October 14th is Global FPIES day.
Why do I care?
Well it turns out this little guy has it. We will be too busy managing this rare disease to blog about it often, but I wanted to take a chance today to explain a little more about it for those who (like the me of two months ago) have never heard of it before.
FPIES (Food Protein Induced Enterocolitis Syndrome) is a reaction to the proteins in food that causes all sorts of gastro-type symptoms. In some cases, vomiting can be so severe that it leads to sudden dehydration and even sepsis-like shock. In other cases reactions cause chronic symptoms such as severe bloating, diarrhea, constipation, increased reflux symptoms, sleep disruptions, rashes and more.
Literally any food protein (including herbs, condiments, oils, etc) can cause a reaction and the hard part is that there are no ways to test for what a particular child will react to. Some children react through breastmilk and others tolerate breastmilk well but have trouble with formula and table food. Some are allergic to only one or two foods and others have many triggers. The only way to know what will cause a reaction is to try a food under carefully controlled circumstances in small amounts over many days. It can take anywhere from 7-21 days to be confident that a food is safe for a child with FPIES.
What does FPIES look like for Tomas?
When Tomas began eating solid foods over the summer, he began getting gradually sicker and sicker. His reflux was back with a vengeance and he was throwing up almost every day just like when we had tried to fortify his breastmilk with dairy based formula. I was so frustrated because I thought we had mastered the feeding, growing thing! It never seemed tied to foods until the night we fed him sweet potatoes for the first time. Over the next 2-3 hours, Tomas threw up six times and a lightbulb went on that it was the FOOD making him so sick. I stopped feeding him solids and within a couple days he was back to his normal, non refluxy and non puking self.
I called our GI who has some experience with FPIES and immediately recognized that it could be FPIES. There were other possibilities, however, and she wanted to rule those out first. There is no test for FPIES and she called it the "can of worms I'd rather not open".
Three months later now, I understand exactly what she meant.
At our GI's direction we changed the way we tried new foods starting with smaller amounts and taking a week or more on each new food. She told us to avoid specific common triggers and gave us a few ideas of foods we might find success with. She ordered a EGD to rule out another condition that could cause similar symptoms, but from the moment I looked up the two conditions I was 95% sure which Tomas had and the test eventually confirmed that he did not have EoE.
We started with pears, a food from Tomas' first few weeks that our GI thought was least likely to be causing his symptoms. He threw up again the same night, however. After a few days break, we tried carrots and other foods, all to no avail. Through all of his fails, however, we discovered a distinct pattern. Day 1- no reaction, Day 2- no reaction or mild increase in reflux, Day 3- Throw up (1-2 times) exactly 2 hours after eating the new food. I was starting to think we wouldn't find anything safe!
Around this time, I ended up with a bucket of fresh blueberries from a friend. I figured, why not try those and surprise! our first safe food. My mom delivered more blueberries from her garden and I started buying the big bags of organic blueberries from Costco practically every week. No matter how many blueberries Tomas ate, he never had an increase in reflux and never threw up.
We went back and forth with trying new things and failing more than we passed. Eventually we passed coconut milk/cream, trombone squash (another garden gift of those friends who knew of our struggles- a gift she has continued to pass on every single week since), beef, and rice (rice is a scary FPIES trigger for many kids, but in our search to find a dairy free probiotic, our chiropractor recommended one that used rice maltodextrin so we were brave and tried it).
In all of this a pattern became clear- always between the 2nd and 4th exposure, always 2-3 hours after eating. Once he started reacting to a food however, he would throw up the first time even after a break (most of these "trials" came with help of messy sibling eaters). His scariest reaction (so far) was quinoa. I laid him down for a nap and he puked several times with so much quantity that it soaked through the double thick flannel quilt I had been using as a spit up catcher to avoid changing his sheets every day during reflux flares, the sheet itself, the waterproof mattress pad, and was close to dripping from the back of the mattress. I'm glad it happened while I was still standing there and not a few moments later. He had a similar reaction to something while we were at the mall one day, vomiting so hard that he was sitting in puddles of puke with puke inside the tip of his pacifier. We still don't know what caused that reaction...likely some sort of cross contamination.
We are working with a dietician and an allergist (our second- the first didn't know much about FPIES) to find foods that Tomas can eat in a safe manner while still meeting his nutritional needs. His GI will continue to monitor his reflux, but we are hopeful that eventually he will begin to outgrow that as most kids do. On the spectrum of FPIES, Tomas has a relative high number of triggers but his reactions are considered more moderate than severe. Our new allergist, who is an FPIES specialist, says that he believes we will not see the scary vomit to shock reaction because kids tend to keep the same reaction to all of their triggers. It's of course possible and something to be aware of, but not something to be constantly worrying about.
Being experienced with FPIES, he also tailored our food trial approach to Tomas' reaction. Now we will only give new foods twice per week and never multiple days in a row until after a food appears completely safe. This will take 2-3 weeks as we have to wait until he has had several exposures before knowing for sure. Tomas has never reacted to a food for the first time later than day 4. As we get more foods into his diet, the allergist wants us to vary his meals as much as possible. Since the initial safe list, we have added white & red potatoes and pork. He also does well with apples from an FPIES standpoint, but they do cause an increase in reflux so we have to be careful with those.
As he approaches his first birthday next month, Tomas is unlikely to have a varied enough diet to receive all of his nutrition from food. This is a big bummer for me, but I'm trying not to be too disappointed. His allergist and dietician will work together to help us find the best option for continuing. The possibility of some whole foods smoothies with supplements exists, but right now he refuses to drink from any sort of cup which makes that impossible. If it's looking like a short term need for supplementation and he remains small in size, they may keep him on his current formula. Or, if it looks like longer term supplementation is needed we will look at one of the hypoallergenic toddler formulas tailored to older kids.
As our allergist put it, Tomas is our N-1...a reference my statistics loving husband appreciated. FPIES has such a range and variety that it is up to us to watch our individual subject (N1) and let him tell us what he needs and what is working for him.
Family gatherings, church events, etc are all going to be tough for the next few years. I'm only going to be able to trust food from a very limited number of sources. Tim and I have already realized that we are going to have to be each other's eyes in terms of checking labels as sometimes one of us will miss something. As Tomas grows he will hopefully outgrow some of his triggers (most? all? time will tell). Most children with FPIES are down to just a few triggers by the time they enter school.
Yet right now, we have no choice but to just deal with one day at a time.
Visit the FPIES foundation website to read more about this rare disease.