Life Without Food
Eosinophilic disorders make some kids (and adults) allergic to eating
As an infant, Aaron Weinbach would frequently cough, vomit and have difficulty feeding. Although doctors dismissed it, his mother Bonnie knew something wasn’t quite right but she had no idea what the cause of the problem was.
She nursed Aaron for 10 months then switched him to soy formula since he was allergic to milk. “He never drank all the formula at once. He coughed a lot. I never understood the moms that could fill up an 8-ounce bottle and their baby would down it in two minutes. I could only put in a few ounces at a time and even then Aaron wouldn’t finish it,” says the Westchester, New York, mother of two.
At 12 months, Aaron was switched to soy milk and he began coughing a lot more. After a couple of weeks, he started vomiting frequently, sometimes once a day, sometimes three times a day. It wasn’t a typical food-allergic reaction because it didn’t occur right after eating. Weinbach called the doctor’s office almost daily and visited weekly for weight checks. Aaron started to look frail and weak and the doctor slapped on a distressing label: failure to thrive.
“While Aaron’s doctor was away, I saw another pediatrician in the practice,” Weinbach says. “I caught her up on what was going on and she said, ‘Do you know about eosinophilic esophagitis?’ She described it as a type of allergic reaction that can cause vomiting and said doctors were starting to diagnose it more often.”
In fact, eosinophilic (ee-oh-sin-oh-fill-ick) esophagitis (EE or EoE) is a disease that has only gained recognition in the past decade. An allergic inflammatory disorder affecting children and adults, EE is an abnormal immunological reaction to food, characterized by elevated eosinophils—a type of white blood cell—in the esophagus. When the body’s immune system attacks a substance, in this case an allergy-triggering food, eosinophils move into the area and release chemicals that can damage the tissue. When the body produces too many eosinophils, it can cause chronic inflammation and scarring of the esophagus.
For younger children and toddlers, symptoms are vomiting, refusal to eat, abdominal pain and poor weight gain. Older children may have difficulty swallowing (dysphagia) and food may get stuck in their throat (food impaction) because of inflammation within the esophagus. Adults may have symptoms that mimic reflux disease, such as heartburn, difficulty swallowing and food impaction.
Although EE symptoms may mimic reflux, they don’t respond to reflux medication. Symptoms usually subside when trigger foods—most often milk, eggs, wheat, soy, corn, chicken and beef (the list varies for each individual)—are removed from the diet or when the patient is on an elemental diet of solely hypoallergenic formula. In some circumstances, medications (often steroids) are used.
Four weeks after Aaron quit consuming his trigger food, soy, the vomiting stopped and he started to gain weight. Aaron also avoids milk, eggs, peanuts, tree nuts, sesame seeds, avocado, peas and mustard, since he has more “traditional” immediate IgE-mediated food allergies to these foods.
All was fine until last fall when Aaron, then 5, got a piece of steak stuck in his esophagus for 12 hours. He could breathe but he couldn’t swallow. The family rushed him to the hospital.
“The GI doctor said they were going to get the food out in the morning through endoscopy, either by pushing it down or pulling it up,” Weinbach says. “Aaron couldn’t swallow anything and spent all night spitting and gagging. By some miracle, it went down in the morning before the procedure.”
Now Aaron’s family is trying to figure out what food or seasonal allergen exacerbated his EE. They suspect it was the black beans he’d been eating in the weeks leading up to the incident, since they’re legumes, just like the soybeans and peanuts that Aaron is allergic to.
EE has a strong genetic component in some families. “My husband gets food stuck in his esophagus once or twice a year,” Weinbach says. “When he was 6 or 7, he had a piece of London broil stuck in his throat and he had to get it surgically removed.”
EE is one of several eosinophil disorders under the broad category of eosinophilic gastrointestinal disease (EGID), explains Glenn T. Furuta, MD, director of the Gastrointestinal Eosinophil Diseases Program at The Children’s Hospital Denver and National Jewish Health.
EGID conditions are named depending on the location of the eosinophilic inflammation in the digestive tract. Eosinophilic esophagitis affects the esophagus. Eosinophilic gastritis, the stomach. Eosinophilic gastroenteritis, the intestine. And eosinophilic colitis, the colon.
EE is the most common. The other types of eosinophil disease are considerably more rare, says Furuta, adding, “A significant percentage of patients who have eosinophilic gastrointestinal disease have some other form of allergy.”
Wendy Book, president of American Partnership for Eosinophilic Disorders, a nonprofit patient advocacy organization, knows what it’s like to adjust to life without many foods. Her son Ryan, now 10, was often sick in infancy and was hospitalized with failure to thrive when he was 12 months old. The diagnosis of eosinophilic gastroenteritis came when he was 2½.
“It’s very frightening when your child is first diagnosed with a rare disease and you feel all alone,” says Book, a physician and mother of two who lives in Atlanta. “It can be terrifying to come home from the doctor’s office and have to change the way you eat and cook overnight. I went from a mom who didn’t cook at all to a mom who could make anything from just one or two basic ingredients. A little water, oil and corn flour and you can make a pancake.
