National Eosinophil Awareness Week - Day in the Life

It’s National Eosinophil Awareness Week.  I haven’t really done anything, but I’m aware.  Boy, am I ever aware.

A lot of families I know posted challenges on Facebook – Eat like an EoE kid for a day!  They’re accompanied by lists of foods that have to be avoided by their children (common culprits like peanuts, soy, wheat, milk, eggs – and less common ones like apples, potato, beef.)  Others post lists of the meager and incongruous groupings of things their children can eat (tapioca, rice, turkey, blueberry) and urge their friends and family to step into these shoes, just for a day.  If you fail, donate $10 to Eosinophilic Disorder research.  If you don’t – think about living every day this way – maybe donate anyway.

I would never ask this of my friends or family.  I’d never wish it on my worst enemy (okay, maybe my worst enemy.)  As you know, if you know us at all, Aidan has no foods.  None.  Nothing.

If you ask him, he’ll happily tell you that his favorite snacks are chocolate, pepperoni pizza, and pretzels.  Those, he’s decided, are really socially appropriate things to say.  Sometimes he needs me to remind him what color chocolate is because he really doesn’t know.  Can you imagine?

While other families are drilling their preschoolers on stranger danger, Aidan is learning to be strong and assertive when someone tries to offer him a snack.  No, I have allergies!  No, I can’t have that!  Just. Say. No. 

So, okay, I won’t ask that you live a day in his life.  But close your eyes for a minute and think about it.  As your friends and family enjoy their snacks and meals, you can have a cup of water.  You can’t sit too close to anyone who might be eating – that wouldn’t be safe for you.  You can’t play with any toys or work on any crafts with food ingredients (sorry, Play Doh!) – not safe.  No soaps, lotions, or shampoos with “all natural” ingredients like fruit extracts or shea butter – might as well just say “all deadly.”  As you go through your day tomorrow, think about how different it might be if food – everything that is food or has food in it had to go. 

Isn’t it all the more impressive that this guy does it all with a smile?

CHOP Inpatient - Musings on Feeds

After Aidan's scary-yet-fun (?) ambulance ride to CHOP on Tuesday night, we settled into our room on 5 South and awaited a plan.  I swear, that's all we ever do here.  Wait for someone to figure out how to help him.  And in the end, it's always a patch up/send home job.

Aidan had no problem immediately falling asleep.  I, on the other hand, stayed up to panic about work and ponder what the appropriate hour was to text my boss.  Finally, I had to reach out and admit that I was here, with no access to my work laptop or phone, and would be missing several important meetings.

I hate this part.  Obviously my child is the most important thing in the world to me - but I pride myself on the work that I do, and not giving 100% does not sit well either.

Listless boy :(

Anyway - what Aidan really needed were IV fluids for hydration and gut rest.  Late in the day, we started up Pedialyte, and he finally produced wet diapers.  He really seemed to perk up quite a bit.

Toddler Problems: he's a righty.  no coloring :(

When we started up the 1/2 Pedialyte/1/2 Elecare mixture, he starting showing signs of a flushed face - but no fever.  Odd, and I actually asked several times that his temperature be rechecked because he felt warm to the touch - but no fever was recorded overnight. 

He was clingy and moaned a lot in his sleep.  But no fever, and no vomiting, so we advanced the feed to full Elecare.

We did get some sleep overnight, despite his discomfort, and when I woke up, I did my usual check of the room.  I always double check his feeds and rates and vitals - just to see how he's doing.  What I noticed made me instantly sick to my stomach.

Elecare Jr. Vanilla.  VANILLA.

Yup.  The doctor ordered the wrong formula.  The nurse hung the wrong formula.  They fed my baby the wrong formula.  Formula he's reacted to in the past.  This is not okay.

I feel guilty for being asleep at 6am when this was hung.  But I can't feel guilty for sleeping.  I can't feel guilty for their error, and their lack of quality review.

