Wednesday, May 20, 2015

National Eosinophil Awareness Week: Greyden's Story

It was early morning, and we were headed to the hospital for the fifth time in the last seven months. Our sweet Greyden, just 2 ½ years old, asked where we were going, and we gingerly told him that we were going to see the doctor. He absorbed our answer for a short bit, and then, in his precious, innocent voice he asked, “Is the doctor going to fix me?”

I felt a bit of my heart break.

It’s been a long seven months in our house, ever since we discovered that our youngest son, Greyden, has various health issues with no clear cut answers. It’s been a trying time, full of life changes, confusion, anxieties, intense self-education (I now read PubMed in my spare time), monetary struggles, and learning to lean into our Lord and our support system of friends and family.

I now know, through various support groups online and through connecting with others, that there are many, many other families going through these same struggles with their own children. My heart hurts for all of us.

This week is Eosinophil Awareness Week. It seemed like the perfect time to join in spreading awareness and share our own personal story with Greyden.

Greyden has been diagnosed with esophageal eosinophils (ee-sof-uh-jee-uhl ee-uh-sin-uh-fils), meaning that his esophagus (the long tube that carries food from the mouth to the stomach) is inflamed and has many eosinophils in its tissues. The current estimated prevalence of this disorder is around 1 in 2000.

Eosinophils are white blood cells that are typically associated with allergies and are a normal part of certain body tissues. They are harmless and often helpful when they’re in places they’re supposed to be, and they’re generally bad when they’re in places where they’re not supposed to be. The esophagus, unfortunately, is not a place where they’re supposed to be.

In an overly basic nutshell, eosinophils are responsible for moving toward areas of the body where allergens (airborne or food-related) are sensed, and then once there they release toxins to fight the allergens. However, sometimes eosinophils show up where they shouldn’t be or show up in above-normal amounts, and in this case they can result in inflammation and damage to body tissue. This is what is happening in Greyden’s esophagus. The eosinophils are damaging his esophagus and causing it to become inflamed. An eosinophilic disorder of this type is not exclusive to the esophagus, and can actually also happen in the stomach, intestines, or any other body organ. The esophagus, however, seems to be the most common place for it to occur.

Common symptoms of esophageal eosinophilia in children are:

  • chest pain or heartburn
  • abdominal pain
  • poor appetite or refusal to eat
  • poor weight gain
  • failure to thrive
  • trouble sleeping
  • difficulty swallowing (dysphagia)
  • pain or discomfort while swallowing
  • food getting stuck in esophagus (food impaction, usually requires emergency surgery)
  • nausea
  • vomiting

Interestingly enough, Greyden does not show any of the typical outward symptoms of esophageal eosinophilia. He does not complain about pain or discomfort, he does not avoid foods, he does not vomit after eating, he is not suffering from failure to thrive or malnutrition. On the outside he appears to be your typical, healthy 2-year-old. He does have seemingly random vomiting episodes, which are actually what led us to discover that he had eosinophilic problems in the first place. However, most if not all of the doctors we’ve seen have believed his vomiting episodes to be a separate disorder, either cyclic vomiting syndrome or FPIES (which is a whole other blog post). Other symptoms he’s had, which may or may not have been related to the eosinophilic disorder, were unexplained chronic diarrhea and minor feeding issues, where he’d chew his food for up to 15 minutes and be unable to swallow it. Throughout the past several months, both of these issues have resolved.

In most cases, it appears that esophageal eosinophils appear as a delayed response to food allergens entering the body. However, prior to identifying an individual’s food allergens (if they are ever identified at all), there is a standard method of diagnosis that occurs in any patient with esophageal eosinophils:

Step 1: If there is the possibility that the eosinophils are caused by reflux (often referred to as GERD), then the patient is put on a trial of proton pump inhibitor (PPI) medications, which are basically drugs that shut down the stomach’s acid production and hence should eliminate reflux. Typically, patients with eosinophils due to reflux are those where the number of eosinophils counted per biopsy (biopsy of the esophagus collected during endoscopy) are below 15 and only occur in the lower esophagus. Since Greyden had eosinophils all the way up to the top of his esophagus, his eosinophil counts were 35 in the lower esophagus and 25 in the upper esophagus, and the eosinophils were not following the typical pattern seen with reflux, his GI doctor did not think this was the case for him.

