Endoscopy #2 - Preview

While upper endoscopies are fairly routine outpatient procedures there are risks involved. Please pray for Oliver and his Doctors when he goes in for his next (of many, many more to come) endoscopy.

Oliver's health hasn't been the best over the last little bit. He isn't sleeping well at night and he is back to throwing up fairly regularly. The worst part is he's not drinking very much of his formula. We told the doctor we were ready to perform another endoscopy and we have one scheduled for Dec 1st. We are excited about this date because it means we'll hit our maximum out of pocket expenses with the insurance and this endoscopy will cost us less than the last one did. In January all of that resets, of course.

What does this mean?

• A more accurate diagnosis. For his second endoscopy Oliver will be on his Proton Pump Inhibitor (PPI) which is an important condition for an actual diagnosis of EoE. PPIs are commonly prescribed for acid reflux. Acid Reflux can weaken the esophagus and result in the presence of some eosinophils in the esophagus - usually a very small amount. In order to rule out reflux as the cause for the eosinophils found in the last endoscopy Oliver has been on a PPI ever since. I hope we'll be able to take him off of it after the next scope because long term use of PPIs can have some negative side effects. It's a difficult dance because it is very possible and quite common for a child to have both EoE and some type of GERD. If there are no eosinophils in the biopsy of his esophagus (this would be good news, mostly) we won't know if it is because of the PPI or because of the diet change. If there are eosinophils we can be sure that he has EoE but we still won't really know if he needs the PPI. The one thing we will know is that he is still reacting to something. It could be food or environmental, but most likely it will be a food.
• A snapshot of his current condition. Oliver has been off of his Corticosteroids (the flovent) for quite a while (which is why we think that his symptoms have returned). This is important because the steroids not only mask the symptoms but also can prevent the formation of the eosinophils. If he were on the steroids the results of the biopsy wouldn't accurately inform us as to whether or not his diet is helping his condition. Since Oliver doesn't communicate well with us the results of the biopsy are very important.
• A tool to plan the next stage of treatment. Alison and I suspect that Oliver's diet still includes some food(s) to which he reacts. These symptoms were masked when he was taking the steroids but now that he is off of the steroids they are manifesting themselves. The results of the biopsy will confirm or contest this hypothesis.

What do we do if he scopes clean?

First, this is not the outcome we expect. In many ways this would be good news, but it also comes with complications. First we have to remove the PPI and scope again in a couple of months to determine whether it was the PPI or the diet which caused the reduction. Either one would be a great thing. Knowing which one it is would be an even better thing.

But, if the eosinophils are gone, why is still irritable and throwing up? This would open up a new mystery.

What do we do if he still has eosinophils?

This is the big question, what to do next? We will need to eliminate more foods from his diet if the biopsy reveals eosinophils in the esophagus. Which foods?

• Our GI has proposed we do another round of RAST tests to see if Oliver demonstrates a sensitivity to anything new. I don't like the idea because RAST testing is considered the least effective of the three allergy testing methods in identifying foods which have a non-IgE mediated reaction like EoE. I would prefer skin prick testing, or better yet, atopic patch testing.
• Another option is to simply remove some popular "offenders" like corn and beef. This wouldn't leave much for him to eat.
• The most drastic action is to remove all proteins and put him on a pure elemental diet. This is tempting only because it would increase the likelihood that insurance would cover the costs of his formula. We really don't want Oliver to have an aversion to foods so we would like to keep him eating some foods if we can, but we also acknowledge that many EoE patients end up on a pure elemental diet at some point, either as a tool in identifying "safe" foods or because they haven't found they can tolerate any foods at all.

What do we do in the meantime?

We want the endoscopy with biopsy to provide the most valuable set of information possible for us. In order for this to happen we need to make sure Oliver is on his PPI and off his steroids. Also, we need to be super vigilant and make sure he doesn't eat any of the 7 foods we have eliminated. (Milk, Wheat, Soy, Egg, Peanut, Chicken, Beans) If he were to sneak a Cheez-It from his brother during the week before his endoscopy, this would greatly reduce the information we can learn about his current diet. Strict adherence to his diet leading up to his endoscopy is really important. Too bad Oliver doesn't understand that. Hopefully his brothers do.

Will He Grow Out Of It?

"Will he grow out of it?" is a question I get a lot. The short answer is no. The long answer is, well, read on.

It's fairly common knowledge that children often grow out of food allergies. Eosinophilic Esophagitis isn't a typical food allergy. Chances are that Oliver has "normal" IgE mediated food allergies* that don't affect his EoE and others that do. For example, blood tests have told us that Oliver has an awful lot of IgE antibodies when milk is introduced to his system. This very likely means that he has an IgE mediated allergy (I say very likely since these blood tests aren't always accurate, especially for young children). Since drinking milk (or even just touching milk) gives him an eczema rash, we tend to think that's a true allergy for him. But, milk may not trigger his EoE. If it turns out that it doesn't trigger the EoE, he could very well grow out of the allergy. If it does trigger the EoE, he will always need to avoid milk.

