Our First Year with FPIES
Jackson (Jack) is a beautiful 15 month old boy who entered the world with a tenacious spirit after 7 months of an incredibly difficult pregnancy fraught with challenges. Jack is a twin; he has a fraternal twin brother named Sean. During pregnancy, hyperemesis gravidarum was our enemy, all day sickness like you wouldn’t believe. It took both Zofran and Reglan to stop the nausea, sort of. Bed rest began at 10 weeks of pregnancy with a 10 cm subchorionic hematoma, the bleeding stopped at week 19. Not fun. Jack had a high leak in his amniotic sac at week 20, they sent us home with a hope and a prayer and it sealed its self over, not common! Then the membrane ruptured for good at week 29. Off to the big city for a high risk delivery by ambulance. Jack and Sean stayed put until 32w 4d. Guess who stuck his foot out the exit door? (Literally!) Yep, Jack! Emergency C section and there were my beautiful baby boys. Jack excelled in the NICU, he was breathing room air in less than 12 hours. He was extubated after 8 hours. Sean needed a little more support; he was never stressed during the pregnancy, Jack had been the one who was distressed from go. One week in the NICU and then 3 weeks in a level 2 special care nursery and my Jackson was home. Sean was hospitalized another week. Hind sight is always 20/20 but when Jack projectile vomited infant acetaminophen in the nursery after a minor surgical procedure we were being warned of what was coming.
The boys were preemies and reflux and preemies go hand and hand. Both were put on Zantac at 6 weeks old. Sean’s symptoms improved, Jack’s did not. The volume of “spit up” from this kid was impressive. The boys never learned to latch to the breast as newborns because of a number of factors but they had expressed breast milk fortified with high calorie formula for 12 weeks. The more formula Jack got, the fussier he got and the more spit ups happened and we changed formulas twice. He was clearly in pain. The pediatrician passed this off as “colic.” Finally, we were beside ourselves and went to a gastroenterologist who was less than helpful. “Try Prilosec” was the result of that visit and it made things worse, back to the Zantac. In the middle of all of this with our new babies, my father died very suddenly when the twins were just 8 weeks old. We were emotionally overloaded and my gut said to me something is wrong with Jack, this amount of spit up is not normal, screaming and crying, horrible gas, loose poops, arching and clearly choking back stomach acid filled burps just wasn’t okay anymore. We had Sean to compare all of these behaviors to and he was just fine on the Zantac. One Sunday night I was totally overwhelmed with this screaming baby who was clearly in pain and I called the pediatricians office. I got a great nurse named Allison. Allison changed the course of our lives that night. I explained to her what was happening with Jack. She said, “Kate, this sounds just like my daughter when she was his age. I don’t think he can tolerate the corn in the baby formulas. I’m calling the local 24 hour pharmacy, go now and get Alimentum Ready to Feed in the bottles, NOT THE POWDER and get Dr. Brown’s bottles. It will take a few days but if it’s the corn he will get better on this formula.” Sure enough, in three days my baby boy emerged. He was happy and relaxed for the first time since he was born.
Then teething began, acetaminophen caused the same painful and spit up symptoms all over again. Then pureed foods were introduced, slowly. We accidentally gave Jack a baby food and the last ingredient was organic corn and bam, the nightmare was here. I have never seen a baby so sick. In 2 hours we had the exorcist baby (minus the projectile) the volume of vomit was incredible. He hadn’t eaten nearly the amount he was throwing up. It would stop and restart and by hour 4 after the corn he was a pale greyish color with dark black circles around his eyes (I know now these are allergy shiners), he was incredibly lethargic, pain filled and miserable. The mucous filled poops began at hour 10 post ingestion. Jack should have been hospitalized. We had no idea how serious what was happening to him was. He was on the brink of what we call in the FPIES community vomit to shock. Jack battled the corn in his system for 7 days. When I discussed this episode with the pediatrician she said we should make an appointment with the allergist.
Jack’s FPIES “bucket” had been tipped. We read every label on every food that we fed this child, literally sick to our stomach during each meal. We never wanted to see him like that again. Acetaminophen is filled with high fructose corn syrup and the docs all thought he would be fine as there is no corn protein in high fructose corn syrup – WRONG! This time we got a new symptom, “slapped cheeks.” He went from a pale greyish color with dark eyes (think Uncle Fester from The Addams Family) to pale with bright red, hot cheeks with allergy shiners and GI pain filled. We tried all different kinds of acetaminophen, (dye free) even had it compounded, just straight acetaminophen powder in a Kosher capsule. Fail.