Aidan is okay.  He was uncomfortable and flushed in the face - but he is okay.  What if the mistake had been a dairy-based formula?  Or a soy-based formula?  What if he'd suffered anaphylaxis due to this mistake?  It's not okay, and CHOP needs to get it together.

The rest of the day was frustrating and disappointing.  The usual CHOP fare.  He's not pooping (surprise, surprise) so we're giving Miralax (no results).  He's feeling much better now that he's hydrated and is really ready to come home.

The main thing slowing things down is that I spoke to a GI, explained alllllll of our sordid formula history (the neocate drama - the G feed intolerance - the GJ tube change - the ensuing frequent hospitalizations).

It is my genuine belief at this point that he DOES NOT tolerate Elecare Jr.  When we give it by mouth or G-Tube, he gets rashes, vomiting, and GI pain (Screaming.  So much screaming.  Especially at night).  When we give it by J-Tube, because we have bypassed the stomach, the symptoms are different - but we get decreased motility (leading to constipation so severe, he ends up hospitalized for cleanout) and pain/discomfort.  He's not getting the rashes and vomiting - something about the chemistry of the jejunum vs. the chemistry of the stomach seems to produce a different set of symptoms.  But it seems clear to me.  Before we started Elecare Jr, do you know how many times he had these lower gut issues?  ZERO.  It's related.  It has to be related.  And I think it's the same, whether it's flavored or unflavored.  We never had these issues on the well-tolerated unflavored Neocate Jr or Neocate infant (but, interestingly, we did have the same set of symptoms on flavored Neocate Jr and Splash).

The GI I spoke to this morning seemed on board.  He said that clearly there was an allergic process in play, and that we needed to investigate the micronutrients in the formula to see what he was reacting to.  He even said he knew of a GI in the hospital today that he thought could help.  But he never came back.

Instead, he sent someone else - a resident, I think - to tell me that he was in the OR for the rest of the day, but thinks we should follow back up with our own GI in a month or so, and consider seeing an allergist.  Sound familiar?  It should.  It's what we're always told.  It's nobody's problem.  It's nobody's responsibility.  GI thinks Allergy should help.  Allergy refers us back to GI.  Everyone feels that things are going "generally well" for Aidan.  Everyone but Aidan, who's living in pain every day.  Aidan, who's dying to eat, but starving to live.

Someone has to help us.  Someone has to care.  I was hoping we would find someone today.  We didn't.

What to Expect When You're Scoping - May 15, 2013

I'm not sure if I've ever really posted about scopes.  We've done a few now, and I remember before the first one, I was so nervous.  I had no idea what to expect.  Aidan was not even a year old when we did his first scopes - and the thought of surgery for your infant is probably the most nerve-wracking thing ever. 

My disclaimer - this is how they go for us.  In our world, a scope is an endoscopy and a colonoscopy.  So the prep and the experience are specific to that.

My other disclaimer - Yes, I am aware that this scope took place two months ago.  I'm a busy mommy.  I meant to write about this sooner, but life happens.

Step 1: We visit our GI and agree that we're going to Scope.  Maybe it's because we've been trying a new food.  Maybe it's because we haven't changed anything but his symptoms have flared up.  Whatever the case, the scope is ordered and we are told that scheduling will give us a call.

This happens within a few days, which is exciting, but then they smack you with the news that the next opening is in something absurd like six weeks.

Maybe it would be sooner if we scoped down at Main Campus... I don't know...  But we always scope at Exton.  Going down to Main Campus is really a bigger adventure and we try to avoid it.  I think it tends to bring me flashbacks of inpatient stays.  Main Campus just *feels* different to me. 

Step 2: We wait.  And we try to change nothing.  And that's nowhere near as easy as it sounds.  If he accidentally eats something, the whole scope could be worthless.  If he gets sick, he may not be healthy enough come scope day.  OR, he may seem healthy enough, but they may refuse him anyway.  See Step 3.

