Fortunately, most patients get better by identifying and eliminating problem foods.
"Now, I can eat everything but three things. Before, I couldn't eat anything," said Eric Molchen, 14, of Morgantown, who was wasting away when he was diagnosed with EoE five years ago at Children's Hospital of Philadelphia.
In the 16 years since a groundbreaking study of EoE was published, awareness and research have exploded.
Still, there are more questions than answers, including: Does EoE signal a change in our food, our immune systems, or both?
Too much of a good thing
Eosinophils play a big role in the digestive tract. Sometimes they help engulf and kill invading microorganisms. For invaders too big to be gobbled up Pac-Man style, eosinophils carry toxic "granules" that they can thrust onto the enemy.
Eosinophils also help the immune system manage inflammatory disorders such as asthma and food allergies such as lactose intolerance.
But with eosinophils, as with other immune cells, too much of a good thing can be bad. A number of perplexing disorders, including ulcerative colitis, are associated with too many eosinophils.
EoE is yet another.
In biopsies of esophageal tissue taken from EoE patients, "not only are there too many eosinophils, but some have extruded their granules into the tissue," wrote Margaret Collins, a pathologist at Cincinnati Children's Hospital, the first in the country to set up a center dedicated to eosinophilic disorders. "This implies that substances . . . in the granules may contribute to the patient's symptoms."
A biopsy - an under-the-microscope look at cells - is the only reliable test for EoE, a national panel of experts concluded this year. But the diagnosis is neither simple nor straightforward.
For one thing, esophageal inflammation has various causes, the most common being gastroesophageal reflux disease, or "acid reflux" - another increasingly common modern affliction. (An estimated 30 million Americans suffer the heartburn, indigestion, and pain of acid reflux.)
For another thing, acid reflux patients can have eosinophils in their esophageal membranes, like EoE patients, and occasionally people have both diseases.
At Children's Hospital in Philadelphia, EoE has been diagnosed in more than 1,000 youngsters in the last 15 years.
"On biopsies in the 1980s, we never saw EoE," said gastroenterologist Chris Liacouras, codirector of the hospital's Center for Pediatric Eosinophilic Disorders. "It was only in the mid- to late-1990s that we started to see this disease. The increase parallels what we've seen with allergies in general."
How prevalent is EoE? A decade ago, experts estimated one in 10,000 people had it. Now, Liacouras said, a better estimate is one in 2,500 - not commonplace, but not that uncommon.
Seeking a diagnosis
In 1994, Michael Craig, a Navy communications specialist, was eating lunch on a ship in the Persian Gulf.
"All of a sudden, I felt like I was choking. I got sick before I ever made it to a bathroom," said Craig, 41, now a computer engineer who lives in Deptford.
The embarrassing episode kept recurring, and Craig began a 16-year quest for a diagnosis of his swallowing problem, or "dysphagia."
Although tests showed his gastric acid levels were normal, he was told he had acid reflux and was put on standard medication. As the dysphagia persisted, Craig began to hide while eating. "I didn't lose weight, but I started not eating at any social event," he said. "I wouldn't even eat with my family."
Regurgitating a morsel lodged in his esophagus could take hours, even though he cut his food into minuscule bites. Sometimes, the obstruction triggered chest pains that mimicked a heart attack. Occasionally, he had to rush to a hospital for removal of an impacted tidbit. A couple of times a year, a doctor would use a dilator to gently stretch open Craig's constricted esophagus.
Finally, last fall, a dysphagia attack at his daughter's wedding drove Craig to consult yet another doctor.
Brian Berberian, a gastroenterologist at Our Lady of Lourdes Medical Center in Camden, diagnosed EoE and put Craig on the recommended drug regimen: an acid reflux medication, an asthma inhaler, and an oral corticosteroid.
Craig will also see an allergist to try to identify offending foods, although EoE can defy even sophisticated testing.
He is grateful to finally know what ails him. But it hasn't yet gotten much better.
"The treatment options aren't all that great," especially for patients with long-term damage, Berberian said.
