Hungry for Answers

Drawings of pumpkins in wobbly scribbles decorate the front door of the two-story brick house in Columbia. Chalk drawings of suns and hearts color the driveway. Enter, pass the couch in the living room that might have been white once but is now the color of oatmeal and follow the trail of little shoes strewn across the floor into the kitchen.

Long windows stream the day’s ending sunlight onto a kitchen table. This nook is a comfortable spot, warm from sunshine and slightly messy. Markers, glue and scraps of paper cover most of the table. The kitchen is equipped with an oversized refrigerator, rows of cabinets and a tiled floor. The counters are bare; the only appliance that occupies the space is an ice machine. The corners are free of lost Cheerios. Instead of cans of soup, boxes of Easy Mac or bags of chips, the cabinets are stocked with Pedialyte, cans of special formula, bottles of prescription medicine, and EpiPens — tubes of medicine injected into the thigh to stop wheezing, swelling, itching and stomach cramps during an allergic attack.

Two girls sit on wooden seats at a bar counter. Six-year-old Zoe hums something-or-other and messes with honey-blond bangs that flop over her orange glasses. Ten-year-old Kedzie silently observes the kitchen with tender eyes. Three-year-old twins Owen and Zackary, a wiggly, inseparable pair, sit at their own small table only a few feet away where the kitchen meets the living room. Their mother, Renee, hands each of them a bowl of what the family calls “pudding.” Owen bounces his legs on top of the table and Renee sets the bowl between them. Zoe adds one drop of orange and one drop of green food coloring to her formula and never looks away as she swirls the mixture into a soft green color. “Oh Zoe, isn’t that a pretty color?” Renee says, her voice comforting.

The children are rarely allowed to add food coloring to the formula. They only add dye when they really need something special; tonight Zoe does. For six months, she ate cocoa in her formula but she developed an aching sore throat. She pretended to feel fine so she didn’t have to give up the cocoa but admitted two days ago she felt sick. Now she stares at the formula.

“I’m. Going. To. Hate. This,” she says. She takes one bite and twists her mouth as if chewing old socks. The hypoallergenic amino acid-based formula is easily digestible and lowers the chance of an allergic reaction. When mixed with water, the formula is nutritionally complete, but all four children race to swallow each bite before the taste zaps the tongue. They finish eating in about four minutes. Kedzie is back to cutting out pumpkins from construction paper, Zoe dances on tiptoe back and forth between the kitchen and living room, and Owen and Zackary take turns smashing a toy truck into a pile of toys. Funny boys, Renee calls them.

The routine is the same every day. For breakfast, lunch and dinner, day after day, they eat the same “pudding.” Kedzie, Zoe, Owen and Zackary can’t eat food. All four children live with eosinophil gastrointestinal disorders, or EGID, also called eosinophilic disorders.

EGID is a strange and invisible chronic disease that makes food the body’s enemy. Eosinophils are a type of white blood cell. For an unknown reason, there can be high numbers in parts of the digestive tract. This causes an eosinophilic disorder in which the body reacts negatively to food and damages the tissue. For some people, eliminating certain foods is the cure; for these four siblings, eliminating all food has been the only successful treatment.

The children eat what is called an elemental diet that consists only of the formula, ice, pure cane sugar and water. They are part of an emerging medical puzzle. Experts are seeing more and more of the disorder, primarily among children.

“Of course we want to feed our kids, but I also know logically that giving them food would be like handing them Drano,” Renee says. Cans back in the cabinet, a dusting of formula wiped from the counter. Done. Meal over.

This isn’t quite the life Renee Martin-Kratzer and Brian Kratzer expected when they married 17 years ago and committed to raising a family together.

Renee was 22 when she landed in Seattle. The year was 1994. Brian waited at the airport for her: tall, 24 years old, handsome and giddy at the sight of her. She was a sunny blonde, an obsessive student and a sorority sister with a creamy complexion. At Kansas State University where they met, she had been a reporter and he was a photographer. He took her staff picture but hadn’t put the film in the camera.

“It would have been cooler if I meant to do that,” he says and laughs.

“I thought, really? This guy forgot to put the film in?” Renee says, smiling. She tucks a strand of her blond hair, now highlighted to cover the years, behind her ear.

