Monday, September 24, 2012

How are EGIDs diagnosed and treated?

How are EGIDs diagnosed?

Diagnosis of an EGID requires an endoscopy/colonoscopy and biopsies.  Visually, the endoscopy may show furrowing, thickening of esophageal folds, abcesses, white plaque, and other issues.  Alternately, it may show nothing.  Without a biopsy and a skilled pathologist, the right diagnosis cannot be made.

Aidan's scope was visually clear, but his biopsies showed very high numbers of eosinophils.

A pathologist will review samples taken throughout the digestive tract, looking for tissue injury, swelling, and thickening of tissue.


Once an EGID has been diagnosed, food allergy testing is usually recommended to guide treatment and/or food reintroduction.

Skin prick testing (SPT) to different foods is the most common method of allergy testing, but since EGIDs are caused by a delayed reaction, SPT is of limited use.

Aidan's SPT so far has shown allergies to peanuts, peas, and soy. 

Patch testing, which looks for delayed reactions, is frequently used with some success.  But it's not at all uncommon for a food that did not show positive on an allergy test to actually be an EGID trigger food. 


Aidan's Patch Testing has revealed allergies to milk, egg, wheat, rice, corn, oat, barley, potato, beef, green bean, carrot, peach, and apple.

So Then What?  How do you treat this??


There is no cure for EoE.  No cure for any of these EGIDs.  The goal is to control the flares and alleviate symptoms.  Treatments take two approaches - Diet and Medication.


Elimination Diet: Dietary restrictions are guided by food allergy testing. Some doctors recommend that the "top 8 allergens" (milk, egg, peanut, tree nut, soy, wheat, fish, shellfish) be removed from the diet, in addition to the foods that were identified via allergy testing.  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.

Aidan's failure to thrive was so scary that alongside his doctors, we chose to bypass the elimination diet altogether.

Food trials
involve adding back one food ingredient at a time while observing for a reaction.  Most doctors recommend that food trials not be started until symptoms have completely resolved and no eosinophils are seen in the biopsies (a "clean scope").  Methods vary, but patients are often scoped and biopsied after each food trial, which can last 2-3 months.  It's a slow process, and patience is really important.

Aidan has trialed (and failed) rice and flavored formula.  A trial (and a failure!) is pretty emotionally difficult, and we're in no rush to try again.  We will, soon, but not yet.  

Elemental diet consists of a prescription medical liquid nutrition without any food proteins whatsoever. Elemental formulas are made of amino acids (the building blocks of proteins), fats, sugars, vitamins and minerals.

Aidan drinks Neocate Jr (with a side of Calamari).




Elemental formulas are available in a variety of flavors, but they don't taste very good and patients often have a hard time drinking enough.  For those people, feeding tubes can help avoid malnutrition and failure to thrive.

Aidan was ultimately given a feeding tube for just this reason.

Medications for EGIDs generally involve steroids to control inflammation and suppress the eosinophilic reactions. Steroids are normally used only if dietary changes do not resolve the symptoms. Side effects from steroids often limit long-term use, and without removing the cause of the symptoms via dietary restrictions, the eosinophils will return once the medication is discontinued.

Aidan did try a steroid, but it didn't work out well for him.  Your mileage may vary.
 
Many patients (Including Aidan) are also on high-dose proton pump inhibitors (PPI's) like Prilosec.  This helps control the reflux symptoms that can also be the cause of failed scopes.



Next week - Aidan's tests and diagnosis!

Monday, September 17, 2012

What is EoE?

It all starts with the eosinophils.  An eosinophil is a type of white blood cell that helps our bodies fight off infections.  Eosinophils are present in our bodies for a number of reasons, including food/environmental allergies, infections, parasites, leukemia, and other medical issues.  One such issue is Eosinophilic Esophagitis.

Why is this thing smiling??


Eosinophilic Esophagitis (EoE) is an allergic inflammatory disease characterized by increased numbers of eosinophils in the esophagus.  In a healthy person, there are no eosinophils present in the esophagus.  But in a patient with EoE, the esophagus can be teeming with them.  These eosinophils can cause irritation and damage, and often quite a bit of pain.

EoE is the most common of the Eosinophilic Gastrointestinal Disorders (EGIDs), but it's not the only one.  When the eosinophils present themselves in the stomach, it is called Eosinophilic Gastritis (EG).  In the stomach and small intestine, it's called Eosinophilic Gastroenteritis (EGE).  In the colon/large intestine, it's Eosinophilic Colitis (EC).