Step 3: About two weeks before Scope Day, CHOP calls to discuss his medical history.  They ask a slew of very important questions, and it's really important to be truthful.  But be careful not to be too truthful.  Example - it's important to tell them that he's used his inhaler within the past six months - because this means he needs to use it in the days leading up to and on the morning of his surgery.  It's important not to volunteer that he has a runny nose today, two weeks before surgery, because they will threaten to cancel surgery.  I am not even joking.  Unless the kid is critically ill, keep your mouth shut.  They will examine him the morning of surgery and if he's too sick to proceed, they won't.  Cancelling two weeks out because he might be sick when the day comes around is just silly.

Step 4: We wait.  Notice a trend?  We wait, we continue trying to change nothing, and we hope against hope not to get sick.  This is where I get psychotic about people coming to visit us while sick, or sick-ish, and I tend to kind of hide out in hopes of keeping him healthy.

Step 5: Two or three days before the Scope, CHOP calls to go over his prep.  I'm not sure how unique our prep is because Aidan is not an eater, but here's what it looks like.

Day -2: Full Liquid Diet.  This means formula only, which is all he ever gets anyway.  No biggie.
Day -1: Clear Liquid Diet: There would be more options for eaters (like broth or jellow or sprite), but for Aidan this means just unflavored pedialyte, starting at 8am and continuing all day and night.  Thankfully, we can tube it in, because unflavored pedialyte is absolutely disgusting and there's no way on earth he'd drink that all day.
Day 0: NPO after 4am.  I programmed the pump so that the clear liquids from Day -1 ended at 4am.  I have a serious love/hate relationship with the pump, but this is one of the times that I love it.  A little bit of quick math lets me figure out how to stop feeds when I need them stopped, no waking up needed.

Alas, my smug slumber wouldn't last long, since we had to wake up at 5am to give him an enema.  I took no pictures.  You're welcome.  I'm not sure how this will work when he's out of diapers, but that's not my worry for today.  It's easy peasy lemon squeezy with a diapered kiddo.  By which I mean he writhes and screams and clenches things that I don't even know how to clench, and for the love of all things holy, why are we doing this at 5am!?

Proof that we left on time!

When we arrive at CHOP, we change into bright yellow CHOP jammies and I get the iPad out to keep Aidan entertained while we wait.  Surgeries happen by age, and he's still young enough to be very early in the morning.  Our arrival was scheduled for 6:45am.

When we are called back, Aidan gets a quick checkup to make sure he's healthy enough to scope.  Happily enough, he is.  Anesthesia comes to talk to us about their plan (for some reason, there was a mix-up and no one ordered pre-meds for him.  With Aidan's severe anxiety around hospitals, pre-meds are a must and I refused to proceed without them.  So there was a slight delay, but we did eventually get some Versed on board.).  The GI performing the scope also stops by to talk about symptoms and anything that may be new or different since we last saw him.  By now, Aidan's getting pretty loopy from the meds, plus it's ridiculous-o'clock, and we Shieldses sleep in.

I think he's a cute little druglet, don't you?

The scope takes something like 45 minutes - it would be shorter if it were just an upper or just a lower - but since it's both, and since they have anesthesia to worry about, I end up sitting there FOREVER while parents of ear tube patients come in and out of the room like there's a revolving door (seriously - ear tubes are like a twelve second procedure)

When the doctor finishes up, he comes out to see me and tells me how great it went.  He always has some pictures to show and he tells me how the scope went visually (our scopes are always visually beautiful, no matter how many eosinophils are present).

I do have a couple of cool pictures to share from this scope - it must be his first scope since getting the feeding tube, because we definitely don't have any pictures like this from past scopes!

This is the balloon that holds the button in place, right on the inside of his stomach wall.

This is the tube that goes down into the jejunum and feeds into Aidan's intestines.