“But it was a good thing I forgot,” he says, smiling back at her. He met her again for a reshoot — this time with film — and soon after they started dating. They continued to date when Brian graduated and moved to Idaho for his first job as a photography editor. One weekend, Renee convinced him to meet her in Seattle. At the end of the weekend, they took a ferry to Bainbridge Island.

They sat on a log on the beach and had the summer evening all to themselves. The marine air was comfortably cool. “Look what I found,” Brian said, holding out a shell. Renee laughed and disregarded the shell. “Look what’s in here,” he tried again. Renee suspected there was a bug inside the shell and didn’t look. “Will you just look?” he sulked, dropping his shoulders. Now he was on his knees and Renee saw the diamond ring inside the shell.

“Whoa,” she responded, frozen in shock at the ring.

Oh crap, this isn’t how it’s supposed to go, Brian thought. This wasn’t the timeline of Renee’s dreams. She was starting her senior year of college and they hadn’t planned their lives together.

“We haven’t talked about kids,” she told him. They sat on the log until the sun dropped. Brian agreed to three children, but then he would have agreed to anything to marry her.

Renee returned to Kansas engaged. While she and Brian were apart, they talked on the phone. One night, Brian called Renee after he had photographed a wedding for the newspaper. “Renee, there were nine kids. Nine! It was awesome!” he told her on the phone. They kept their elbows off the tables, didn’t cry or stick out their tongues and kept people entertained. That did it. There was no question that Brian and Renee would have children.

After Renee graduated in December, the couple reunited in Kansas, married in a small church, and danced together for the first time as a married couple in an old train station. Afterward, they moved around the country a few times, sometimes with no job prospects but always trusting life would fall into place.

“We’re eternal optimists,” Renee says.

Life did fall into place. They settled into Columbia, worked on their careers and pursued advanced degrees. Renee got pregnant.

Empathy, Brian decides. That’s what changed in him the day he became a father. The day Kedzie was born, Brian stopped viewing the accidents and tragedies he photographed as events and began looking at them as people. Brian’s empathy grew quickly when he saw Kedzie begin to suffer in strange ways.

When Kedzie was 1, the family visited Renee’s mother in Kansas. Kedzie grabbed a peanut from a dish of Chex Mix in the living room, sucked on the peanut and spit it out. That night, Renee unzipped Kedzie’s pajamas and discovered pink, swelling splotches that covered the baby’s entire body. After avoiding peanuts for one year, Renee and Brian decided to take Kedzie to an allergist who found she had an extreme peanut allergy. Renee vigorously joined every allergy group online. Renee and Brian carefully watched their daughter’s diet; although things seemed fine, she sat in her highchair and slowly chewed each bite of food over and over and over again, as if waiting for it to disappear before carefully swallowing. Renee watched mystified, wondering what was happening to her baby. She sat at the computer late at night reading everything about allergies and hunting for clues to what was wrong with Kedzie. She found nothing.

When Kedzie was 2, the Kratzers’ careers took them to Florida. Renee taught as an assistant professor at the University of Florida and Brian worked as an online and multimedia editor for the Gainesville Sun. Kedzie grew into a quiet, curly haired 3-year-old, and Brian and Renee decided to have another baby.

Like Kedzie, Zoe appeared healthy, but things changed quickly. There were the times that no matter how Renee changed her own diet, Zoe broke out in rashes during breastfeeding. There were the nights when Brian and Renee awakened to crying, but not the usual cry of a baby. Zoe was in pain. She arched her back and screamed in agony. Then there were the sleep-deprived mornings after a night spent with Zoe as she projectile vomited.

“It’s just allergies,” doctors snuffed in a tone that shouted, “Jeez, relax, you overdramatic mom.” But no matter what type of formula Renee and Brian fed Zoe, the baby’s body relentlessly rejected every food. When she turned 1, answers seemed bleak. “That was the worst time of our lives,” Renee says. “We didn’t know what was happening. I knew something was wrong but no one was helping me.”

Renee ferociously scoured the Internet for hours at night. One night, she desperately typed, “Top allergist in the country,” and then hit “enter.” The search returned “National Jewish Health” in Denver. She contacted an allergist and sent a list of all the foods that made Zoe sick, which was every food Zoe had ever eaten. The allergist mentioned eosinophilic disorder; Renee and Brian had never heard of it. Renee returned to the Internet and looked up the disorder. Slowly, then faster and faster, her spirits lifted while reading what perfectly described Zoe.