Step 2: It has been discovered that there is a subset of patients with esophageal eosinophils, not apparently caused by reflux, who still respond to the PPIs. Research is still being done into understanding this disorder, but for some reason, the PPIs shut down the eosinophilic response and the patients heal. Therefore, the standard method of diagnosis requires that all patients with esophageal eosinophils go through a trial period of PPIs to see if they respond.  If they do, the continuing treatment for these patients, then, is to remain on PPIs long-term. These patients are diagnosed as having PPI-responsive esophageal eosinophilia, or PPI-REE.

Step 3: If a patient does not respond to the PPIs, then the patient is diagnosed with eosinophilic esophagitis, or EoE (sometimes seen as EE). The only current treatment for EoE is an elimination diet to find the food allergens and/or use of steroids.

So where does Greyden stand on this crazy diagnostic ladder?

That’s where things get tricky.

See, let’s back up for a minute, and discuss me. If you know me personally or have been around this blog recently, you know I have a slew of health issues, and most of them are autoimmune issues that stem back to food or digestive issues. Due to all the self-education I’ve been doing for myself, I know that there can be negative effects from long-term PPI use due to the resulting lack of acid in the stomach. This made me incredibly wary about putting my 2-year-old on PPIs. When I heard that we were expected to put him on a high-dose of PPIs (Step 2 above), I couldn’t help but feel the hairs bristle on my neck. Honestly, justified or not, it made me freak out inside. Panic. The effects of digestive issues were so raw and upfront in my own life, I couldn’t handle the thought of predisposing my child to them through medications. If medications were the only option, then yes, I would have used them. But I knew that eosinophils in the esophagus are often believed to be triggered by food allergies, and so I knew there was more than one path to try to heal him. And so I wanted to skip the PPIs and try the elimination diet (typically saved for Step 3) first.

Fortunately, my wonderful husband understood my concerns and fears and was willing to agree to my plan.  It was a rather complicated process with some purposeful and accidental decisions, but we skipped the PPI trial and went straight to diet changes. But this, unfortunately, means that Greyden does not currently have a clear diagnosis. We don’t know whether he has PPI-REE or EoE. I’ve been willing to accept this unclear diagnosis as long as we were allowed to try to heal him through diet prior to using medications. I’m not completely against medications, but I want them to be the last resort, not the first.

So rather than following the typical plan for eosinophilic esophageal disorders, we’ve followed the below path:

Step 1: Endoscopy #1: Find out Greyden has eosinophils in his esophagus. Lower count was 35, upper count was 25.

Step 2: Try an elimination diet recommended by our GI doctor: removal of gluten, soy, dairy, eggs, and beef. Eat this way for 2-3 months. Note that his vomiting episodes (supposedly unrelated to eosinophils) have seemed to stop, suggesting a possible issue with one of the foods that has been removed.

Step 3: Endoscopy #2. Discover that Greyden still has eosinophils in his esophagus. Lower count was 50, upper count was 20.

Step 4: Do some research, and decide to take Greyden to allergist who specializes in working with eosinophilic gastrointestinal disorders, nearly three hours away, to have allergy testing performed. Through skin patch testing, discover that Greyden has delayed allergic reactions to wheat, soy, milk, beef, chicken, almonds, peanuts, and green beans. Remove all additional foods from his diet, so he’s now avoiding gluten, soy, dairy, eggs, beef, chicken, peanuts, tree nuts, and green beans. Eat this way for 2-3 months. Experience one vomiting episode in the middle of this time period, which unfortunately means the cause for vomiting has not been completely resolved. After the vomiting, he sometimes begins saying during or after eating that he needs his bucket to throw up (but never actually does). On a positive note, however, his chronic diarrhea has stopped and he has normal stools for the first time in his life. His incessantly runny nose has stopped running. Also, he no longer chews foods for long periods or refuses to swallow them. We all feel hopeful.

Daddy found a recipe for plantain pizza crust that Grey could actually eat.