As I'm gazing fondly at Aidan's jejunum, a nurse comes to fetch me and inform me that Aidan's ready to be seen and adored.  And when I get to him, someone's clearly already been adoring him and his puppy.  Puppy pup is wearing Aidan's beloved socks, and Aidan is all tucked in and high on life (and oxygen).

It is always very hard for Aidan to wake up.  I've heard that they wake up more easily without pre-meds, but for us, it's just not worth it.  I talk with the nurse for a bit while he snoozes - she goes over how the procedure went (fine), and what I should expect when we get home (really nothing special, he might be groggy but overall he'll be just fine, and he'll be ready to go to school tomorrow).  We turn off his oxygen, and it's time to wake him up.

I start by untucking him and starting to let in the dreaded cold air.  No effect.

I hold him, I tickle him, nothing works.  I finally have to strip him down and get him re-dressed.  He's awake.  And he's miserable.

How could you dooooooooooooo this to me?!

 His tummy hurts a little bit because they introduce some air during the scope - and as a family, we tend to be very emotional as a side effect of anesthesia - but he's really none the worse for wear.

In this particular case, he cried all the way home, at which point he and I both promptly fell asleep.  But when he woke up?  Good as new!

So really, that's it.  That's a scope.  The last piece is waiting another week or so for the GI's nurse to call with results.  That's always the longest week of everyone's lives - and I have to imagine it's even more nerve-wracking when you're waiting to find out if you get to keep all of your newest foods! 

I'd love to hear more about what scope days are like for you!

Diagnosis: EGID

Like any child, Aidan underwent lots of tests and doctor's visits to get to a diagnosis of his EGIDs.  An upper GI was performed, to check for anatomical issues and problems like Delayed Gastric Emptying.  That involved everyone waking up BRIGHT AND EARLY and making the trek to CHOP Exton.  At this point, Aidan knows that being allowed to leave the house in pajamas is not a great sign.  It usually means procedure day. 

I hate how tiny and scared he looks here.  He actually did really really well.  This was taken right before they gave him barium to drink.  There were 2oz in the bottle and we were told he needed to drink at least 1/2 an ounce.  My little champ drank all 2oz and cried for more.

Once he finished the barium, we had to hold him down (while he screamed at the top of his lungs, and YES, we do bring earplugs with us) for xrays.

Aidan's Upper GI was blessedly normal.  It showed his reflux (we know) but nothing else.  No deformities, no DGE, nothing else of any concern at all.  What a relief - but not an answer.




Next, we scheduled a scope.  A "scope" for Aidan is really an upper endoscopy and a colonoscopy, both with biopsies.  Dr. Liacouras sends a camera in to examine his esophagus, duodenum, stomach, and colon.  While he's in there, he takes small samples from several places in each area and sends them out to be examined under microscope.  A child can look totally normal visually (Aidan did) but still have terribly high numbers of eosinophils (again, Aidan did).

Aidan's scope was done on December 14, 2011, when he was 11 months old.  The procedure went very well.  He had a blast in the waiting room and charmed all of his nurses.  He's the bravest kid I've ever seen, really.

And you can't tell me you've ever seen a kid who looks cuter in CHOP jammies!

As we all know, Aidan's scopes were not normal.  They were what led to his diagnosis of Eosinophilic Esophagitis (25 eosinophils per high powered field) and Eosinophilic Colitis (100+ eos per hpf).


This was what tied all of the pieces together.  The pain, the screaming, the reactions to food, the failure to thrive, the refusal to eat.  It all makes sense in light of what we now know.

I am thankful that we've found doctors who knew what to look for and how to help him.  I'm excited to move forward.  There may be no cure (yet!) but we will make his life better!

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!

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?

Quick Update and Next Steps

A lot of you have been wondering what's happening right now with Aidan.  We have a few things scheduled, but for the most part, we're just working on figuring out how to do this thing called everyday life.

I've felt a little (a lot) too overwhelmed to blog lately.  I know, barely noticeable, right?  Not having a job has taken an emotional toll, and my time has been a little torn between being with my boy and finding the right professional fit.  I do want to go back to work, but I need to make careful choices for the right fit.

