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Family faces future where son will always feel hungry

Exchange Rare Syndrome
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In this Sept. 5, 2017 photo, Blake and Natalie Brenneman play with their 20-month-old son Evan, who has a rare genetic disorder called Prader-Willi syndrome at their home in Plainfield, Ind. In newborns Prader-Willi syndrome symptoms include weak muscles, poor feeding, and slow development. (Matt Kryger/The Indianapolis Star via AP)

Exchange Rare Syndrome
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By MAUREEN C. GILMER
Associated Press

Sunday, September 24, 2017

INDIANAPOLIS — Evan Brenneman doesn’t know it yet, but there will come a day when he will be hungry, and no matter how much he eats, he will never feel full.

Natalie Brenneman dreads that day and the days that follow.

Evan, who turns 2 in December, was born with Prader-Willi syndrome, a rare, nonhereditary genetic disorder that has a most cruel and unusual symptom — chronic hunger.

PWS tricks the brain into thinking the body is starving all the time. The chronic hunger generally begins around age 2, though for some children, it kicks in later. The technical term for the symptom is hyperphagia.

“Think about a time when you thought you were starving,” Evan’s mom said. “Someone with Prader-Willi has that sensation all the time. I think about driving home from work and I feel hungry, and then I think, ‘Will my son always feel that way?’ “

The thought of it leaves Brenneman and her husband, Blake, anxious, even though Evan is doing pretty well at the moment.

“We definitely feel like the clock is ticking.”

Healthy pregnancy

Evan Brenneman should have been a perfectly healthy baby. His mother’s pregnancy was closely monitored after she suffered two miscarriages. Medical staff repeatedly assured her that everything looked fine with the baby.

Prenatal screenings generally don’t include PWS because it is rare, afflicting about one in 15,000 to 20,000 people.

Evan was born full-term, but had to be resuscitated at birth. He couldn’t breathe or eat on his own and was transferred from IU West Hospital to Riley Hospital for Children at IU Health.

It was a shock to the Brennemans — first the traumatic delivery, then the possibility that their son had a serious disorder that was a complete mystery to them.

Once the diagnosis was on doctors’ radar, the Plainfield couple made the mistake of searching Prader-Willi syndrome online. They learned that their son could be nonverbal and might not walk. He could die as a young adult of morbid obesity because of the chronic hunger that will eventually afflict him.

Natalie Brenneman called it “our darkest day.”

“It just breaks you down,” her husband said. “You have a brand new baby, and it feels like their future is crushed right there when you read about it.”

“It was really pretty scary,” said Natalie Brenneman, a kindergarten teacher. “We felt like we were in a bit of a black hole.”

They climbed out of that hole by slowly opening up and talking about Evan’s condition. At first, they weren’t sure they wanted to, but once they did and people supported them, “that felt good.”

The couple connected with other families in the area and nationwide who are affected by PWS. They saw that a lot of those families were managing the symptoms of the disorder. They started fundraising for Prader-Willi research and were heartened to see so many people who wanted to support them.

Two weeks ago, they attended the national conference of the Foundation for Prader-Willi Research, which was held in Indianapolis. This past weekend, they were to co-host the Foundation for Prader-Willi Research Walk in Avon.

Online searches still paint a scary picture, both said. PWS is a spectrum disorder, so not everyone is affected the same way. Some people can live fairly independent lives, even go to college, with proper supports in place. Those are the lucky ones.

Susan Hedstrom is executive director of the foundation, based in Walnut, Calif. She is also the mother of an 8-year-old boy with the disorder and can relate to the Brennemans’ anxiety.

“You should never Google a rare disease,” she said. She knows because she did the same thing. “We were scared out of our minds. What you find is a very cold scientific definition of what this diagnosis means, and then you find photos of people that don’t look like what you had dreamed your child was going to look like.”

‘A wild man’

To see him today, Evan Brenneman is a typical toddler.

“I think that’s what surprises me most about him,” his mom says. “He likes Elmo, he likes anything with wheels, he loves our dog. He’s walking, he’s talking, he’s doing all those things they said he might not do. He’s a wild man.”

Indeed, this past week Evan played peek-a-boo under the sofa pillows, danced with his musical Big Bird and high-fived a visitor. He chattered about his dog and his toys, supplementing his toddler vocabulary with sign language.

It helps that he has had speech and physical therapy practically since birth.

The blond-haired boy is good-natured and friendly. He’s of average height and weight, thanks to a nightly growth hormone injection his parents administer in his thigh.

That growth hormone is the only approved treatment for PWS, Hedstrom said. “It addresses the growth challenges and some intellectual issues, but it does not touch on many of the challenges that our loved ones face.”

Like chronic hunger.

The Brennemans strictly control Evan’s diet. That means no sugar other than a small amount of fresh fruit, few carbs and more high-quality fats.

Giving him sugar is “like giving him cocaine,” Natalie Brenneman said.

A typical lunch is grilled salmon, bell peppers and sliced mushrooms. His go-to snack is avocado slices. What he can’t have is the kinds of things he sees his peers eating at day care, including crackers and cookies.

While his home environment is controlled and his parents eat healthier too, he’s starting to notice that his lunch is different than other children’s at day care. Staff are trained to see that he doesn’t nibble off a neighbor’s plate, but that will become more difficult to manage as he gets older.

Evan’s mom says her son is interested in food and always finishes what’s on his plate, but that is not necessarily a sign of PWS. She recently went back to work as a teacher and wonders if she’ll be able to continue working full time as Evan grows.

She knows that some families cannot do holidays, birthday parties or cookouts because food-focused events are too dangerous for their children. “We hope that’s not the case for Evan, but it could be.”

Hedstrom’s 8-year-old son attends school with an aide by his side at all times to see that he doesn’t have “unauthorized access to food.”

“We know if our children are not closely monitored, they may steal food, they may choke,” she said.

At home, the Hedstroms have taken measures that might seem extreme, but are lifesaving for children with PWS. Those environmental controls help keep them safe.

“We lock our refrigerator, we lock our pantry,” she said. “We cannot leave him unattended because he will eat toothpaste.”

Evan’s future

The Brennemans know the same precautions may be in their future.

While they’re hopeful there will be a treatment for hyperphagia in their son’s lifetime, they are realistic.

“That’s hard for me to think about, you know, sending my child to bed hungry or sending him to school hoping that he can concentrate and learn when he feels hungry,” Natalie Brenneman said. “It’s frightening. As a mother, your instinct is — protect my baby, feed my baby. That’s disrupted with PWS.”

Research proceeds painfully slowly for this family and all of the others dealing with the disease. But they continue to raise money, keeping their hopes alive that new treatments will be developed.

The Prader-Willi mantra is live life full, Brenneman said. “We want Evan to live life full — to feel full in his belly and to feel fulfilled. We want him to live a good, happy, long life.”

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