Some recent studies at Cincinnati Children’s Hospital have shown that Eosinophilic Esophagitis is even more common than more well-known GI diseases like Crohn's and Cystic Fibrosis.


The most common symptoms of EoE include:
  • Reflux that does not respond to medication
  • Difficulty Swallowing
  • Food impaction in the esophagus
  • Nausea and Vomiting
  • Poor Growth or Weight Loss
  • Malnutrition
  • Abdominal or chest pain
  • Poor appetite and refusal to eat
  • Difficulty sleeping due to pain or nausea

 The most common symptoms of lower-GI EGIDs (EG, EGE, EC) include:
  • Nausea or Vomiting
  • Diarrhea
  • Poor Growth/Weight Loss
  • Abdominal or chest pain
  • Reflux that does not respond to medication
  • Difficulty swallowing
  • Food impactions
  • Gastroparesis (Delayed emptying of the stomach)
  • Anorexia/Poor Appetite
  • Bloating
  • Anemia
  • Blood in the stool
  • Malnutrition
  • Difficulty sleeping

It's a lot to deal with - but a diagnosis is the first positive step toward feeling better.

(Big thanks to NJPAEOS for this information!)

Next week - how do you diagnose an EGID - and THEN what?

Wednesday, September 12, 2012

How To... Overnight Bag!

Does he need to stay overnight?

Nope!  We'll be home this afternoon!

Sooo...  What's that?

My overnight bag.


Yeah, I've met our son.  And I've been to CHOP.  There's no way whatsoever I'd be setting foot in that hospital with that kid without an overnight bag in the car.  And I've gotten really good at packing one.  I feel kind of like a foreign correspondent, or a high-powered consultant.  Overnight-bagging is an art form.  I used to bring soooo much more.  But I've got it down to a science.  The barest essentials.  Everything else is either unnecessary, or can be obtained there.


  • Change of clothing that's comfortable enough to sleep in but still passes for daytime attire.  Extra shirt, because you *will* be vomited on.
  • Toothbrush, deodorant, contact lens case with solution, hair scrunchie.  
  • iPad with kindle app, netflix app, facebook app.  Charger (doubles as phone charger)
There's really nothing more I need for a night at the hospital.  Clothes.  Bathroom necessities.  Entertainment.  There are other comforts that help when you're there for days - but at that point, someone is coming from home to see the little hamsteak anyway.  They can bring what you need.

Do you have a "just in case" bag that comes to hospital visits with you?  What's in it?

What kind of Thirty-One Consultant would I be if I didn't pack into a super cute bag??

Monday, September 10, 2012

What I wouldn't give for a button kit....

Behold, a Button Kit (this specific kit belongs to http://www.adventuresoftubegirl.com/).  This holds everything we need to change Aidan's button in the event that it needs to be replaced.  A new button, a button introducer, a catheter tip syringe, a slip tip syringe, a packet of lube, a continuous feeding set, a bolus feeding set, sterile gauze, and some helpful literature.



Behold, what we received from Interventional Radiology after surgery:



I guess I should be thankful we got the feeding sets?  And hope the balloon doesn't deflate, because we don't own the appropriate syringes needed for refilling?  And who needs that pesky "helpful literature" anyway?

Come on, IR.  I'm roughly one zillion percent sure you billed my insurance for the entire kit.  Is passing it along really too much to ask?

(Rhetorical question, obviously.  It's far too much to ask.  I asked anyway, and was told that they don't give out the syringes because they don't like to encourage parents to check the balloons too often.  Which is absurd.  Without the syringes, I am completely unable to care for this tube.)

Friday, September 7, 2012

Huevos y Flu Shots



Mama Bear's Message to Dr. Allergist: Hello, Dr. Allergist's Office.  I'd like to leave a message for Dr. Allergist.  My son has recently patch tested positive - very positive - for an egg allergy and I am now unsure of whether or not to allow him to receive a flu shot.  Can Dr. Allergist please give me a call so we can discuss?

Dr. Allergist's Nurse's Response via voicemail: I spoke with Dr. Allergist and he feels that it's okay for Aidan to receive a flu shot as long as he does not have anaphylaxis to egg - meaning any reaction when he ingests it.  So he said it's fine to get the flu shot, and it doesn't have to be done at our office.

Mama Bear's message back to Dr. Allergist's nurse: I'm calling in response to a voicemail I just received.  The message I left stated that we recently learned via Patch Testing that my son IS allergic to eggs.  I do not know what happens when he eats them, as he has not had them or any other food in nearly a year.  I'm sure you can understand that with a positive test result for egg, I am certainly not going to be feeding him eggs to find out for sure what his reaction will be.  I just need to know how to proceed regarding this flu shot.  Can you please have Dr. Allergist give me a call to discuss this?

