Center for Pediatric Eosinophilic Disorders

About Eosinophilic Disorders

• What is eosinophilic esophagitis?
• What are the symptoms of eosinophilic esophagitis?
• What causes EE?
• Who gets EE?
• How is it diagnosed?
• How is it treated?
• Why is EE becoming more common?
• Will my child's EE go away?
• What does an EE diagnosis mean to my child's future?
• What should you do if you think your child may have EE?

What is an eosinophil?

An eosinophil (ee-oh-sin-oh-fill) is a type of white blood cell. Eosinophils are the least common of the white blood cells and make up about 1 to 4 percent of the blood's cells. Named after "Eos," the Greek goddess of dawn, eosinophils are characterized in their laboratory slides by their bright red-pink color and double nucleus. Eosinophils are most commonly associated with allergic diseases and parasite infections.

What is eosinophilic esophagitis?

Eosinophilic (ee-oh-sin-oh-fill-ick) esophagitis (EE for short) is an allergic reaction in the esophagus, the tube that carries food from the mouth to the stomach. The foods that trigger EE vary from person to person.

During an allergic reaction, various cells congregate and cause symptoms like redness, swelling and itchiness. A white blood cell called an eosinophil (ee-oh-sin-oh-fill) is one of the types of cells behind an allergic reaction. Eosinophils are an important part of the immune system and there are always small quantities in the blood and intestine fighting parasites and performing other duties. However, eosinophils cause problems when they appear in high quantities in areas other than the blood and intestine. If you have seasonal allergies, eosinophils are in your nose; if you have asthma, they are in your lungs; and if you have EE, they are in your esophagus.

The suffix "-itis" means inflammation. So eosinophilic esophagitis means an inflammation of the esophagus caused by eosinophils. The disease is commonly called EE but you may also hear it referred to as EoE.

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What are the symptoms of eosinophilic esophagitis?

Children experience nausea, regurgitation, vomiting, abdominal pain and a burning feeling similar to acid reflux (heartburn). They may have difficulty swallowing and gag frequently. Often, they feel like something is stuck in their throat. This is called dysphagia. If EE goes untreated, the esophagus may narrow because of scarring. This is called stricture.

Infants with EE don't want to breastfeed or take a bottle, and may frequently spit up and arch the back, a sign of pain. Older children eat reluctantly and slowly, don't grow as quickly as expected, develop seemingly irrational aversions to certain foods, and may have trouble sleeping. They may complain of heartburn and dysphagia. Sometimes EE is diagnosed after a child or teenager comes to the hospital with food in the esophagus that he or she is unable to cough up. This is called food impaction.

EE shares many symptoms with acid reflux, so a doctor will first prescribe an antacid like ZANTAC. These medicines do not help EE, so if symptoms persist, the doctor will know reflux isn't the cause. It's estimated that up to 10 percent of children with reflux have EE.

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What causes EE?

We don't know what causes EE, or why children from higher-income families are affected more frequently. There may be a genetic basis for EE — often it seems to run in a family. Some point to the hygeine theory, which posits that without enough exposure to germs, the immune system becomes weak and out of practice, and turns on itself. According to this theory, children from homes that are spacious and clean are moer likely to develop certain illnesses, including EE.

The hygeine theory is just that — a theory. The bottom line is that we don't know what causes EE. We have a lot to learn about the disease.

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Who gets EE?

EE affects children of all ethnicities and family income levels. Children with EE often have other allergic disorders like asthma or eczema.

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How is it diagnosed?

The only way to diagnose EE is by a biopsy of the esophagus, performed by a gastroenterologist. The child is sedated and the physician inserts an endoscope into the mouth and through the esophagus, stomach and upper part of the small intestine, where a small sample of tissue is taken. Sometimes, the gastroenterologist can see signs of EE in the esophagus, such as "rings" or "white plaques". However, in many cases a child can have EE and esophageal tissue that appears to be normal. Therefore, it is critical to obtain an esophageal biopsy sample.

Typical allergy tests, such as skin or blood tests that identify immediate allergic reactions, are not usually effective for diagnosis of eosinophilic esophagitis because the allergic reacting involved in EE is often delayed several days after an allergen is introduced. A skin patch test is being developed but is not routinely offered by most allergists.

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How is it treated?

Almost always the underlying cause of eosophilic esophagitis is a food allergy. A gastroenterologist or allergist may implement dietary restrictions to pinpoint the food that triggers the allergic response. The most commonly involved foods include:

• Milk
• Eggs
• Tree nuts
• Beef
• Wheat
• Fish
• Shellfish
• Corn
• Soy

However, almost all foods have been implicated. Some patients may simply be allergic to a single food while others may be allergic to many foods. Because allergy tests are often unable to determine the causative foods, complete elimination of all foods may be required. In these cases, patients must be placed on a strict elemental formula for one to three months in order to heal the esophagus. After this, foods are slowly reintroduced in an attempt to discover the food(s) causing the allergy.

Repeat endoscopy with biopsy is often necessary. Several medications have been tried including corticosteroids, cromolyn sodium and leukotriene inhibitors. While these medicines reduce esophageal inflammation, when withdrawn, the disease recurs.

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Why is EE becoming more common?

Thirty years ago, EE was unknown. Diagnoses have risen dramatically in the past five years. We don't know whether this is because the disease is actually becoming more common, or because it's being recognized much more often. Probably the rising number of cases is due to some combination of the two factors. EE is still a rare disorder, occurring in an estimated 1 in 3,000 children.

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Will my child's EE go away?

Some children fully outgrow EE, just as they outgrow other allergies. Others partially outgrow it — the number of foods that trigger it decreases over time. But some children don't outgrow it at all. Because EE is a new disorder, we can't tell you what the chances are that your child will outgrow it. As the current population of children with EE ages, research will tell us more.

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What does an EE diagnosis mean to my child's future?

With careful attention to diet, your child will lead a normal life, even if his or her EE never completely goes away. However, we don't know the long-term effects of EE. Some results of the disease, such as scarring of the esophagus, are troubling. As the current population of children with EE enters adulthood, we will be able to answer more questions about its long-term health effects.

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What should you do if you think your child may have EE?

If you think that your child may have eosophinilic esophagitis, contact your primary care physician. He or she may then refer you either to a pediatric gastroenterologist or allergist for further evaluation.

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