Oh yeah, there's more...

In addition to all of our complicated medical issues, Stanton also has really low muscle tone in his face. This combination has complicated our efforts to obtain a firm diagnosis for Stanton and understand what the underlying issue really is. Is the low facial tone contributing to his inability to swallow or is the throat damage and eosinophilic responses he exhibits the smoking gun? I flip-fllp between these ideas throughout the day. Some days it’s really obvious and others, it’s cloudy. We are treating Stanton’s gastro-intestinal problems as if it’s eosinophilic esophagitis until we know otherwise. He really is responding well to this. My car still has a bag full of extra clothes for those unexpected, “just in case” puking in public moments, but, I haven’t needed them for a couple of weeks now. This is HUGE progress!

Stanton’s low facial tone has manifested itself in many forms. As a baby, I noticed his left eye crossing. The bigger he got, the more frequent his eye would turn in. By the time we saw an ophthalmologist, Stanton was 10 months old. The ophthalmologist quickly diagnosed Stanton with esotropia. Because of his age, the ophthalmologist said Stanton had a 95% chance of regaining his vision and scheduled surgery for the following week. Just six months post surgery, Stanton’s chart stated “normal vision.” We only have to go back to the ophthalmologist once a year!

Pre-surgery

"Eye Patching" Pre Surgery

Post Surgery

3-months post surgery

From day one, my little man has always presented with a protruding tongue. This means that his tongue sits wide and fat on his bottom lip. He hardly ever has his mouth closed and really never keeps his tongue in his mouth. When Stanton drinks his bottle, his tongue sticks out even as he sucks. The protruding tongue has really complicated the feeding issues as well as severely impacted his expressive speech. That combined with the trauma of feeding has lead to some sensory issues on his face and in his mouth. Washing his face and brushing his teeth are sometimes impossible!

He receives speech therapy and occupational therapy each week. The therapists are both focusing on oral-motor and working on transitioning Stanton from a bottle to a cup, tongue muscle strengthening exercises, lip closure, chewing and biting, increasing his communication and decreasing the sensory defensiveness around his face. Yes, between doctors, testing, specialists and therapy, we stay on the road a lot! I am beginning to notice that my life revolves around the whim of the “experts.”

What is an Eosinophilic Disorder?

What in the world is an eosinophilic gastro-intestinal disorder ? (this does take practice….but here’s how to pronounce it: “e-o-sin-o-feel-lic”)


I thought I’d take a moment to define the diagnosis Stanton has been given and is being treated for. Most of this information came from a wonderful site for Eosinophilic disorders. Thank you http://www.apfed.org/ for being in existence for parents like me! Of course, you’ll see that I’ve added in some information particular to our story….

Eosinophilic esophagitis (EE) is an allergic inflammatory disease characterized by elevated eosinophils in the esophagus. This is where it’s tricky for Stanton. He had an endoscopy in April to specifically look for eosinophils and none were found. Stanton began treatment for EE three weeks prior to the endoscopy so some of our specialists feel that this proves the treatment is working, while others on our team think it shows he may not have EE. So, for now, we are treating Stanton as if he has EE while still undergoing tests to rule out other things that could be going on. Stanton matches each and every symptom of EE and has responded well to the treatment!

Eosinophilic esophagitis is characterized by a large number of eosinophils and inflammation in the esophagus (the tube connecting the mouth to the stomach). Stanton had severe inflammation and damage before starting treatment for EE. Before, we treated him with a whole variety of reflux medications. He never showed progress while on acid blocking medications. There may be an inherited (genetic) tendency. (We have an appointment with a geneticist in July.) EE can be driven by food allergy or intolerance: most patients who eliminate food proteins from their diet (by drinking only an amino-acid based formula) improve. (Stanton switched to an amino-acid based formula, Elecare at the end of March.)

Common symptoms include (Stanton matches ALL of these):
o Reflux that does not respond to usual therapy (medicines which stop acid production in the stomach)
o Dysphagia (difficulty swallowing)
o Food impactions (food gets stuck in the esophagus)
o Nausea and Vomiting
o Failure to thrive (poor growth, malnutrition, or weight loss) and poor appetite
o Abdominal or chest pain
o Feeding refusal/intolerance or poor appetite
o Difficulty sleeping

At present, the only way to definitively diagnose EE is through endoscopy with biopsies. The endoscopy is often performed after treatment with reflux medications (acid suppressors) have failed to relieve the symptoms. During an upper endoscopy, the gastroenterologist looks at the esophagus, stomach, and duodenum (first part of the small bowel) through an endoscope (small tube inserted through the mouth) and takes multiple biopsies (small tissue samples) which a pathologist reviews under the microscope.