Anyway, coming up next for Aidan...

Patch Testing - Later this month, Aidan and I will go down to CHOP's EGID clinic to see one of the allergists for Patch Testing.  Patch Testing consists of placing small amounts of the suspected allergens on the skin and leaving them there over the weekend, to be read 48 hours later.  I have mixed feelings about this - I'm not sure what substances we're testing or what this will tell us about his EGID.  To some extent, I feel that this is a little bit pointless.  But most of me, most of the time, feels like more information can only help.  Even if this tells us nothing about his disorder and how it's presenting, we'll have more pieces of his allergy puzzle.  The hope is that this will guide us as we re-introduce foods in the coming months and years.

Surgery - This is (should be) a very minor one.  Aidan's feeding tube is currently a PEG and will need to be switched out for a Mini-One Button.  This requires day surgery down at CHOP in early September.  The thought of going back for another surgery makes me a little nauseous, but we're looking forward to getting rid of this PEG.

Once those things are done, I don't think we'll be messing with him any further this year.  He goes back to the GI in October, at which time we'll start discussing his next food trial, assuming his weight is still good and all else is going well with the G-Tube.

It feels good to be back to the blog.  I still struggle with feeling like I have nothing to say worth reading - but you've all given us so much love and support, the least I can do is let you know how Aidan's doing :)

Update: 4/24 Visit to CPED

Today, we had our second visit to CPED (Center for Pediatric Eosinophilic Disorders) downtown at CHOP.  We always leave the house early, and it's a good thing, because on this particular trip, it took us nearly two hours to get there.  Aidan was a happy passenger, and he got a little nap along the way, so it wasn't too bad.

When we arrived, we headed right up to the third floor, where Allergy and Nutrition hang out.  We were fifteen minutes early, but it didn't matter - Allergy was running 90 minutes behind.  After waiting for the better part of two hours, we were finally brought back to a room.

First, we did the ever-enjoyable (enter sarcasm here) weigh (20lbs - 2.2%!!!) and measure (28 inches - 0.2%).  Aidan wasn't exactly pleasant - I wish I knew why he loathes being weighed and measured so much - but once the scale was put away, he started to chat with me, play with toys, and explore the room.

Next, the Nutritionist came in.  We like Michele - and she rightly thinks Aidan is the most adorable kid ever.  We talked a lot about how things were going with the tube and where we go next.  She agrees with us that we are in the tube business for the long haul and that the G-Tube is the best solution for us.  We are struggling with the NG Tube, and given the road that's ahead of us, we don't feel it's necessary to do this for an extended period of time before starting the (different, but still present) struggle with the G-Tube.  So she will be sending her recommendations to the GI, and I'll be following up.

After we finished speaking with Michele, the Allergist came in.  We rehashed a lot of things we've already spoken about (No, he cannot have Neocate Nutra - he's allergic.  No, he cannot have flavored Neocate, he's allergic.  Yes, we've done SPT.  Yes, we're willing to do Patch.) and discussed our future plans.  She also agrees that the G-Tube is the way to go.

We will be going back to CPED to see Allergy and Nutrition for Patch Testing toward the end of the summer, in preparation for his next food challenge, which won't be until closer to his 2nd Birthday.  We are in no rush with food, and our focus right now is on getting him healthy, seeing him gain weight, and getting our lives on track with the tube.

Not a terribly exciting update, I'm sorry to say.  But he is doing well and we are moving forward :)

Fun Finds - "When Jeremy Jones' Stomach Stopped Working: A Story for Children with G-Tubes"

I stumbled upon a great website the other day - http://www.mitoaction.org/

Mito Action is meant to educate, support, and advocate for families dealing with Mitochondrial Disease (Mito). Mito is a genetic disorder where mitochondria of the cells fail to produce enough energy for cell and organ function. Mito can cause poor growth, weakness, neurological problems, delays, and diseases of nearly every organ in the body. The prognosis is really unclear - some people live fairly normal lives, while others are severely compromised.

