Eosinophil Hell

It’s been two days and we are still in shock.

To understand what I am about to share, you first need to understand what Eosinophils are, what they do and how they can help you or destroy you. This little diagram is a pretty good description.

When something harmful enters the body, your immune system responds and moves into action. However, for someone with an Auto-Immune Disorder, this process is broken. Instead of seeing a cold/flu as harmful, Annabelle’s immune system see’s chicken as dangerous and attacks – not just the chicken, but everything in her GI system that the chicken would have touched. Eosinophils should not be attacking anything in one’s esophagus, stomach, intestines etc – they should be waiting to fight other true dangers like the flu. Annabelle has a ton of Eosinophils in her esophagus, and that’s how we gain our diagnosis. Eosinophilic Esophaghitis (Eosinophils in the esophagus… it’s an EGID: Eosinophilic Gastrointestinal Disorder).

The problem with eosinophils in the esophagus, is based on what happens when they get there. The way eosinophils fight is to eat, eat, eat, destroy and destroy everything - finally they’ll release harmful toxins throughout the area to ensure everything is obliterated. Right now, the eosinophils are eating, attacking and destroying her esophagus. Let’s talk about painful?

When this happens, we call it an EoE Relapse – something has triggered her immune system to FIGHT and attack. Eosinophilic Esophaghitis is always there and she will always have the disease, but the goal is to keep it in remission at ALL times. The problem with EoE however, is that we don’t have a clue what that trigger is, there is no way to determine what actually sent her immune system into attack mode. It could be a multitude of things. If you can specifically pinpoint what made the relapse occur, then you remove that component, treat and calm the attack, and pray like hell that the damage done can be healed.

Understand EoE a little better now? Slightly? It’s ok. It’s taken me over a hear to speak the terminology correctly, and I study this stuff daily. I just wanted to share a little more to make it easier to understand the next curve ball that has just happened.

When Annabelle was diagnosed, her Eosinophil count was 40. That means, in a teeny tiny biopsy, under a small specific little area studied beneath a microscope, there were 40Eos/hpf in that one spot. Her worst area was at the bottom of her esophagus with 40. The middle of her esophagus was 25 and the top number was low. Immediately when those results came back, we removed every single food away from Annabelle, put her in a bubble, and flooded her with drugs and steroids as fast as we could. We prayed we could stop the damage and prayed harder she would heal. For months we kept Annabelle in a bubble until we repeated the biopsy and it showed the number of Eosinophils were dramatically lower and our treatment was working. For nearly 9mon we continued treatment until December. December was surgery day for several procedures, one of which came down to the realization that the bottom of her esophagus would never heal or work properly from the damage done from that first EoE attack. Therefore we made decisions towards a procedure that removed the damaged area and allowed us to rework Annabelle’s GI system a bit that she could still use most her esophagus – just with a little more help from the surgeons.

Tuesday afternoon our newest biopsy’s came in. And just as our GI told us, Annabelle is really sick, and in a lot of danger. This time the Eosinophils have flooded every part of her Esophagus from her throat to the stomach with an Eos count in the Upper 50s. This is so much worse than when we imagined. And much worse than when we were initially diagnosed.. the spread of damage, the sheer coverage of the eosinophils that have covered her and are eating and destroying my child is just unimaginable. How Annabelle is able to laugh, smile, eat and play just baffles me. It makes me prideful that she’s so strong, but at the same time I crumble knowing her strength comes from accepting this pain as a normal part of life. Without a doubt she is in terrible pain, she will occasionally complain, very rarely though – vomiting comes along with an EoE Relapse and she has been trying to vomit a lot more lately. But she pushes thru. She fights harder and tries to find more and more to laugh and smile about. Her strength is nothing less than heroic. But my baby shouldn’t have to live a life depending on heroic strength. She’s silently suffering and I didn’t know. I didn’t know she was so sick this time, and there is absolutely nothing that I can do to make all go away.

Please excuse the baby belle mucus/saliva on my sleeve and the filth! I blame the snow gloves and cluttered van on AK and the small people that ride in the back seat ;) This was Annabelle about a week before the scope/biopsy's. She has been trying to vomit a lot. The only good part about having a Nissen = she literally cannot vomit. Her stomach is sewn too tightly around the bottom portion of her esophagus to get anything up. So this is how she vomits. Right before I put her in the van this day, I did pull most everything off her tummy to make it a little more comfortable for her - but that's all the comfort I can provide.. we just watch her go thru this and pray it eventually stops.