The gastroenterologist may be able to see a problem through the endoscope, but eosinophilic esophagitis may be present even if the esophagus looks normal to the doctor. That is why the biopsy samples are important to making the diagnosis of EE. A high number of eosinophils throughout the length of the (> 15 per high power field) suggest the diagnosis of EE. GERD also causes eosinophils in the esophagus, but typically far fewer and only in the part of the esophagus closest to the stomach. The pathologist will also look for tissue injury, inflammation, and thickening of the esophageal layers. With EE, the increased eosinophils are limited to the esophagus and not found in other areas of the intestinal tract. Once the diagnosis of EE is confirmed, food allergy testing is typically recommended to guide treatment. THIS IS EXACTLY WHERE WE ARE IN THIS PROCESS. Stanton is currently undergoing all the food allergy testing through a specialist at Ochsner’s.

Watch this video to see other families and children living with this diagnosis.  It sooooo describes our life and the stories sound so much like Stanton.

Raisins are a No Go

Oh Bother! Food just isn’t very fun once it finds its way into Stanton’s body! My Mom offered Stanton raisins on Monday. He wasn’t really too excited or interested, but my Mom sent the opened box home with us. I’ve had the box out at his eye level, so if he decided he might want to try one – he could. Today, that enticing little red raisin box caught his eye. Stanton ate 14 raisins throughout the morning. The blow-out diapers began after raisin number 5. He continued to have diaper issues all day. Then tonight, the grouchy, clingy, crying began and was quickly followed by vomiting.


Please don’t tell me that “now you can mark that off your list”. Or that I’m “one step closer to finding something he can eat.” Coming back to the drawing board isn’t really a good place to be. It’s as if we’re traveling over-seas with no translator or map and have lost our passports…confusing, exhausting and frustrating. I just pray that those sweet little raisins move on out soon. I don’t want them to get comfortable and decide to stick around and invite pneumonia over for a party.

Our ENT Rocks

We just returned from our post-scope follow up visit with our new ENT at Ochsner’s in New Orleans. In April, Stanton had a bronchoscopy in combination with our last esophageal and upper GI scope. Why did we end up needing a bronchoscopy? In January, Stanton had tons of constant congestion and 2 episodes of pneumonia. So, in February, after battling our ex-ENT to please just look at Stanton’ s throat, our pediatrician ordered a soft tissue x-ray of his face and neck. We were thinking his adenoids might be inflamed and/or large and may need to come out, however, the x-ray showed no issues with his adenoids or tonsils. Instead, we were alarmed to find that Stanton displayed a severe narrowing of his airway. Once our pediatrician saw how small Stanton’s airway was, we were sent directly to an ENT/airway specialist at Ochsner’s and the ENT, in turn, scheduled this bronchoscopy. This procedure allows the ENT to look at the airway system. Through the bronchoscope, they evaluated Stanton’s respiratory system, including his voice box, wind pipe, and airways for any abnormality or infection.

We learned they day of the scopes that his airway had returned to normal size and no abnormalities or infections were found. This was great news! But – I had to wonder, what does this mean?

The visit was a really good one! The ENT completely congratulated us for being aggressive and starting the elemental diet before the scope (switching Stanton to the special new formula, Elecare). He credits this with Stanton’s airway healing. No food = no reaction.

I brought my gag/vomit/poop/food journal along to the visit so I could show the doctor our one attempt at introducing food/episode since the clear scope. We introduced eggs and that was a complete nightmare! Stanton, of course, loved the scrambled egg and ate almost half of the egg! John and I were amazed, as we watched a child who shows no interest in food or eating, actually seem to enjoy the egg. Well, two hours after eating the delicious eggs, the screaming, retching, gagging began and was quickly followed by uncontrollable vomiting. Poor Stanton vomited profusely for 3 full days and nights. He aspirated while vomiting and we ended up with fluid in the lungs, swollen throat, etc., etc. Not a fun way to spend the week – steroids, breathing treatments, etc. He ended up with this rash on his face, which our pediatrician said was from vomiting so much.

The ENT said the egg episode clearly verifies Stanton’s diagnosis of Eosinophilic Esophagitis (EE) and we should wait 6 weeks before introducing another food. Ugh. The waiting is the worst! Then again, the trauma that spews after trying food is pretty awful too.

The ENT said he’s amazed that Stanton is maintaining his weight and looks so healthy! We hear that with every new doctor we meet. They see his extensive records and notes and surgeries and expect to see a pale, lethargic child. I suppose having severe EE, feeding aversions and swallowing issues are all serious issues that could indeed produce a unhealthy looking little one. We work so hard at keeping Stanton healthy. This is no easy task….but we’ve made it our mission.