How do we know what triggers the EoE? First we have to get to a point where we've eliminated everything that might possibly cause a reaction. Hopefully he will have another endoscopy before the end of the year. Either it will be clear (meaning no eosinophils) or not clear. If it doesn't come back clear, then we need to eliminate more foods (while still keeping him off of the ones he's already off). If it comes back clear we know we've eliminated the offending foods and we can start to add some back because may have also eliminated foods that really aren't triggering his EoE. If we are able to add a food back with no reaction from him (we'd probably need another endoscopy to know for sure), then that food can go on his okay list.
Basically it's a long game of trial and error.

Clear as mud, right?

*IgE antibodies are produced when immune cells encounter the food, the antibodies see the food as an invader and trigger an allergic response. This itself has nothing to do with EoE other than the fact that many patients have IgE allergies along with their EoE and testing for IgE allergies can be a good (and much less invasive!) way to guess what EoE triggers might be. For some people, their IgE allergies ARE their EoE triggers straight across the board, but I don't believe that is very common. (last paragraph updated 12-13)

What can I do?

There are several things anyone can do to support those with Eosinophilic Disorders.

1. Write your congressman and encourage him or her to become a member of the bipartisan Rare & Neglected Disease Caucus chaired by Representatives Joseph Crowley and Fred Upton. For more information on the caucus and how to contact your representative click here. Already did that? Well, you just might have a new congressman who needs an invitation.
2. Donate to the Cured Foundation (Campaign Urging Research for Eosinophilic Disease). CURED is a charity that we are actively involved with that raises money to find a cure for EE. CURED has donated over $2 million to research over the past 6 years. CURED has begun a $1 challenge. They are looking to find 500,000 people to each donate $1 or more to CURED, since that is something that almost everyone can do in these hard economic times. If you are interested in donating, please mail it to CURED at P.O. Box 32, Lincolnshire, IL 60069. Or visit their web page and donate via Paypal.
3. Donate to APFED Hope Research Fund or signup with iGive.com or BuyForCharity.com and choose APFED as your charity of choice.

The Mother of All Food Allergies

EoE has been called by some the Mother of All Food Allergies but it is so different from "normal" food allergies. The mechanisms which cause negative effects which we normally associate with allergies are different for EoE than they are for EoE. What does this mean?

Up until now, our allergist has only performed RAST (blood IgE) tests to determine to which foods Oliver has a sensitivity. The RAST test is moderately effective in determining which foods may cause a typical reaction in patients with normal food allergies. These tests tend to deliver false positives and can be followed up by food trials to determine clinical results. The RAST test generally is not used to determine foods which trigger EoE reactions because it delivers too many false negatives. Skin Testing and Patch testing have proven more effective for determining offending foods for EoE.

So, for now we follow the diet proscribed by the RAST tests and Oliver doesn't eat any milk, wheat, soy, egg, chicken, peanut, or black beans - or products with any of those ingredients. Hopefully after a follow-up endoscopy we'll know whether we have eliminated enough.

• A clean endoscopy would mean we have eliminated all the offending foods. We would be able to then trial some of the foods we have removed to find out if he really can tolerate them or not.
• Poor results will mean we have to identify some other foods that may be causing problems. This doesn't mean that the ones we have removed are offenders - some of them may not be but we can't really try adding any foods back in until we have a symptom free esophagus to work with. -- Unfortunately this is what I fear will happen and it will be even longer until we can determine of what Oliver's diet will consist.

Next time we see the allergist I'm going to ask him about patch testing. At the very least I think it will be necessary if his follow-up endoscopy (to be performed at an undetermined future date) has negative results.

Learning in Slow Motion

Dad here:

One of the most frustrating things about this disease is that it affects each person differently. What causes one patient to react won't affect another. It is like each EoE patient has their very own personalized version of the disease. I've read dozens of articles about EoE. I've attended the APFED Conference. I've done lots of research so I feel like I know a lot about the disease, at least as much as is commonly known (there is much that isnt' known.) The problem for me is I know nothing about Oliver's version of EoE.

It's been 4 months since Oliver had his first Endoscopy and was diagnosed with EoE. He's been through several different medications. We have eliminated 7 foods to which he may or may not have a sensitivity. Has it made a difference? Yes, he is definitely doing better but I want to know exactly which foods cause Oliver's version of EoE to flare up. I want another endoscopy to see if the changes we made have had a real effect. Everything moves so slow. I don't want to torture our little guy with constant endoscopies, but I want scientific data. I don't know with certainty that any of the foods we eliminated are causative with regards to his EoE.

I know it will take years to get Oliver to a stable state (or maybe he'll never be there - I've heard recently of allergies morphing) but it still bugs me. I tell people this all the time. I tell myself this all the time, but it isn't any comfort. The process is just too slow for my linking.