We read that often Zantac is suspended in a substance made from corn – PANIC. We’re giving this baby corn twice a day unknowingly! We have it compounded, instructions say NO FILLERS CHILD ALLERGIC TO CORN, they promise us, no fillers. One dose of the Zantac, Uncle Fester reappears. We thought maybe he was teething, second dose and we had a screaming pain filled baby. I feel like we are poisoning our son. No more of this stuff. I called the pharmacy; it took me 3 days to get them to tell me that they added a flavoring that may have had dextrose in it. (CORN!!!) They corned my kid. You’re FIRED!
We meet with the allergist and she says let’s skin test him for corn, peaches and apricots. (Peaches and apricots left him with acid “burns” on his mouth and bum.) He’s negative for all three. The doc says well he looks like he’s sensitive to corn not anaphylactic so just don’t give it to him any until after he’s 12 months old. She leaves the room for about 5 minutes and returns. “Forget everything I just told you, I think Jack has FPIES, Food Protein Induced Enterocolitis Syndrome. No corn until he’s over 3 and we will trial it in the hospital where he can be monitored for his reaction. Corn is rare when it comes to FPIES but I really think this is what he has.” FPIES, this madness has a name. We go to the lab at her request for a blood draw. She says it will most likely return negative as FPIES will not show antibodies in the blood like a child who has an anaphylactic reaction to a particular food. She was right, negative.
The next 6 weeks were kind of a blur as we waited for the GI and allergist appointments in the big city. The local allergist felt that Jack’s level of FPIES was in need of more specialized care than she could provide and we were incredibly grateful for her honesty. So we kept away from acetaminophen using non medicinal measures to help soothe his teething pain and stayed away from any new foods. He ate only his 9 safe foods which were sweet potato, carrots, peas, prunes, winter squash, apples, blueberries, baby oatmeal and chicken. All of his food is Earth’s Best organic and GMO free baby food with the exception of the prunes and the chicken which are organic baby food from the local supermarkets Wild Harvest brand. I became and FPIES internet and FaceBook junkie, searching for information on this rare non mediated IgE food allergy.
The big day arrived and we traveled to the world renowned children’s hospital in the big city, with high hopes of help for Jack and emotional support for ourselves. Many people in the FPIES community told us we would love these doctors, we were excited. Very shortly into the appointment as we explained Jack’s reaction to corn it was clear these doctors were not for us. They said that FPIES to corn is incredibly rare and they he shouldn’t be reacting to any corn derivatives because there is no corn protein present. “Well I don’t know what to tell you Doctor, Jack has an acute or classic FPIES as well as a chronic reaction to corn, we are clear, and we live with him 24/7 as he’s going through this.” The allergist essentially scoffed at us and handed me a can of elemental formula and the first ingredient says, “57% corn solids,” REALLY? We agreed he’s never going to try this formula and that we are never returning to this clinic or these doctors, back to the drawing board.
We called another world renowned hospital in the big city and had heard wonderful things about two doctors there, one an allergist and the other a GI. We were incredibly cautious and skeptical. We were able to get an appointment with them in the pediatric food allergy clinic but the earliest we could get was 12 weeks away! While we waited Jack’s teething was out of control, he was in dire need of ibuprofen for pain management. Our pediatrician was uncomfortable prescribing anything and wanted the specialists to do it and we didn’t trust the allergist we had seen in the big city but had actually liked the GI. The GI was hesitant because they don’t write pain management scripts for compounding the pediatricians do. So we waited while they played pass the buck for a week until I had had it. I had done my own research and knew that a straight powdered ibuprofen could be packed filler free in a kosher capsule and we could then release if from the capsule into his food and give him a dose of a fever reducer/pain med. I called the pediatrician in tears and said she needed to write him a script for ibuprofen exactly this way but they needed to figure out the dosing based on his weight. It took another 24 hours but FINALLY the script was written and sent to a different compounding pharmacy then then one that corned him with the ranitidine. An anxiety filled ibuprofen trial that week he was teething resulted in a PASS! Finally we have our safe fever reducer/pain medication, whew!