“In general, making everything from scratch is the safest way to go,” advises Book, who utilizes the APFED cookbook, Extreme Cooking for Exceptional Diets. She lives by the motto, “If you fail to plan, you plan to fail,” mapping out menus a week in advance and bringing Ryan’s food with them wherever they go.
She also advocates “teaching your child that the disease does not define them. It’s just something they live with.” Book has re-oriented their life to include non-food-focused activities like family game night and weekend hikes. During holidays, they’ve created new traditions that revolve around games and crafts, rather than food.
It isn’t always easy. Ryan lives in Atlanta but travels to Denver for medical care. He had 15 endoscopies during the first nine years of his life.
When he was first diagnosed, he was put on a very restricted diet but even then he still had troublesome symptoms. At age 4, he was put on an elemental diet of solely hypoallergenic formula. Because of the large volume of formula needed and its unpalatable taste, patients on an elemental diet usually have a surgically placed feeding tube.
“It’s very, very effective—but it’s a challenge,” Book says. “He couldn’t eat anything by mouth. The formula was his sole source of nutrition.”
Ryan showed great improvement under the new dietary regimen. About nine months later, they slowly re-introduced one new food at a time.
Ryan still avoids dairy, eggs, fish, nuts, soy, rice, wheat, apples, sweet potatoes, many meats and more. Because his diet is so restrictive, he can’t get all his nutritional needs met, so he receives additional nutrition through the feeding tube. He pours elemental formula into the tube three times a day, even during school. He also takes steroids to control inflammation.
Each year, Ryan and his family put together a presentation for his classmates to explain and demystify the feeding tube.
“We do a presentation for the kids at the beginning of the year,” Book says. “We give them an opportunity to ask questions. It opens up a lot of discussion and takes the mystery out of it all and then they’re not interested any more. Otherwise, kids would be gossiping and saying, ‘What is he doing over there?’”
EE has been diagnosed in every continent except Africa. Reports of its prevalence range from 1 in 1,000 people to 1 in 10,000. The onset of EE symptoms can occur at any age and at any time; however, it’s more often diagnosed in childhood and more predominant in males than females. People with EE commonly have a personal or family history of food allergies, hay fever, seasonal allergies or asthma. In certain families, there may be a genetic tendency for the disease.
“It’s one of the hot topics in the allergy and GI field,” says Marc Rothenberg, MD, director of the Cincinnati Center for Eosinophilic Disorders at the Cincinnati Children’s Hospital Medical Center. “There’s been a large increase in the number of patients being diagnosed with this disorder.”
“No one knows the reason for the rise—this disease was only described about 25 years ago—but the prevalence has risen over time,” says John Lee, MD, an allergist and co-director of the Eosinophilic Gastrointestinal Diseases Program at Children’s Hospital Boston. “I’m sure some of this is due to increased awareness. But the rise in EE parallels the rise in other allergic conditions, like food allergies, atopic dermatitis and asthma. The etiologic cause for these conditions is also undetermined. There are theories about environmental exposures predisposing us to allergic diseases—the hygiene hypothesis (our environment is too clean or germ free) or exposure to chemicals in our environment and foods—but none have been proven. My opinion is that the cause is likely multi-factorial.”
Like celiac disease, EE is a chronic condition that requires ongoing treatment. Unlike celiac disease, where one ingredient (gluten) triggers the disorder, there’s no one-size-fits-all trigger for EE. Many patients with EE have sensitivity to 10 or 20 different foods. Plus, there’s no easy-to-administer screening test for EE, as there is for celiac disease.
“EE doesn’t yet have a blood test,” says Rothenberg. “You have to do endoscopies over and over just to monitor the disease.”
Patients who are trying to reintroduce foods quickly may have as many as three to five invasive endoscopies a year, often under general anesthesia. During the endoscopies, doctors have to take three or four biopsies because the disease is not continuous; it may affect one area of the esophagus or digestive tract and not another.
The first step is to treat symptoms with strong antacid medicines called proton pump inhibitors (PPIs), such as Prevacid, to address any underlying reflux and rule out reflux disease.
“If someone has recurrent upper GI symptoms that do not respond to conventional treatment with PPI therapy and there is a strong history of allergy in the family, they should raise the question with their doctor, ‘Could this be an eosinophil-associated disorder?’” Rothenberg says.
A Special Diet
There are three main dietary options for EGID, along with medical treatments, often steroids, to fight inflammation.
Six-food elimination diet. This treatment removes six highly allergenic foods—milk, eggs, wheat, soy, nuts and seafood—from the diet without conducting allergy testing. Some researchers have found, however, that seafood isn’t usually a big EGID trigger and that beef, chicken, corn and other grains may be more involved.
Specific-food elimination diet. Foods are removed based on allergy testing with RAST blood tests and skin prick tests. However, EGID is a delayed allergy, not immediate or anaphylactic, so these tests are not always effective. Doctors also use patch tests, where small amounts of food are placed in aluminum disks and taped to a patient’s back for 48 hours to determine if there’s a delayed allergic response.