 

Our immediate decisions were made between the GI and myself talking on the phone for quite some time. We discussed over and over everything in her diet and concluded that we just don’t think it’s Annabelle’s diet that could have done damage this quickly and this severe. She has been on small amounts of wheat but only for a short few weeks prior to the endoscopy – there’s no way the little amount of wheat could have escalated an attack like this. We are definitely still removing all wheat away, but leaving fruits and veggies because of fear if we remove too much of her diet - we will have a flood of other issues with liver / protein etc. Weve finally found a small, safe balance and we cannot jeopardize that yet. 

Between the two of us, we also made a preliminary conclusion that whatever has set this off, is likely airborne. That's right – it doesn’t only have to be food, it can be the water she touches, air she breaths, or even stress to bring on an attack. Based on the fact that the highest counts were at the top of her esophagus and visible damage in throat, we believe that it’s entering thru her mouth but not by food.

Controlling a food or something you ingest is 294610x easier to control of course. Fighting an invisible danger in the air?? …oh good grief! Can we catch a break?! PLEASE!

We had to sell our house and moved in with family since Annabelle’s last clean scope/look. The environment she lives in now is not the same as she did before. The seasons have changed and there is all sorts of crud in the air floating around. I asked our allergist/immunologist  “How am I supposed to do this? How am I supposed to prevent her from inhaling the air inside her house or outside? I just bought her first bike for goodness sake! She loves being outside! She is finally enjoying life and I now have to strip her from that and put her in a bubble. A sanitized, germ-free, allergen free, hypoallergenic bubble. Impossible. She made it pretty clear. 'Do nothing. Don’t fight because she’s loving life, and she’s going to lose that organ and everything else associated with it.' Her words were harsh, but truthful. If we allow Annabelle to continue to be exposed to whatever is initiating the immune system to fight, the eosinophils will eventually eat away at everything it’s attacking.

 

Our GI has indefinitely placed Annabelle on a liquid prednisone slurry, and instructed that I just begin to pray to move from twice x day to only once x day of the steroids. We have a mountain of other medications part of our EoE Action Plan and have begun the usual treatment. Only this time, the steroid dosage has been increased (prepare yourselves for my posts on Toddler-Baby-Roid-Rage.. it gets ugly.) and no more outside play. I have no idea how to begin tackling air inside a home beside a flood of air purifiers. I have no idea how to encourage and make a toddler keep a face mask over her sweet little face. We've had to do this before, but she was so sick during that time, she didn’t fight it. I just can’t imagine she will actually keep it on this time. We will work hard. She has to. We don’t have a choice any longer.

I picked up a few of the new prescriptions last night on the way home (thank you again, so very much from the bottom of my heart for Annabelle’s donations – it’s expenses like these that you make possible to afford and keep moving forward.). 

I called our hero in the doctor world and she and I talked for quite a while about what’s happened. We both have the same impression of EoE and have many suspicions about how her Immune System works and what else is really going on. Each and every time Annabelle would crash last year, it was usually in reaction to another change. Multiple systems are always affected when something begins to go wrong but always without explanation. Our big question is, “If there are a flood of eosinophils in her esophagus, and we know this because it’s the only place we’ve looked. How do we not know there are floods of eosinophils everywhere else within her body, attacking and destroying in all the wrong places? We don’t know what has set it off besides a really good, educated guess about airborns, but outside of that – how do we not know that her body is fighting other things and causing damage elsewhere too? Liver perhaps? That could explain a lot… a lot. She is making contacts now for us to see another specialist that has a lot of experience in Immunology. If nothing else, perhaps they can offer another perspective to the auto-immune world and why her body is doing the harmful things it is.

In conclusion, we are left with heavy, heavy broken hearts. Annabelle is very sick again. She is an absolutely crazy, silly, hilarious little monkey on the outside – but inside she is literally being destroyed with every minute. This is the shock and truth in the disease she’s fighting – this is the part where Eosinophilic Disorders become life changing to manage. We are grateful beyond belief to watch Annabelle’s energy and optimism, but do not mistake that as a healthy child. Our family is being stripped of heartache from the truth behind biopsy’s. Our little girl has a really really big battle to fight ahead. We have a lot of treatments to work thru, we have a flood of adjustments to lifestyles and we have even bigger prayers that a miracle can heal all the damage that’s been done already to her esophagus and we can safely manage this god-awful disease better moving forward. Next steps are to follow up with the new Immunologist, consult with our current allergist in Norfolk and schedule followup endoscopies/biopsys to keep an eye on our progress to slowing down this relapse and watch the treatment WORK!