“We thought ‘Oh, my God, there’s a word for this?’ ” Brian says and laughs a bit. The allergist suggested the Kratzers bring Zoe to Children’s Hospital in Denver, which was just starting a clinic for the disorder. The hospital works as a team with National Jewish Health to treat eosinophilic disorders. But before traveling to Denver, Zoe needed an examination by endoscope, which is one way to diagnose the disorder. The endoscopy procedure places a tube into the mouth, esophagus and stomach, and takes images of the tissue. The toxins that eosinophils release can sculpt scars into tissue, which show up on endoscopy results.

So Renee took chubby, smiling Zoe to a hospital in Florida. The doctor whisked her away while Renee sat in the waiting room letting the fear of not knowing what was wrong with their child fester and ooze like a picked scab. She adjusted and readjusted in her chair until the doctor returned.

“Why did you put your child through that?” he asked. Heads turned in the packed room. The endoscopy showed no tissue damage. “Everything was officially OK; nothing was wrong with her. Why would you make her do that?” he said, as Renee felt scolding eyes looking on her from all angles. Utterly shocked, for half a second she wondered if she was a crazy mother. But she didn’t cry. She grew angry and didn’t care that the endoscopy looked normal.

“I knew she was sick,” Renee says. Unbeknown to the doctor in Florida, EGID is an invisible disease. There can be high numbers of eosinophils without visible tissue damage. The only way to truly diagnose the disorder is with a biopsy, examining a sample of cells. From her research, Renee knew this, so when the doctor’s office called with an official report of Zoe’s healthy results, she requested the medical records. At any moment she could have surrendered to being a crazy mother and canceled the appointment in Denver, but she quickly stuffed that thought away. At the bottom of the record she read, “Possible eosinophilic disease.”

“They never told me because they didn’t know anything about it,” she says plainly in a voice laced with anger. “And apparently they don’t know how to read a pathology report over the phone.”

The Kratzers traveled to Denver for an extended visit at Children’s Hospital with Dr. Glenn Furuta, one of the leading physicians and researchers on the disorder. Renee reeled off question after question about Zoe’s symptoms. Finally, someone knew what they were talking about. After testing several foods with Zoe and recording her reaction, Furuta diagnosed her with EGID.

“When tragedy strikes and that tornado happens, you’re excited,” Brian says. “But then there’s that ‘I shouldn’t be excited because it is a tragedy.’ ”

Zoe has eosinophilic esophagitis (EoE), a form of the disorder that affects the esophagus. When food enters the throat of a person with EoE, eosinophils irritate the healthy cells and cause inflammation that blocks the food. This form is better understood because it is more common, affecting 1 in 2,000, mostly males. The common symptoms, such as food sticking in the throat and trouble swallowing, are often mistaken for other conditions such as heartburn, which leads Furuta to believe many people have EGID without knowing.

Renee and Brian started feeding Zoe an amino acid-based formula, and like magic, she no longer got sick. They started to feel sane again but less than one year later, when Kedzie was 5 years old, she started choking on every food she ate. Renee and Brian quickly recognized the symptoms and for the first time, they knew eosinophilic disorder to be a real genetic disease. They traveled back to Denver and Kedzie was also diagnosed with EoE.

Just 15 years ago, an EGID diagnosis didn’t exist. When Furuta began his medical research on eosinophils in 1990 as a fellow at Harvard Medical School, there was no research on the cells. Since then, scientists and physicians have begun to understand the condition.

In 2011, a panel of 33 experts on EGID met and reviewed all of the medical literature, which has more than doubled since 2007. Furuta and the Kratzers’ current allergist, Dr. Fred Atkins from National Jewish Health, attended the discussion. Other experts came from the nation’s leading centers for eosinophilic disorders: Children’s Hospital of Philadelphia, the Cleveland Clinic and Cincinnati Children’s Hospital Medical Center. The center in Philadelphia has the largest EoE population and treats 1,500 children, infants and teenagers. Experts gather ongoing research from patients and families at these centers. Together, they discovered that EGID is genetic and identified five or so related genes but suspect other genes go under the radar. They think a protein called interleukin 5 (IL-5) might be in charge of eosinophils but even that is debated. Exactly why and how eosinophils form still boggles brains. What makes these cells think food is the enemy?