Step 5: Endoscopy #3, which was last week. Discover that Greyden’s number of eosinophils in his esophagus have nearly doubled. Lower count was >50 and upper count was >80. Cry and seclude self from the world for a couple days to adjust to news as a concerned mother. Pull self together, start reaching out to others again, talk to some doctors, and write this blog post. We’re taking him to see his allergist and dietitian this Friday, during which we’ll discuss potential paths moving forward. We’re also looking into finding an integrative pediatrician to take him to, who can help us navigate the world of diet versus medicine in dealing with this chronic health condition.

At this point, my concerns about the state of Greyden’s esophagus are now outweighing my concerns about PPI use, so I expect that we will probably be putting him on PPIs within the next week or so. The official decision will be made after we talk to a few more medical professionals, but we need to stop the current trajectory and get his little body to start healing. Whether or not PPIs will help with his healing, however, is completely up in the air; it is estimated that only around 30% of patients with esophageal eosinophils respond to PPIs.

And if the PPIs don’t work, which is apparently around 70% likely, where does that leave us?

In a mess, it seems. We don’t know.

At minimum, I believe we will have to remove more foods from his diet. He may need to take steroids to calm his inflammation. There is the possibility that he will end up on elemental formula, meaning he will have to drink all of his food in a special formula and will not be able to eat any solid foods. As difficult as this diet sounds, over 95% of patients heal when they follow this regimen. Then, one by one, they proceed to add foods back into their diet and see which ones they react to. It’s a long, tedious process, but sometimes it’s the only option.

Fortunately, he still shows few if any outward symptoms of his eosinophilic disorder, which I don’t understand. It seems that he should be in pain or discomfort. During his second endoscopy, his poor little esophagus was so swollen that the GI doctor could barely get the scope down his throat. But when we ask him if anything hurts, he always replies with a “no.” This is complete blessing at this point, and I am so grateful for it. We don’t have to deal with a child in daily pain and distress, as I know so many parents do. However, this symptomless freedom won’t last forever. His GI doctor said that if we don’t get the disorder under control soon, he is going to start hurting, and he assumes it will happen within the next six months to a year. This puts pressure on us to figure out a remedy soon.

An additional concern is the number of times we are putting our child under anesthesia. Each time we try a new diet or treatment with him, we have to put him under anesthesia and perform an endoscopy to check his esophagus and assess his progress. He has been put under four times in the last seven months, one of which was deeper anesthesia during which he had to be intubated because it was for surgery. (Yet another separate health issue, fortunately now resolved.) I have honestly not read the research about the implications of multiple episodes of anesthesia for young children, because at this point we don’t have a choice. I am aware that there are implications, but I don’t want to know about them right now. I’ve decided that the knowledge is not worth the anxiety it will cause. We are trying to spread out his endoscopies as much as we can, but at the same time we need to get him healed and assess his progress. Unfortunately, many times we’re being forced to choose between the lesser of two evils.

Oh, and if you haven’t noticed, I’ve mentioned a lot of doctors, specialists, hospital visits, and specialized diets in this post. If you see the dollar signs racking up, you are correct. This disorder is anything but cheap. Balancing expensive diets and medical bills has become one of our new fortes.