There is so much more to learn than I could ever tell you - head on over to Mito Action to read more and to get involved.

What brought me to the page was a book that the organization puts out. It's called When Jeremy Jones' Stomach Stopped Working: A Story for Children with G-Tubes.

Jeremy's a fun, active kid.  He sings during class and catches tadpoles in the creek.  He also has gastric dysmotility, which means his stomach feels very full after only a few bites.  He gradually loses weight, and has less and less energy to do the things he loves doing.  Jeremy needs a G-Tube, and we learn more about them right alongside him.  Jeremy loves his new tube, and it helps him grow strong and tall.

Mito Action was kind enough to send a book to Aidan.  I am looking forward to reading it to him and using it to explain to and educate others about G-Tubes.

Update: April 16th - GI

This is a short update, because there's not too much to say.  It was a fairly uneventful visit with GI - we are always rushed in and out so quickly with very little chance to talk.

Dr. Liacouras again confirmed that the scope results were good (I really want a copy of that report), that we're doing the right thing, and that we now need to just sit back and watch him grow.  We're happy with the tube decision, though Dr. L feels that we should continue with the NG for another 2-3 months before considering the G-Tube.

We'll see about that... There's a call into his office to discuss that timetable...

The bright spot of the day?  We met up with a sweet little girl named Rylee in the waiting room.  Rylee is a Super Tubie too!  She was wearing a turtle sticker on her cheek, and for the first time since Aidan became a tubie, I was able to see myself on the other side of the table.  She didn't look sick.  I didn't feel sorry for her.  She looked beautiful and strong and healthy and brave.  I hope that's what someone, somewhere sees when they look at Aidan.

Update: April 4th Scope & Biopsies

A few weeks ago, Aidan had his second scope.  The first scope took place on December 14th and showed lots of eosinophils in his esophagus and colon.  We were hoping for better this time.  Aidan had been almost entirely elemental for the past seven months (minus one very very sad rice trial), so hopes were high.

The morning began with a very early wakeup.  I packed a sleepy, confused baby bug into the car, tucked him in with his blankets, and hoped that he fell back asleep (nope).  We arrived just as CHOP Exton opened - we were the first patients in the door that morning.  We were taken directly back to the pre-op waiting room, where Aidan changed into his CHOP jammies and we played.

As nerves began to set in (mine, not his!), I gave Aidan his beloved balloon (Ballooney!) and his beloved Tubie Friend (Toby!) and we waited to be called back.  We didn't have long to wait - Dr. Liacouras starts his day bright and early.

We soon found ourselves in the back, having vitals taken and being chatted up by the anesthesiologist.  We argued back and forth for a while about whether Aidan was well enough for the procedure - ultimately we agreed that he was - and were finally given the all-clear for the Versed.  Versed is the happy juice that children at CHOP get before a procedure.  It makes them a little silly, a little sleepy, and totally willing and happy to wave bye bye to mommy and be taken off to places unknown with a strange nurse.

Aidan went from happy to sleepy to zoned out to almost totally asleep within about ten minutes.

Around this time, the nurses told me that they were ready to take him back.  I was left in the pre-op waiting room, thinking about him and hoping that things went well.  He was in excellent hands, I know, but it's always a bit nerve-wracking to see your child put under anesthesia.

Less than thirty minutes later, Dr. Liacouras came out to let me know that he did wonderfully.  Things were visually "beautiful" and we'd have more results in about a week.

A few minutes later, I was brought back to see my sleeping beauty, who took his time waking up and didn't exactly come out of it smiling.  Waking up from surgery is always tough, isn't it?