A strange complication to the disorder is also appearing. Children with EGID have been diagnosed with mitochondrial disease, which is failure of a special part of cells that is responsible for 90 percent of energy the body needs to live and grow. The Kratzer children started showing signs about 1½ years ago with muscle weakness and joint pain. Kedzie, Zoe, Owen and Zackary most likely have the disease, their parents believe. Like EGID, mitochondrial disease is not well-understood. Experts wonder if eosinophilic disorders cause mitochondrial disease. Or, is it the other way around? Does one worsen the other? The Kratzer family is currently awaiting genetic test results they hope will provide some insight to these questions.

Until there is more research, the lack of medical understanding of these two disorders means there are few treatment options. In EGID, what works for one person doesn’t always work for another. “The challenge is balancing what is worse: the treatment or the disease,” Furuta says. The Kratzers are his only patients who use formula for long-term treatment.

Aside from its terrible taste, the formula is expensive. The family goes through 2½ to three cans a day. Each can costs $35, which adds up to about $40,000 a year. Then there is the cost of doctor visits, additional medications and flights to Denver. The family receives insurance coverage through Brian’s employment as an assistant professor of journalism with the University of Missouri — the coverage pays $5,000 per child up to the age of 13, leaving $20,000 in out-of-pocket expenses. Health care providers argue they should not cover formula because they would never pay for a family’s food. This infuriates Brian and Renee. The formula is a medical cost for the family, they argue, adding that they would happily pay for food if they could; the cost of the formula, they point out, would buy extravagant amounts of food. But limited coverage persists, and Renee and Brian hope for better treatment options soon.

“We’re going to make it so you can eat some food someday,” Furuta told Kedzie at the end of their visit in Denver.

After receiving the second diagnosis in 2008, the family returned home and prepared for a new life without food. Brian called his mother with the news. She listened to Brian explain the disorder and how the girls can’t eat food.

“I don’t believe you,” his mother said. She wasn’t convinced the disorder is real and thought Renee and Brian were cruel parents not to feed Kedzie and Zoe.

“That was …” Brian begins, then stops. He sits in his office at the Columbia Missourian, where he is director of photography. A large window overlooks the street below. His desk is covered with stacks of paper; a few license plates from his collection hang on a bulletin board. His long legs cross over each other; his fingers toy with a pen. His gaze drops to the pen. “That was probably one of the toughest moments,” he says as he recalls the conversation of five years ago, his fingers fiddling a few more seconds. He pieces together a hurt smile and says, “So, anyway …”

Two years later, Brian and Renee faced another EGID diagnosis, this time for Zackary and Owen. Unlike Kedzie and Zoe, they caught the disorder early in the twins.

When Owen was 4 months old, he started getting rashes on his legs, bottom and stomach. Brian and Renee quickly decided they needed to be closer to doctors in Denver and family in Kansas. They left Florida and moved back to Columbia; right away, they were thankful to be closer to Furuta when Owen’s symptoms worsened and Zackary also began showing symptoms. Renee sent Furuta photos of rashes on the backs of the twins’ legs and videos of them arching their backs after eating. Furuta suggested feeding the formula and an endoscopy when the twins were 14 months old. But without exposure to food, a scope passes as perfectly normal, sometimes masking EGID. Although it seemed cruel, Renee and Brian were forced to give Owen and Zackary food. Furuta advised the boys be exposed to food for at least two months starting immediately.

Next week, Renee thought. The next week she agreed on the next and the same for the next week and again the next.

“Why would I want to make them sick?” she says with dread in her voice. But she could no longer postpone the endoscopy and fed Zackary and Owen their first tastes of food. The twins chewed and their eyes began to pop as they buoyantly bobbed in their high chairs. They stretched as far as their little arms reached and opened and closed their hands. They knew exactly how to put food in their mouths and smiled while chewing. But the longer Zackary and Owen ate, the more often Renee and Brian awakened to crying in the night. They couldn’t bear to make them sick. Exhausted, they quit after two weeks.

“I hope they don’t remember now because it was so sad. It was weird for them. They could eat and then they couldn’t eat,” Renee says. They hoped the two weeks was enough exposure for the endoscopy but had no guarantees.

