Eosinophilic Esophagitis.....

What a mouthful, right?

Gavin just got diagnosed with this chronic, long-term, seemingly heart-breaking disorder. I hate to say this, but in a way, it's almost worse than Grace's cardiac defect. At least with Grace, she doesn't experience chronic pain ALL day EVERY day. I know that is silly, but at this point, I am 100% overwhelmed.

Frustrating to think of two very chronically "ill" children. I had dreams of going back to work someday, now that seems doubtfully possible. Our days are filled with appointments, therapies, feeding battles (for both), and sleep, sweet, sweet sleep. I'm a self-described loner these days. Except for a very few close friends, it's hard to find others that understand me and my kids, and what we're going through. I'm having a pity-party day- and I'm in a poopy mood.

This too shall pass.... right?

Here's some info I found about the disorder. Happy reading!

What is Eosinophilic Esophagitis?

Eosinophilic Esophagitis (“EoE”, formerly “EE”) is an allergic inflammatory disease characterized by an infiltration of increased numbers of eosinophils in the esophagus (the tube that runs from the mouth to the stomach). This infiltration causes inflammation of the tissue lining the esophagus.

In a healthy person, there are absolutely no eosinophils in the esophagus. There can be a few in the lining of the gut, but there should be none in the esophagus. A few can be found with reflux. A biopsy with an eosinophil count of 15-20 or more per high power field (under the microscope), may be caused by Eosinophilic Esophagitis. Other potential causes are Gastroesophageal Reflux Disease (GERD), food allergies and irritable bowel disease.

Unfortunately, many people go undiagnosed for years, suffering with what can be severe symptoms. Some people aren’t diagnosed until they are teens or adults and seek treatment when food has become impacted in their esophagus.

Recent studies at Cincinnati Children’s Hospital have shown that Eosinophilic Esophagitis is even more common than other well known diseases that affect the gastrointestinal tract such as Crohn's Disease and Cystic Fibrosis. Eosinophilic Esophagitis is the most prevalent of the Eosinophilic Gastrointestinal Disorders.

The current (*) estimated prevalence of Eosinophilic Esophagitis is 1 in 2000.

(*estimate as of 2007)

Symptoms of Eosinophilic Esophagitis include:

Reflux that doesn’t respond to acid reducing medication (i.e. Proton Pump

Inhibitors, such as Prevacid, Prilosec and Protonix)

Nausea

Vomiting

Dysphagia (difficulty swallowing)

Food impactions (food gets stuck in the throat)

Abdominal or chest pain

Failure to thrive (poor growth or weight loss)

Malnutrition

Poor appetite

Early satiety (feeling full sooner than you should)

Difficulty sleeping

In addition to the above, some people also experience pain in their lower limbs (legs, ankles & feet) which is not uncommon with GI disorders in general, ear infections, asthma, croup, migraines, mysterious fevers, and more frequent “colds” when they are reacting to a food. Behavioral changes have been reported in some children (*) and Autism Spectrum Disorders are not uncommon. (*If your child has ongoing behavioral issues, social skills difficulties or any developmental issues, you should speak to your child’s Dr and consider an evaluation by a Developmental Pediatrician.)

Diagnosis:

A diagnosis is made when an endoscopy is performed (by a Gastroenterologist) and multiple biopsies are taken. Eosinophilic Esophagitis can NOT be diagnosed by symptoms alone. An endoscopy with biopsies is the only way to properly diagnose EoE. Even if the esophagus looks fine to the person performing the scope, the biopsies may still show EoE. It should be noted that even if the esophagus looks normal, the presence of eosinophils (which would be seen in the biopsies) can, and often will, still cause symptoms. However, the Dr. may also see rings or furrowing, thickened folds, microabcesses, white plaques, etc. The pathologist will also look for tissue injury, swelling and thickening of the esophageal layers. With Eosinophilic Esophagitis, the eosinophils are limited to the esophagus and not found in other areas.

The formal diagnostic criteria should be in place soon, but Eosinophilic Esophagitis can be diagnosed when the number of eosinophils in a esophageal biopsy is greater than 15 – 20 per HPF (high power field) under the microscope. Multiple biopsies need to be taken, as it tends to present in patches. It is recommended that 4-5 biopsies be taken from each area of the esophagus: 4-5 from the top (proximal), 4-5 from the middle, and 4-5 from the bottom (distal esophagus), because with any less, it may be missed.