All I know for certain right now is: He had eosinophils in his esophagus 4 months ago. He develops a rash (eczema) when exposed to milk (orally or topically). When we eliminated wheat his stools improved. Topical Corticosteroids administered orally seem to make him feel better. I want to know more. I want the process of investigation and learning about Oliver's specific version of EoE to speed up, but there doesn't seem to be any hope of that.

I guess I should just be grateful we got a diagnosis so quickly. Many people don't receive an accurate EoE diagnosis for many, many years.

TKO

Our ordeal with the Insurance company has come to an end for now. Unfortunately we are the ones on the mat. TKO! We've lost our fight.

• Our appeal began with a request for a pharmacy exception as an effort to get our insurance company to cover some of the costs of an elemental formula. The formula costs $196 for a case of 6 cans of formula which Oliver consumes in about 15 days. Obviously, as he grows his nutritional needs will increase and he will consume the formula at a faster rate.
• July 26 -- Our drug request was denied because "EleCare Formula used to treat Eosinophilic Esophagitis are specific exclusions and are not covered benefit on this members plan."
• Sep 15 -- We appealed the adverse determination but the denial was upheld because "this is not a covered benefit per [our] benefit plan."
• Oct -- We requested an internal panel review hearing.

Today we had the Internal Panel Review Hearing. It was the last and final step in the appeal process. In preparation for the hearing we supplied the panel with copies of Oliver's medical records. Oliver's GI, PCP, Allergist, & Nutritionist wrote letters on Oliver's behalf explaining the importance of EleCare in his treatment as well as citing medical articles which indicate that elemental formula is the accepted medical practice for children with EoE. We also furnished complete copies of a dozen journal articles about EoE and its treatment. After all of this work the insurance company simply reminds us that "PHP determines whether a healthcare service or supply is a Covered Benefit. The fact that a Provider/Practitioner has prescribed, ordered, recommended, or approved a healthcare service or supply does not guarantee that it is a Covered Benefit even if it is not listed as an Exclusion."

The hearing went something like this:

• The participants were introduced
• We stated our case - i.e. that Oliver needs EleCare, it has been beneficial for him, and it is what all the doctors recommend
• The doctor for the plan stated their case - i.e. they don't cover Nutritional Supplements
• We were asked how much formula he consumed (currently 24-30 oz. per day) and what other foods he consumes
• We left and the panel deliberated

Based on our conversations it would appear that because EleCare is not Oliver's "sole source of nutrition" it is a supplement and therefore not covered. We contend that EleCare is Oliver's primary source of nutrition and the small amounts of fruits and beef that he eats are a supplement. He receives between 750 and 900 Calories a day from his formula, as well as a complete set of micro-nutrients, vitamins & minerals.

One of the big problems with the whole process is I still don't know what I needed to prove to the insurance to get them to cover his EleCare . . . perhaps there is nothing we could have done. It would appear that the appeal process is simply a formality to satisfy the requirements of the state, not something designed to help the consumer.

Where do we go from here? Now that we have a complete and utter denial of coverage from the Insurance provider we will look to other sources for help. The manufacturer has an assistance program, will we qualify? I'll let you know.

Things That All Make Sense Now

EoE symptoms seem to vary from person to person. Young children often experience nausea and vomiting, failure to thrive, feeding refusal/intolerance or poor appetite, and difficulty sleeping. Older children and adults tend to experience dysphagia (difficulty swallowing) and food impaction (food gets stuck in the esophagus). Reading lists of symptoms and reading what others have experienced with their children is making it all click inside my head.

I've nursed Oliver and have always thought he's had latching issues. He also would eat quickly and fairly frequently, which seemed odd (in comparison to my other two children). I didn't worry too much because this was back when he was actually gaining weight! By about six months old he either got the hang of it, or I got so used to it that I didn't notice it so much. At this point, though, he started vomiting (this probably is due to us introducing solid foods to him). Not throwing up, but actual vomiting. He would throw up on average once a week. It would come and go so that once I was worried about it, he would stop for awhile.

He was not a very good napper when he was little. He wanted to be held and would only sleep for short spurts. It's a lot like my oldest son, so I wasn't too alarmed. However, I did not know how to put him to sleep. He wouldn't nurse to sleep, he wouldn't rock to sleep, nothing worked. This bothered me a lot. He would wake up screaming in the night and I could do nothing to soothe him back to sleep. In retrospect, I'm sure his stomach was hurting him and there really was nothing we could do.

Don't Google You Son's Rare Disease

Last night I was up late. I decided to do a google blog search for eosinophilic esophagitis. Bad idea? Maybe. I found several blogs of parents who are dealing with this, and now I know that this could end up to be a lot worse than I ever imagined. On the flip side, it's good to know that we are not alone in this.