I have so much love and affection to the nurses at Exton.  They treat us as though we're the only patient there that day (which is FAR from the case).  By the time Aidan left, he was all smiles and flirts.  Toby Tubie was feeling pretty good too :)

Several days later, we got the call from Dr. Liacouras.  Somehow, we've made the right decisions, because HIS SCOPE WAS NORMAL!!!  What this means for us is that now we know that he has the ability to feel good.  We have a good baseline for our future food trials.  This is really exciting news!!!

When will he get better?

Variations on this are probably the second most often asked questions (right after "Uh, so, what's wrong with him anyway?").  Will he ever get better?  When??  He'll outgrow this, right? Oh, is that all still going on?

The thing is, EGIDs are still "new" disorders.  They began to be diagnosed (sparingly) in the 1980's.  The incidence has increased rapidly since then, but it's still relatively rare and the long-term data just isn't there.  Further complicating things is the fact that what little research that is being done, is being done on Eosinophilic Esophagitis.  Not that this is a bad thing - it's the most common of the very rare EGIDs - but it's only part of the puzzle for Aidan, since his colon is affected as well (and is actually generally in worse shape than his esophagus.).

There have been several studies done to try to analyze the limited data that is available, and what seems clear from my "research" (Googling is research.  Shut up.) is that few patients have shown complete resolution of the disease.  Most patients show that the disease persists, but that some triggers may be outgrown.

The important piece to note is that there are no studies showing that EGIDs limit life expectancy.  Furthermore, although this is a frequent worry of many EGID parents, no good link exists between EGIDs and cancer of the esophagus.

There are some complications of EoE that we are aware of and will look out for.  EoE patients can develop strictures (narrowing of the esophagus), making swallowing difficult and often leading to food impaction.  There's not much information out there about complications of EC, but Aidan has a fantastic team of doctors working tirelessly to keep him well.


Soooo, to answer your questions:  Yes, he will get better.  If I told you when, I'd be making it up.  No, he probably won't outgrow this.  And yes, I'm sorry to say, this is still going on. 


What does all of this mean to us?

We anticipate that Aidan will develop a list of safe foods, and that it will grow over time after we get some successful food trials under our belt.  Food trials are a long, slow process, so please don't be shocked when you ask and we tell you that he's still not eating.

We are confident that Aidan will figure out what's going to be normal for him, and he'll lead a long, happy, non-food-centered life.

Don't you worry about me...  I'm going to be just fine :)

What's wrong with him, anyway?

Here's the zillion dollar question.  What's wrong with him?  


The short version:
Aidan has an eosinophilic gastrointestinal disorder (EGID) that involves his esophagus (Eosinophilic Esophagitis - abbreviated EE or EoE) and his colon (Eosinophilic Colitis - EC).  He also has environmental allergies, potentially life-threatening food allergies and Gastroesophageal reflux disease (GERD).  He also has Reactive Airway Disease.

Um...  What?
I know.  It's a lot.  It's a lot to read, and don't even get me started on living with it.   But we do (and "we" is a very broad word.  "We" are a team that includes Mommy and Daddy, friends, family, teachers, doctors, nutritionists, pharmacists, and online strangers whose kids have these problems too.) and we do it well.  See, I mentioned the EoE, the EC, the food allergies, the reactive airway, and the GERD.  But what I haven't mentioned yet is vastly more important.

Aidan has all of the issues above, but he also has a severely infectious laugh, an independent streak a mile wide, and a bigger smile than I've ever seen on a 1 year old, bar none.

Mommy wasn't kidding about that smile...

Let's take it one step at a time...


Food Allergies
Nowadays, most of us know someone with food allergies.  "Food allergies" can mean so many things.  Some reactions are scarier than others, but it's really important to know that a past reaction isn't a great indicator of a future one.  Yesterday's soy-related eczema flare-up could be tomorrow's anaphylaxis.  It's so, so important to be vigilant and careful, especially when dealing with small children who can't articulate how they feel.