Dr. Allergist's Nurse's response via voicemail: Dr. Allergist would like you to come in for a flu shot.  We can test him for egg at that time.


My brain?  Fried.  Please enlighten me.  Do some doctors just not use Patch?  Is the Patch reaction not considered a "real" reaction?  We were told that we needed to carry an Epi for ALL things that he reacted to, via Patch or SPT.  Dr. Allergist has notoriously useless nurses - maybe he's just not getting the message I'm attempting to send?

Wednesday, September 5, 2012

Another Day, Another Gastrostomy Tube

The most eagerly-awaited day in history.  Recent history, anyway.


The road to this day has been trying, but I'm so glad that we're here and I'm nervously anxious to see what's next for us.


April 15, 2012 - NG Tube



May 31, 2012 - PEG Tube



September 5, 2012 - Mini-One Button!




Tuesday, September 4, 2012

Patch Outcomes

So, I guess I didn't write this, but as I composed my thoughts on Patch Weekend, I was feeling optimistic.  Really optimistic.  Aidan was understandably annoyed at all the crap we taped to him, but he was acting okay and things seemed to be going well.  By Sunday, I actually convinced myself that we'd be passing things.  Okay, laugh all you want.  I (delusionally) thought he'd pass everything.  We'd have a plethora of things okayed for food trials.  Unicorns would be sneezing glitter onto rainbows.  The whole nine yards.

It didn't quite happen that way.

So here are the patches before we removed them.  Nothing sneaking out from under the discs.  No angry redness.  So stop your laughing, because this looked pretty hope-inspiring!

For the record, we tested milk, eggs, wheat, rice, corn, oat, barley, potato, beef, chicken, ham, turkey, green beans, carrots, peaches, and apples.

When I removed the patches, I immediately saw lots of redness (thanks to the adhesive), some stuck-on food (it smelled divine, as I'm sure you can imagine), and one welt.  One, we can handle.  I could see the outlines of the discs used to hold on the foods, but nothing that looked reaction-y other than the one.  It was egg, and it wasn't surprising.  We know from experience that Aidan loves eggs but can't eat them without immediate and severe vomiting.  We've considered them a probable allergen since he was 9 months old.  We even take egg-allergic precautions like having his flu shot done by the allergist instead of the Ped.

No biggie. 

We gave Aidan about half an hour to play while the re-drawn sharpie dried and then checked him out again.  By this time, more welts were popping up.  It's difficult to photograph an itchy, squirmy baby - but I'm a blogger so obviously I had to try.




I never did get fantastic pictures of some of the reactions. Rice and Oat were weaker reactions that were obvious in person but didn't photograph well.  Apple and Carrot look more like discolorations in the pictures than the welts they were in person. 

I actually worried that if we bathed him as instructed, the reactions might go away and no one would believe they'd ever been there.  Ha.  (For what it's worth, it's been over a week now, and egg and wheat are STILL plainly visible).

Monday saw us back to CHOP's Market Street annex to have the patches read.  Again, the outcome didn't photograph all that wonderfully, but the end results were fairly obvious in person.  Weak positives on rice, oats, apples, and carrots.  Positives on milk, wheat, corn, barley, potato, beef, green bean, and peach.  Strong positive on egg.  No reaction to chicken, ham, or turkey!!!

I wish this picture was clearer.  Trust me though.
So those are the results.  Lots of new allergies.  Reminder to carry epi-pens everywhere (because I so need to be reminded, ha!).  And it's time to start thinking about what and when we'll trial.

One final thought.  We've shared these results with a few people and realized that they might be a little confusing.  We've had a lot of reactions that sounded like "That's wonderful! Now he has three foods!"

To be clear - he doesn't.  He has zero foods.  We have three foods that have been approved for trial.  Each trial will happen on its own, over the course of 2-3 months, with a scope and biopsy in between to check for eosinophil counts in his esophagus and colon.  I'm not trying to be a downer, I'm just realistic.  Having three foods would (Will!) be wonderful, but we aren't there yet and don't know when we'll get there.  There are a number of things that go into the decision regarding when to trial - this deserves a post of its own - but please believe that we are just as anxious as you are to get him "safe" foods - safely.

Big huge thanks and hugs to Miss Nikki for coming to CHOP with us, and to Baby Aidan (Our Medical Me doll!) for keeping Aidan brave.  I look forward to sharing some pictures of Baby Aidan's first day with Big Boy Aidan soon!