The 1995 study that put EoE on the medical map was conducted by Johns Hopkins University physicians.
They described 10 emaciated children, ages 8 months to 12 years, all with diagnoses of acid reflux. Despite dutifully taking antireflux drugs, they had chronic abdominal pain, vomiting, and feared eating.
Six of the children had even undergone a last-resort acid reflux surgery in which the upper part of the stomach is wrapped around the lower esophagus and stitched in place. It didn't help.
The Hopkins doctors suspected the real problem was food. They weren't the first to think so - previous studies had eliminated individual or multiple foods identified as allergens by skin-prick tests - but they were the first to convincingly show it.
Their method was draconian: Replace all food with a liquid formula containing amino acids - the molecular building blocks of food.
Within six weeks, all 10 children got better, and biopsies showed a drop in eosinophils.
Foods were reintroduced, one by one. Although some children were allergic to as many as six foods, all 10 were able to resume a normal, albeit restricted, diet.
The liquid "elemental diet" is now standard therapy when all else fails.
A toll on families
Dean Harris, 8, and his brother Cole, 7, are as cute as those TV commercial kids who name a detested food, then dig into a plate full of chicken nuggets.
The brothers would love to have just one detested food.
They are among the minority of EoE patients - Liacouras estimates 10 percent - who are hypersensitive to virtually all foods, not to mention other allergens.
"They've had problems since birth," said their mother, Suzanne Harris, a marketing consultant who works from their West Chester home. "We tried all sorts of elimination diets. They kept getting worse."
In November, specialists at Children's Hospital of Philadelphia put the two boys on the elemental diet at $100 a day. "It tastes and smells like vomit," Harris said. "We explained to the boys that you can drink the formula, or get it through a feeding tube. They're very brave kids. They chose to drink it."
Recently, the boys' biopsies showed normal eosinophil counts, so they tried reintroducing chicken. It made Cole sick.
Last week, they tried brown organic rice. "Cole has been complaining that his tummy hurts," Harris said. "I picked him up today at school and he vomited."
The emotional toll on the family is huge. Harris says she and her husband, Steve, guiltily "grabbed a protein bar and hid in a closet to eat" when the boys went on the formula.
But their courage is even greater. They created sokidscanbekids.org to raise money for EoE research. On Friday, they will host a race/walk.
"My hope, obviously, is for a cure, or for some breakthrough drugs," Harris said.
Theories and research
Researchers have zeroed in on possible genetic markers in EoE patients. They are also testing a new drug that targets the protein that signals eosinophils to infiltrate the esophagus.
But no one has a solid theory to explain what's going on.
"I think it has to do with food preservatives," Berberian at Lourdes said.
Pediatrician Marc Rothenberg, at Cincinnati Children's, said EoE is "a disease of Western industrialized countries." Yet he believes the food supply is safer than ever.
"Everything you eat is a foreign body for the immune system," he said. "People used to die from milk poisoning because it wasn't homogenized. Just because some people become allergic to food doesn't mean we have poisoned our environment."
Kristina Molchen isn't so sure.
Five years ago, her son Eric, then 9, was a skeletal 40 pounds - and dropping - despite acid reflux medicine and frequent hospitalizations. Eating sent him into paroxysms of abdominal pain.
That's when she and her husband, Mike, took drastic action. With the help of specialists at Children's in Philadelphia, they took Eric off food, quarantined his dishes, then rebuilt his diet - one food and biopsy at a time. They also moved from Roxborough to rural Morgantown, where Kristina Molchen grows much of their food.
At 5-foot-3 and 100 pounds, Eric is now a robust, hockey-playing eighth grader who avoids the three foods that, oddly enough, turned out to trigger his EoE: peas, peaches, and carrots.
"When I counsel other parents, I don't know why it is" that EoE exists, Molchen said. "The only thing I can tell them is how we helped Eric live with it and what we did to keep him safe."
Contact staff writer Marie McCullough at 215-854-2720 or firstname.lastname@example.org.