“We don’t really know how to tell you this,” Furuta said, looking at the scope. Both twins were diagnosed with eosinophilic gastroenteritis (EGE), a rarer form of EGID that affects the stomach and small intestines — for Owen, it affects his entire digestive tract. About 2.8 in 10,000 people have eosinophilic gastroenteritis but doctors aren’t sure of that number. In fact, doctors know very little about this form of the disorder.

Surely with four diagnoses, Brian’s parents would believe their son now. Silence descends. Thirty seconds. “I don’t know,” Renee answers quietly, eyes sad. “I don’t know what they believe.”

Today, all four children still can’t eat food. “Eggs!” Zoe shouts. That’s the food she would eat first. Kedzie’s choice is bacon.

Zoe and Kedzie have tried several foods since they were diagnosed. In EoE, few or many foods may be the problem so food trials are meant to target those. They can only eat one food at a time, though, because there is a delayed response. A food may seem fine while the number of eosinophils secretly increases and causes tissue damage until eventually symptoms emerge.

“I think I am a very optimistic person but it’s such an exhausting process,” Renee says. Over and over Kedzie and Zoe try a food, love it and then get sick. “It’s so mean because they taste the good life and go back to nothing. How can you do that?” Renee says.

Kedzie and Zoe have very different personalities and different ways of dealing with the disorder. Zoe is a comedian and a social butterfly. Her favorite way to make people laugh is her “classic Zoe face,” which is made of one big eye, the other almost shut, mouth tightened in a circle and head tilted to the side. Kedzie would never be caught doing this. She says only a few words at a time and sometimes speaks so quietly only Renee hears her. Kedzie’s dark brown curls stick out in every direction. Zoe’s blonde hair lies flat against her head. At home, it is easier for them to cope, but their lives get complicated when at school and with friends.

Zoe never misses a social event, even though each one features food: pink cupcakes with heart-shaped sprinkles, cake with edible candy rings on top and chocolate brownies with gummy worms. For Zoe, food has never looked better than now. Every day at school, she picks up her formula from the nurse and joins friends in the lunchroom. Zoe eats her pudding and chats the whole time, but she doesn’t say how she wants to rip the food out their hands. She fakes a toothy childish smile as she grows jealous of the colors and smells at the lunch table.

It is not until Zoe goes home that she is open about her frustrations. She tells Brian, “You know, I know we’re not supposed to hate God, and I don’t hate God, but you know I’m really mad at him for giving us EoE.” She starts to cry.

Little Zoe is quite eloquent, Brian says. He tells her she is right: this sucks. He has never denied that their situation is unfair but knows feeling sad will not change the world for them.

“Renee and I agree that if you sit and dwell on things too much you can feel pretty crappy,” he says. He tells a sobbing Zoe that she is a champion for putting up with EGID.

Zoe is open about her feelings, but Kedzie internalizes hers. And unlike Zoe, Kedzie has accepted that food hurts her; she would rather feel better than eat. Kedzie has tried every treatment for EoE and still the elemental diet has been the only success.

Diagnosed as a 5-year-old, Kedzie struggled to give up eating. She started with the elimination diet, which removes the six common allergenic foods: milk, eggs, wheat, soy, nuts and fish. The strict diet is supposed to lower the chance of allergic reaction but Kedzie continued to get sick no matter what she ate. For a while, she tried topical steroids, which is one of the earliest treatments for EoE. The steroids, meant to be inhaled to reduce asthma inflammation, are puffed into the mouth for EoE and swallowed to coat the throat. Most children are able to eat food with the steroids; Kedzie has tried three steroid medications and found success with one. But while on the medication, she had aggressive, tear-soaked tantrums and wrote a mean letter to a close friend. Renee and Brian wondered what happened to their sweet girl and then discovered change of mood is a side effect of the medication.

Brian and Renee have switched formulas multiple times, hoping one might make food trials more successful. Renee thinks about the failed treatments. “We’ll just try again,” she says. “That’s the name of the game.” When she tries to laugh, she sounds sad.

