FPIES- Food Protein-Induced Enterocolitis Syndrome
Last week I had the pleasure of meeting a young lady with infantile FPIES. Both of her parents were with her for the evaluation. The family alerted me to a contribution to the literature written by allergists for a condition that may not be commonly seen by an allergist. What I learned from that encounter has broadened my perspectives. FPIES or Food Protein-Induced Enterocolitis Syndrome is a clinical condition rarely seen in the allergy clinic. Thankfully it is a condition that is very uncommon. Based on how these children present, I would think that FPIES would be most often seen by our colleagues in pediatric gastroenterology. FPIES would not have been something that we could diagnose by a skin prick test (SPT) or by specific IgE in the blood. This is an immune reaction that is cell- mediated, not antibody mediated. IgE is not involved with the reaction. This cell-mediated reaction is more akin to how contact dermatitis or poison ivy affects susceptible people.Â
This young lady’s mother had with her an article that escaped my attention. The article was written by known experts in the field of Allergy (the lead author was Anna Nowak-Wegrzyn with Hugh Sampson, Robert Wood, and Scott Sicherer as contributing authors). The paper was a nice review of FPIES and a study of 14 special children. I think that any allergist who sees young children should review this paper. These young children can present with signs that are possibly consistent with anaphylaxis.
 The article was published in the journal Pediatrics in 2003. It is a review of 14 children who presented over a five year period at the Mount Sinai Pediatric Allergy and Immunology Clinic (New York, NY) and to the Allergy Clinic at Johns Hopkins Children’s Center (Baltimore, MD). The reactions that these children experience include severe diarrhea and vomiting which can lead to dehydration and shock. This is a clinical diagnosis; there are no specific laboratory tests that make the diagnosis. A food challenge can confirm the diagnosis.
Milk and soy have been the most commonly implicated foods causing FPIES. This article shows that other foods specifically solid foods have been shown to be associated with this syndrome; rice, oat, barley, peas, string beans, squash, sweet potato, chicken, and turkey. These children underwent food challenges to show the cause-effect relationship between the exposure and the symptoms. There were many combinations of foods causing the problem; cow’s milk alone, soy milk alone, both cow and soy milk, a single solid food, and more than one grain. The group was compared to children who were only milk/soy sensitive.
               The profile of the Solid Food FPIES population was as follows;
- Age at onset of the reaction:Â Â Â 5.5 months (range 3-7 months)
- Age at resolution:Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â 24 months (range 14-44 months)
               The Milk/Soy FPIES profile was the following;
- Age at onset of the reaction:Â Â 1.0 months (range 2 days to 12 months)
- Age at resolution:Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â Â 28 months (range 14-21 y)
This was the first published study of FPIES triggered by solid food. Oat was the most common food causing solid-food FPIES. The study also showed that breast-feeding may have a protective role in preventing/delaying the development of FPIES. The diagnosis of solid-food FPIES was not made until after two reactions. It was also noted that these reactions were severe. The delay in diagnosis was attributed to a number of possible factors; low incidence of the disorder, a presentation that looks like septic shock, and the belief that solid foods such as grains, vegetables, and poultry are of low allergenic potential. It was also noted that the time course of the reaction may delay making the correct diagnosis. The daily feeding of milk – cows and soy, leads to chronic problems. The re-introduction of the milk causes symptoms two hours after the exposure. As mentioned previously another problem is the lack of any test (other than avoidance and a food challenge) to confirm the diagnosis.
Another point that was made was that almost half of the children in this series had multiple food sensitivities. Children who were already on a casein hydrolysate formula had a median of four solid-foods that they were sensitive to.
No infant developed FPIES with exclusive breast feeding in this series. The authors pointed out that they were unaware of any reports of FPIES during breast feeding with absolutely no direct oral feeding of an offending food. No infant developed FPIES to milk/soy after age 1 years and the oldest child who had the solid-food FPIES was 7 months old. There were no ‘predictors’ of which child with milk/soy FPIES would go on to develop solid-food FPIES.
The Bottom Line-
The reaction of vomiting/diarrhea possibly leading to shock can be consistent with an IgE-mediated reaction and these are perhaps more common than FPIES. Such a reaction would lead to an allergy evaluation which will be negative if the diagnosis is FPIES. However, the infant is still at risk for a severe reaction with re-exposure.
Board certified allergists are credentialed in the care of allergic conditions in both pediatrics and internal medicine. Some of us went into allergy after completing training in pediatrics and others were trained in internal medicine. FPIES would not have been a clinical entity seen during internal medicine training. It may have been seen/talked about for a pediatric oriented allergist. FPIES favors infants. My point to all this is that although very rare, we need to keep this type of presentation in mind when seeing young infants with scary episodes of vomiting leading to shock with solid-food exposure. Their evaluation will show no evidence of allergic sensitization. We can help by teasing out the history of exposures and clinical course. We can offer recommendations for avoidance of the common foods that have triggered solid food-induced FPIES. This profile of young infants reacting in such a violent way needs to be considered in the evaluation especially if they have had issues with cow’s milk or soy milk.
This young lady made an impression on me. Her story was very scary. She caused me to go back to the literature and review what is known about her presentation.