Elemental diet. This “no food” diet is the strictest and most effective form of dietary therapy, with more than a 95 percent success rate. The patient ingests only a hypoallergenic amino-acid based formula (such as Neocate or EleCare), thus avoiding all proteins that can prompt allergic response.
“To be on a formula-only diet can be psychologically and socially challenging for the child and the family,” says Lee, calling EE “one of the most challenging diseases I have dealt with as an allergist.” Lee uses an elemental formula as a nutritional supplement for toddlers and young children who have milk and soy removed from their diet and tries to introduce food back into the diet as soon as it’s safely possible after healing is achieved.
“Removing just one or two foods from the diet can result in a change of lifestyle and a change in how kids interact with families and friends in situations like birthday parties and school,” he says. “But like other things in life, families adjust to these diets and after a while, it can feel like a normal part of their life.”
Not Just Children
Although more prevalent in kids, eosinophil- associated disorders can affect adults, too. Jessica deCsesznak, a 20-year-old college student, was diagnosed with EE when she was 18 but she suspects she’s had the disease for much longer.
She had multiple food allergies her whole life–to most of the top eight allergens. Then as a teen, she kept feeling like food was getting stuck in her throat. The list of food triggers kept getting longer and longer. By October 2008, she found that she couldn’t tolerate any foods, so she was placed on an elemental diet only. She’s tried to reintroduce ten foods over the past two years but after a week or two of eating them, the feeling of food stuck in her throat returns. The only food she can tolerate is cotton candy.
“My doctors tell me that they’ve never seen an older patient like me not be able to tolerate any foods,” says deCsesznak, who receives formula throughout the day on a continuous feed pump, which she wears in a small backpack that connects to her gastrostomy tube.
“The first year on the G-tube, I felt really great. I was biking, swimming, running,” she says. But in the last year, major fatigue and brain fog have set in. It gets worse in fall, not a surprise since EE symptoms can be exacerbated by seasonal allergies.
“I haven’t been able to do as much as I used to,” says deCsesznak, who is majoring in integrative physiology at the University of Colorado at Boulder. “I go to class, I come home and I lie down for the rest of the day.”
Yet DeCsesznak remains hopeful. She recently started a rotation diet, in which she’s eating six different foods. She tries just one each day and then doesn’t eat it again for six days. While no studies have been conducted on treating EE patients with a rotation diet, the logic behind it is that some people have system overload when trialing the same single food for two weeks, which causes them to react.
DeCsesznak is also working to get off her continuous pump by slowly increasing the time that she’s off the feed.
In the United States, a handful of multidisciplinary EGID treatment facilities are now providing coordinated care from physicians across various specialties. Noted centers are at the Children’s Hospital of Philadelphia, Cincinnati Children’s Hospital, Rady’s Children’s Hospital San Diego and The Children’s Hospital Denver/National Jewish Health.
At a multidisciplinary center, a gastroenterologist assesses GI symptoms and monitors the disease through endoscopies, an allergist performs food allergy evaluations and manages concomitant allergic diseases, and a dietitian ensures that restricted diets contain enough nutrition.
Currently, there’s a gap—an average of two to four years—between initial onset of symptoms and diagnosis but that gap may be narrowing, says Furuta. The medical community is becoming increasingly familiar with the disease. The American Academy of Allergy, Asthma & Immunology included a day-long symposium on EE on the agenda of its 2011 annual meeting in March.
“There’s a lack of understanding and recognition of the disease. Hopefully that’s changing as more people learn the warning symptoms and realize that this is an allergy that can be treated with food elimination,” Furuta says.
Although awareness is increasing, more research is needed to find better treatments for EGID—and a cure.
“We’ve a come a long way in the past decade but we still have a long way to go,” Book says. “We need expanded education to reach the public and healthcare providers. Research funding is desperately needed if we are to see positive change in the future.”
APFED’s HOPE research fund, she says, helps advance the treatment of eosinophil-associated diseases through a peer-reviewed grant program. Grant recipients have made important contributions, including the development of a potential new therapy for EE and the discovery of a gene associated with the disease.
In Cincinnati, Rothenberg, a leading EGID researcher, is creating mouse models for the disease, developing genetic testing to find its causes and conducting clinical trials on new drugs. He also started the Registry for Eosinophilic Gastrointestinal Disorders, which groups together researchers and patients, allowing researchers to conduct larger, more effective studies and clinical trials.
Weinbach is heartened by physicians’ increasing knowledge and research into EGID, which has helped her son expand his eating options, including reintroducing steak in small amounts.
“With the help of specialists, we’ve learned a lot more about Aaron’s condition and are paying much closer attention to his symptoms,” Weinbach says. “We’re also staying on top of his emotional well-being to make sure that EE doesn’t take anything away from his self-confidence. Even though Aaron can’t eat a lot of foods, he has an incredibly positive attitude toward what he can eat—and we do, too.” n
Eve Becker (glutenfreenosh.com) is a Chicago-based health writer.