About a month before the appointment we had the go ahead from the previous specialist team to try another food with Jack so we did pursue that. White potato was a PASS! Then we moved to another green vegetable and unfortunately the green beans were a massive fail. At this point we are also beginning to notice a trend with Jack. His acute reactions are lasting about 12-18 hours post ingestion but the lingering effects are moving into chronic reaction territory for days and days at a time. Jack was acting like he was in terrible GI pain for 2 weeks after this green bean fail. He had loose stool with mucous, pale greyish skin, slap cheeks, allergy shiners, and significant mood and behavior changes. Jack “disappears” when he is in this level of discomfort. He can no longer maintain eye contact or respond to his name. Just one week prior he was holding court with a room full of ladies from our early intervention program for his sixth month program review. He was smiling, being coy, and completing simple age appropriate tasks and now he can’t even look at me when I’m feeding him his bottle. Our hearts are breaking, he is so distressed and painful and his symptoms are mimicking early signs of autism. We wonder if he will ever come back. Thankfully after those two weeks our happy baby boy returns. We get brave again and trial Cheecha Puffs (a Canadian snack food made from potato starch and potato flour with a hint of salt, a pass for lots of FPIES to corn kids) and it’s a PASS! A crunch texture for Jack!
Just 10 days before Jack went to the big city for the appointments with the new allergist and gastroenterologist he had a seasonal flu shot, 4 days later he was completely gone. He was incredibly fussy, painful, allergy shiners, mucous in his stool to total constipation, spit-ups, slap cheeks and red flag autism symptoms, most noticeable, no eye contact. We have no idea if the flu shot triggered this reaction or if he had an accidental exposure to a corny food. We pulled the Cheecha Puffs, his last food pass out of desperation. He didn’t return for 34 days and essentially was miserable for he and his brothers’ first birthday. It was so sad, scary and discouraging. We had no idea what happened, what was happening and if we’d “see” our Jack again. Once whatever it was cleared his system he was back to his spunky old self, mostly. UPDATE: His eye contact isn’t as good as I’d like to see it and he doesn’t really respond to his name. He is now participating in “quirky” behaviors such as clicking and watching his double jointed thumbs and swinging small toys in front of his left eye to reorganize himself. These are ASD (Autism Spectrum Disorder) and or SPD (Sensory Processing Disorder) red flag symptoms. Some days Jack is connected, he is happy, babbling and seeking us out for comfort and to be picked up. Other days he appears too painful and or distant to even notice we are in the room with him.
The trip to the new docs was amazing! They listened, offered ideas, supported our ideas about what we thought was happening to Jack and believed us and agreed when we told them it was FPIES to corn. They took a comprehensive medical history and the allergist skin tested Jack for the top 8 allergens plus corn and 6 more. He didn’t want us to worry about anaphylaxis too while we are trialing new foods with him. The skin test is not 100% accurate and shifts over time (will need to be repeated in a year) but it’s a step in the right direction. It makes a HUGE difference in the world of FPIES to have a supportive medical team that has your back and your child’s back. We left with a plan to trial foods in the same families that Jack already had passes in. They handed us a paper the listed all kids of foods and both docs in the room helped us pick which ones to begin to trial. This visit was a complete 180 to our experience at the other clinic. We left this time with hope!
It’s been 8 weeks since we visited the new Food Allergy Center and we return this week for a check-in. Jack has since passed pink Himalayan salt and pure organic cane sugar. Today we re-introduced Cheecha Puffs original flavor, he just loves them and had a wonderful time munching on them this afternoon! Fingers crossed it’s a solid pass this time with no question. UPDATE: We had to pull the Cheecha Puffs again as he began to react to something. We will retrial as he had some water that was unsafe for him that could have been the culprit.
So our life with FPIES is fraught with anxiety, stress, sadness, anger, confusion and frustration. FPIES causes pain in our sons’ body and he can’t tell us what is happening yet. It’s slowed down his ability to meet some of his developmental milestones on time because he is so painful he cannot concentrate, play, or explore because he has to move inward to maintain himself. However though it all, Jack has shown he is a fighter with a tenacious, loving, sweet, spirit. The older he gets the stronger he becomes. He and his brother are our hearts. We would not be where we are as a family living with FPIES if it weren’t for some amazing friendships we have made along the way. (Hope for) Wyatt is Jack’s FPIES buddy and we live about 2,400 miles from one another. Using Skype with the boys will be fun someday soon! Jessie has been a God send at 2:30 am when I am in a panic because Jack has just spent an hour screaming bloody murder in horrific pain. The FPIES Foundation (http://www.thefpiesfoundation.org) has been our safety net and go to for support and education, Joy, Amanda, and Jenn, amazing! Then there is Cyndi, Penny, Jeniffer, Maureen and the list goes on. We aren’t alone, FINALLY we aren’t alone with FPIES and most importantly, Jack is not alone with FPIES. Everyday we celebrate the small victories and live as much as we can in the present moment.