenage son.
Keeping everything locked and secure hasn't been easy on Cami's brothers, either, but they've learned how to make sure their sister is safe. The burden on them also causes their parents guilt. "Most of their friends don't have the responsibility of locking their bedroom door every time they go in and out of it," Kim said.
'An absolute joy'
When they first heard Cami's diagnosis, Kim said she struggled to accept that her child may not have all the opportunities in life that she thought her girl would have.
"When you have a child that is born and you find out that they are not the child you thought you were going to have, you have to mourn that child and learn to accept what you have," she said. "I have a beautiful girl who has challenged me in great ways, ways that I never could have done without her; she's opened me up to a whole new world of understanding and understanding people and emotions."
Despite her challenges, Cami is a funny, talkative young woman who attends school, participates in work program, and loves spending time with her family. She has won 17 medals on the track and field team through the Groton Special Olympics and regularly attends Camp Horizons in South Windham, where she goes horseback riding and boating and dances with her friends.
Cami goes to camp 16 weekends through the year and for four weeks during the summer. Every time she goes, she has to bring boxes of dog treats for any dogs that might be there. Her love of animals, her parents said, is one of Cami's most endearing traits. She is incredibly empathetic, especially toward animals, the elderly, pregnant women and kids.
Many people with the syndrome love animals, babies and pregnant women and anyone else they may view as vulnerable, Ward said. "They love to take care of people and want to feel needed," she said.
Kim and Larry laughed as they explained that Cami's caregiving tendencies "are sweet, but expensive." The Grundys find themselves buying gifts for every baby expected in town, and treats for every dog Cami meets.
During the week, Cami attends the Transition Academy at Groton Public Schools, where she goes to different job placements throughout the week. On one day, she works with the elderly at Fairview in Groton, helping to fold clothing protectors and towels, a job she particularly enjoys. On another day, she helps color tag clothes and organize shoes at the local Goodwill.
Her favorite day, though, is Friday: "I get to go bowling and it's so much fun," Cami said. She and her friends help clean ball holders, lockers and menus at the bowling alley, and get to end their work week with a game of bowling, she said.
Her job opportunities are limited, but with the help of paraprofessionals and behavior analysts, Cami is able to gain work experience and learn new skills.
Christine Rebelo, behavior analyst for the nonprofit Institute of Professional Practice Inc., which assists Groton Public Schools with the Transition Academy, said that in the program, they focus on "creating specific opportunities for Cami to be independent while maintaining some level of assistance and monitoring."
Recently, said Rebelo, they've been working on helping Cami develop skills, such as using a microwave, composing an email and doing laundry, that will help her live more independently. It isn't without its challenges, though.
They also try to limit situations where Cami can feel distracted by food. They avoid going to the high school on days when other students are learning to cook. And once a month when students go to a restaurant, they make sure Cami has her usual meal packed.
The Grundys say that the program, which Cami is aging out of this year since she just turned 21, has helped them keep a routine for their daughter. They are currently in the process of finding a new program for her to enroll in.
Despite the challenges the family has faced, Larry Grundy described his daughter as "an absolute joy."
"She's engaging, she's playful, she's fun, she has a great sense of humor," said Larry. "She is very thoughtful, caring, and is really great with people that are older and have younger children. "
Larry said he is thankful for the special moments they have had where their daughter has been able to feel "normal." One of his greatest joys as a father was seeing her go to the prom; Cami got her hair and nails done, bought a new black and white dress and hit the dance floor with her friends.
"Sometimes you even forget that she has a syndrome when she's in those moments," he said.
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