So that’s where we are for now. In summary:
  • His eosinophilic disorder continues to worsen at an unfortunately fast rate.
  • He interestingly enough still shows few, if any, symptoms of his eosinophilic disorder. He used to chew foods indefinitely and refuse to swallow, but this is no longer occurring. He occasionally tells us that he needs to throw up in the middle of or immediately after eating, but he never actually does.
  • He tested as having positive delayed allergic reactions to many foods, the worst of which were wheat, milk, and beef. Even though removal of all positive foods did not heal his esophagus, removal of them did lessen his number of vomiting episodes, allowed him to have normal stools for the first time in his life, and stopped his runny nose. What do we do with this knowledge moving forward? What do we do with his diet?
  • We will likely now need to try using PPIs to see if he responds to them in an attempt to find anything to stop the current trajectory.
  • Unsure if we will be doing further food eliminations. If his disorder is very possibly a reaction to food allergens, do we want to continue to pursue what those allergens might be?
If you know anyone around you with eosinophilic disorders or with family members who have an eosinophilic disorder, give them a hug this week. Especially those who are experiencing a lot of pain or other symptoms, or who are on huge food restrictions. I can’t attest to the pain that I know many people experience with this disorder, but I can speak to the difficulty of completely changing your child’s diet. And I’ve also completely changed my own diet for health issues, so I know what it’s like to go through personally as well. It’s hard. I have to send food with Greyden everywhere. At birthday parties, he has to watch the other kids eat cupcakes while he eats something I’ve brought for him. He can’t partake in pizza parties or have macaroni and cheese for a quick lunch. The foods he can eat are only available at various stores scattered throughout the area, so I oftentimes have to drive to four different stores to collect everything for him, and then the food is incredibly expensive. I have to cook all the time. We can never eat out, or at least we haven’t been brave enough to so far. If I forget to send a snack to school or church, that means he doesn’t get to eat and has to watch all the other kids munch down on food while he just drinks water. Halloween and Easter are not the fun candy free-for-alls that most kids experience. We have to constantly watch him, make sure he doesn’t touch allergenic foods, make sure other kids don’t reach into his safe food using contaminated hands. We have to instruct his teachers and caregivers on how to work with him, and tell them to wash all the kids’ hands if they do a craft using Cheerios or Fruit Loops, and to watch Greyden like a hawk to make sure nothing enters his mouth. He can’t play with play dough because it has wheat in it, and we have to check his body care products to make sure they don’t contain allergens that could end up in his mouth. Every time I drop him off with anyone besides me or Dave, I feel a bit of angst inside, wondering if the temporary caretaker will remember all the ins and outs of protecting him, because I know it’s a long, difficult list of dos and don’ts.

I actually created a printout to give to caregivers or people who want to serve Greyden food. Here it is below, in case you’d ever like to babysit. ;)

Greyden’s Foods
** Please read all labels for any items that come in a bag, box, or can (including canned veggies, lunchmeats, and even bagged frozen fruits and veggies).  You’d be surprised what they sneak into seemingly basic foods.  We have been instructed by the dietician to treat all of his “no” foods as if they were so dangerous that they would send him into anaphylactic shock. **
OK to eat
NOT OK to eat
  • turkey (turkey breast, turkey bacon, lunchmeat, ground turkey, turkey pepperoni, etc.)
  • pork (ham, bacon, ground pork, pork pepperoni, etc.)
  • fish
  • all fruits
  • potatoes
  • rice
  • quinoa
  • rice noodles
  • corn noodles
  • oats that are labeled gluten-free
  • seeds (sunflower, hemp, chia, flaxseed, etc.)
  • sunbutter (sunflower seed butter)
  • corn
  • popcorn
  • coconut
  • coconut oil
  • olive oil
  • “butter” spread that is free of dairy, soy, and all other items listed in the list to the right
  • Daiya “cheese” (dairy-free cheese replacement)
  • salt, pepper, spices, herbs
  • chicken
  • beef
  • venison
  • green beans
  • gluten (wheat, barley, malt, rye)
  • oats that are not labeled gluten free
  • soy (including edamame)
  • eggs
  • dairy (milk, cheese, yogurt, sour cream, butter, cream cheese, etc.)
  • peanuts
  • tree nuts (almonds, cashews, walnuts, etc.)
  • any item which has an ingredient list that includes any of the above
  • any item which has an ingredient list that has an allergy warning or says “may contain:...” for any of the above
  • any item which has an ingredient list that says underneath it, “Produced in a facility that also processes…” any of the above items

Yes, its complicated. It’s confusing. But ya know what, we’re doing it. And even if it hasn’t healed his esophagus so far, it’s fixed other health issues for him, and that brings me at least a small bit of satisfaction and hope as his mother. I’ll be curious to see what his allergist and dietitian say on Friday regarding how to move forward with his diet...nothing seems simple anymore.

I hope this blog post helps clear up some confusion that I know friends and family have over Greyden’s disorder. I also help that I’ve helped raise some awareness to the effects that eosinophilic disorders can have on people’s lives. There are many children and adults affected by these disorders, and they all have their own difficulties and struggles, whether they be similar to or very different from Greyden's.

We're going to get through this I know, but we still love and appreciate all support and prayers from friends and family.  Thank you to all of you. :)

God bless.


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