Once Eosinophilic Esophagitis has been diagnosed, food allergy testing is usually recommended to guide treatment. Skin prick testing (SPT) to different foods is the most common type of allergy testing, and may prove helpful, but EoE is caused by a delayed reaction, not an immediate reaction as is tested for via SPT. Patch testing, which looks for delayed reactions, is also being used with some success. However, it is not uncommon to have negative allergy testing and still react to those foods that are being tested. An EoE reaction can range from days to weeks as the number of eosinophils increase and cause damage to the esophagus.

Please note that if you or your child have previously had an endoscopy, but the number of eosinophils was not quantified (counted), you are still able to ask the doctor to have the biopsy slides reviewed and have them counted. Even if the biopsies are several years old, they can be pulled from storage (they are archived) and the can be reviewed / re-read. The report can then be amended to indicate the eosinophil count, even if it is zero.

Treatment:

There is NO cure for Eosinophilic Esophagitis, but the goal of treatment is to eliminate the eosinophils in the esophagus, thereby alleviating symptoms. The treatments include dietary restrictions and medications. Since EoE is usually food-driven (caused by a reaction to certain foods), most kids and adults with EoE respond well to dietary treatments, and this may be all that is needed for many people. However, once treatment is discontinued, the eosinophils along with the damage they do, will return. Treatment needs to be ongoing, which may mean continuing to avoiding the food triggers and/or taking medication.

Elimination Diet: Dietary restrictions are guided by food allergy testing (skin prick testing, RAST, and patch testing). Some doctors are recommending that the top 8 allergens be removed from the diet, in addition to the foods that were identified via allergy testing. The top 8 allergens include milk, egg, peanut, tree nut, soy, wheat, fish, & shellfish. Beef is also a common trigger of EoE, as are certain other foods. However, a person with EoE can be reacting to any food or combination of foods. While allergy tests are used to guide an elimination diet, there can be false negative and false positive test results. You only have to eat one food that was a “false negative” in an allergy test for the elimination diet to fail. When an elimination diet does not do enough to clear the GI tract of eosinophils (as evidenced by scope with biopsy), sometimes a stricter diet is needed. This may mean just removing some additional foods from your diet, or going directly to an elemental diet. Food trials can begin once the symptoms have resolved and the eosinophils are gone, as confirmed by a clear scope. They involve adding back one food ingredient at a time, looking for a reaction, to determine which specific foods are causing a reaction. Typically, one single food ingredient is trialed for a 2 week time, looking for a reaction. Some Drs vary the time period by patient (1-3 wks, typically). If no reaction is seen, another food can be added and the same time period applies. Usually, when 3-5 foods are added back, the person has another endoscopy and the Dr looks to see if the eosinophils have returned. If not, the foods are ok for that person to eat and they can move on to additional food trials. If the scope shows that the eosinophils have returned, all of the foods that have just been trialed must be removed from the diet once again. It could be that the person is reacting to only one of the foods, but without noticeable symptoms to confirm which one, they must all be suspected. Some people need to scope between fewer foods. Some even have even found that they need to trial only one food per scope, but this is not the norm.
Elemental diet consists of a medical food (elemental formula), without any proteins, either in its whole or incomplete form (pre-digested or hydrolyzed). Elemental formulas are made of amino acids (the building blocks of proteins), fats, sugars, vitamins and minerals. Amino acids do not cause allergic reactions but whole or partial proteins can.

Although new flavors are available to make the formulas more palatable, kids and adults who need elemental formula may have a difficult time drinking enough of it. To maintain proper nutrition, some require enteral feeding tubes, to allow the formula to go directly into the stomach.

Medications for Eosinophilic Esophagitis most commonly include steroids to control inflammation and suppress the eosinophils. Steroids are used if dietary changes do not resolve the symptoms. They can be taken orally or topically (swallowed from an asthma inhaler). Side effects from steroids often limit long-term use of oral steroids. Without removing the cause of the symptoms via dietary restrictions, the eosinophils will return once the medication is discontinued. The doctor will determine which, if any, medications are appropriate for each individual.