At times, Brian and Renee have kept Kedzie out of school to avoid events with food. When Kedzie’s school hosted an Easter egg hunt, which means candy and treats all day long, Renee took the day off and took Kedzie to a movie. They had the best time — or so Renee thought. When they returned home, Kedzie ran to her room and burrowed underneath the covers on her bed. Renee followed and asked, “What’s wrong? Wasn’t this a great day?”

Kedzie cried. “I miss my friends at school at the party,” she said in-between hiccups and sniffling. Renee wondered why she had never realized this. We never go for the food; we go for the friends, she thought. Renee admired her daughter’s early decision to not avoid food, and decided herself the children would no longer be the social outcast at every event with food.

For her children’s birthdays, Renee crafts unconventional cakes. She used to make them out of ice or flowers but now laughs at those cakes. “We have upgraded,” she says. Before Zoe’s sixth birthday, she sent out flyers apologizing to parents and telling them to not expect cake because it is inedible for Zoe. She made a cake with an 8-by-10-foot picture of Zoe in her favorite leopard and cheetah print outfit. The cake read “Happy Birthday Zoe” and had a safari diorama scene decorated with glitter, sand, candles and safari toy animals that Zoe later played with. “I probably had a little too much fun making it,” Renee says with a childlike laugh.

At classmates’ birthday parties, some parents have served sugar cubes for Zoe and Kedzie. On Halloween, neighborhood parents have given little toys instead of candy. “People don’t have to do that and when they do it’s really touching,” Renee says.

Renee and Brian haven’t faced as many situations involving food with the twins. Zackary and Owen are in preschool and much less bothered by food than Kedzie and Zoe. Their identical caramel-blond hair hints is just long enough that the ends are beginning to curl. They wear matching Crocs and are never more than two feet apart. “What’s really neat is they have each other,” their teacher, KP Kritchfield, says.

At school, the twins go straight to their own table for snack time while the other children sit at a larger table. Every day, Renee packs a bag with two amino acid-based formula juice boxes for snack time and formula powder for lunchtime, which either Kritchfield or the classroom’s other teacher, Lisa Jennings, mixes with water. Because of the cost, the teachers label and save any leftover “juice” for Renee. Owen and Zackary drink from their juice boxes and stare at their classmates eating crackers. When the twins finish, they stand near the table and curiously peek at the crackers over the shoulder of a girl in a tutu. Once, a cracker fell on the floor and Zackary never stopped pointing at it but wouldn’t go near it. They notice that other kids eat but the boys don’t cry or fight about it.

Kritchfield calls the smiling twins “little light bulbs.”

“You would never have any idea they’re sick,” Jennings says.

That’s the most important thing to Brian and Renee. While not eating food is awful, they believe their children can be just as happy as other children.

Each night, Brian and Renee wait for their children to fall asleep before they make dinner. They don’t hide food from their children but they also don’t show it off; neither parent suffers from EGID. Renee usually microwaves two vegetarian burgers; anything more is extravagant. Cooking has never been Renee’s calling and fortunately, the couple has never been drawn to fancy restaurants. Food has become more of an afterthought in their life ― something only for survival.

Most dinners are quick. Brian and Renee usually eat at the table but some nights stand at the kitchen counter or sit in front of the television for a couple of minutes. If they have time, they catch up on their days and Renee shares something new she read about EGID. Instead of researching for journalism, she now puts all of her energy into researching the disease. At 40, she is an adjunct professor, teaching magazine design at the University of Missouri; she also teaches online courses in magazine journalism for the University of Florida and West Virginia University. She teaches during the day, picks up the children, and then grades her online class after the children go to bed.

“I haven’t ever figured out how that woman handles it,” says 43-year-old Brian with a laugh.

The two rarely feel bad about their lives. “It’s funny to think back to that first diagnosis because now it’s like, well, this is our family,” Brian says.

The two of them have the attitude that a cure will happen when it happens. They don’t expect that day to be today or this week or next week. They are prepared to see Kedzie in high school and still facing EGID. They will be disappointed, but prepared. “There’s gotta be something out there that these kids can eat, something like soufflés and sugar and whatever,” Brian says. “Once we identify that thing, man, we’re going to go crazy. But I don’t think I’m going to wake up tomorrow and — poof — it’s fixed. I’m not waiting for a miracle. I’m going to be an old man and see how my kids make it through this.”

– Madeline Schroeder (source: insidecolumbia.com)