In Aidan's case, the line between his food allergies and his EGIDs is a really hazy one.  We do, however, know that he is definitely allergic to peanuts, peas, and soy.  They are severe allergies with potentially life-threatening consequences.  We carry epi-pens for him, and are thrilled beyond thrilled to show and teach others about how to use them.  We carry a trainer pen everywhere with us and have given one to his daycare so that they can all practice using it.

There is also a list of "suspected" allergies that haven't shown positive on his last round of testing, either because we didn't test for it, it was a false negative, or he truly isn't allergic.  Those things include eggs, milk, and red dye (hello, random).  Further testing in the future should help us get to the bottom of these foods (but that's another post, for another time). 

Contact with these allergenic foods (even on his skin) can cause hives, swelling, red/itchy/watery eyes, itchy/runny/stuffy nose, itchy/swollen mouth, trouble speaking, trouble breathing, shortness of breath, rapid breathing, coughing, wheezing, vomiting, abdominal pain, nausea, diarrhea, agitation, weak pulse, anxiety, or loss of consciousness.  Obviously some of those look a little scarier than others (trouble breathing?  loss of consciousness?  Eek!) but for someone young and unable to advocate for himself, it can be hard to tell when he's really in trouble.  What looks like coughing can actually be a child struggling to breathe.  A child making "funny noises" may in fact be wheezing.  And how does a 1 year old indicate that his tongue is swelling or his mouth is itchy?

It's serious business, food allergies.  Not for the faint of heart.  And the very scariest thing?  Many people don't even know they have a food allergy until after a serious reaction occurs.

So if you hang out with us, be ready to be shown the business end of an epi-pen.  Be ready to use it.  If you have questions, ask them.  If you still don't understand, ask again!  This little guy counts on all of us to keep him safe.


GERD
This one's a lot more straightforward.  I actually know few babies that don't have some reflux in their first year of life.  Aidan's no exception here, and continues to be treated with a high dose of a Proton Pump Inhibitor (Prilosec) twice a day.  His medicine has to be compounded for him, we really wish our insurance would pay for this, but what can you do?  Any parent dealing with feeding issues would agree that whatever keeps food in the tummy is worth its weight in gold.


EGIDs
Ah, here we are.  This is the reason you came, I know.  The ever-mysterious Eosinophilic Disorders.  There will be many, many posts in the future explaining more about this, so I'll go with a short version for now.

Inside everyone's body are white blood cells called eosinophils.  They're super important because they help fight off nasty infections (including parasites).

In Aidan's body, the eosinophils are a bit confused.  They hang out in his esophagus and colon whenever he eats because they think food is a parasite.  They do what any good parasite-fighter would do - attack!  Unfortunately, there aren't any parasites around(I promise, we checked), and so they attack the food instead (and it's awfully hard to grow when everything you eat gets attacked before you can digest it!).  Once the food is gone, they attack the inside of his body (and this hurts, a lot.).

What this means for Aidan on a daily basis is that he can't eat.  Not that he can't eat some things.  He can't eat anything.  You know how helpful it is to distract your kid with cheerios, or bribe him with a cookie?  I don't. (But I hear it's grand.)

It's a full time job keeping food away from a toddler.  Just ask his teachers! 


Reactive Airway Disease
Reactive Airway is a way of describing how Aidan's body reacts at certain times.  It might or might not indicate asthma later on.  At the moment, it indicates that Aidan frequently coughs, wheezes, and struggles to catch his breath, especially when he's sick.  He is treated at home and at school with albuterol in a nebulizer, and he's surprisingly good about taking the treatment.

At this point, Aidan's too young for reliable diagnosis of asthma.  At his age, we call his collection of symptoms "Reactive Airway" and keep an eye on it.



So that's what's "wrong" with him.  It's my sincere hope that those of you who know him have just as much to say about what's "right" with him too.  He's a fantastic kid, he just needs a little bit of extra care to keep him safe and healthy.

There will be lots more to say about all of this in future posts, including how we got here and where we're going next, but this seemed a reasonable place to start.  Feel free to ask questions.  Thanks for listening.  Thanks for caring.