Fred Leickly
February 17, 2010
· 
fleickly · 
11 Comments
Tags: Article Review, Food Allergy, Gastrointestinal Allergy, Unusual reactions to foods · Posted in: Food Allergies, Gastrointestinal Allergy, Interesting Stories
11 Responses
Thank you for your interest in FPIES. I wish there was more information available to doctors and parents. I have a 8 month old grandson with FPIES and the reactions are terrifying. If only there was testing to determine the offending foods. Although the condition is rare, the the numbers of infants with FPIES seems to be growing. My daughter has joined a internet FPIES group and moms of newly diagnosed children are added every few days (approx. 110 moms). All the moms are desperate for more help. I truly appreciate your post and awareness of FPIES.
Dear Dr. Leickly,
I am the the mother of an almost two-year-old with FPIES. Where we struggled to get to a diagnosis and in the process encountered several doctors, including specialists across various fields, who dismissed the severity of her symptoms and were unwilling to give her the FPIES label or even recognize the existence of the condition, your posting provides an interesting and refreshing perspective.
Thank you.
Hi Dr. Leickly! Thank you for taking the time to listen to this Mother and to further research FPIES for yourself or future patients. All of us parents of children with FPIES are trying to educate as many professionals as possible so that our children get the help that they deserve. Thanks again for having an open heart and mind.
If you would like to see a video of an FPIES reaction, I have a video that I took of my son reacting to soy. My son was sick for 12 months and I was determined to convince our pediatrician that something was wrong, so I took my son, some soy milk and my video camera and showed her what was happening. Here it is: http://www.youtube.com/watch?v=24nHsxopOyw
Thanks again.
Thank you,
Your note was the first of three on this topic. The reason for my delay in responding was that I was in New Orleans for the American Academy of Allergy, Asthma, and Immunology. I attended a session on FPIES. I plan to offer an update on this topic. It was a well-received talk. Among the attendees at the AAAAI conference, there was genuine concern and interest.
Warmest regards,
FEL
At the recent AAAAI meeting in New Orleans, Dr. Scott Sicherer gave an excellent presentation on this topic. There is concern and interest and FPIES needs to be part of the differential diagnosis for infants who present with this constellation of symptoms.
FEL
We (physicians) need to always be good listeners. And from my perspective, if I am not aware of a topic, I look into it so I can be conversant about it. I am preparing a talk for a conference for general pediatricians here in Indianapolis (May, 2010). The topic is allergic problems that ‘perplex/confuse/puzzle’ pediatricians. This is a vast topic and I plan to mention FPIES. I wonder if it is possible to show the tape at the conference(?). Is there a way to put it on a disc?
Thanks for the comments. As noted earlier, I plan to share what I learned about FPIES at the AAAAI meeting soon.
FEL
Thank you for your post. It’s given me valuable insight into the general lack of knowledge about this disorder among pediatricians and some allergists. My exclusively breastfed 7 month old experienced chronic diarrhea (I suspected a milk allergy) until I gave up dairy, and more recently, had two severe reactions to oat cereal and 1 moderate reaction to rice cereal. A recent diagnosis of “failure to thrive” has led us on a frustrating journey with our pediatrician to determine the cause – initially attributed to a stomach virus. After the second time he experienced frightening and severe vomiting, lethargy, and pallor within 2 hours of consuming oat cereal, I knew something more serious than a milk allergy or stomach virus was going on. I, too, came across the Nowak-Wegrzyn et al. article, and I suspect FPIES might be the culprit. We have an appointment in a few weeks with a pediatric allergist, and thanks to your post, I will not assume she is familiar with FPIES. I will definitely bring along additional information.
Very interesting. Our daughter presented at 8 weeks with colitis while exclusively breastfed. After an elimination diet, on my part, of chicken and rice, she continued to get worse… more and more blood in the stools and projectile vomiting. She was eventually put on Neocate which resolved all symptoms until foods were introduced around 7 months. Around 11 months old, rice was introduced and with it came a severe reaction. She vomited until she became lethargic and then an additional 45 minutes after that. As soon as the reaction is over she is her old self again. Her reactions come between one and two hours after eating rice. She is going to be 4 in a few weeks, and as of June 2009 she was still having reactions to rice. We have been very cautious over the last year and are now ready for a food trial, but all allergists seem scared to do it. I would love to hear your thoughts on her case. Is a food trial necessary at this point? Thank You!
I do not profess to be an expert in this area of FPIES. From what I see from those who have experience with these children is that it may take time for the problem to be outgrown. It would be great if there was a test to show that the sensitivity is still there, however with FPIES no test exists and the food challenge is the only way to know what is going on. Rice seems to be a food that could be avoided and one that is not essential for her growth and development. The older the child becomes, the easier it is to do a food challenge- they can communicate early symptoms so much easier. You need to decide on her risks and exposures and need for rice in regards to doing a challenge
Also, talk with your primary caretaker about resources and comfort levels in approaching a food challenge. In some instances the patient could be hospitalized for the challenge. Mount Sinai hospital in New York City has had significant experience in running food challenges for FPIES (at least according to their article).
Thank you for the question
FEL
Very interesting article. I posted it to my blog to inform others. We have struggled with my daughters FPIES diagnosis, mostly because of the insane lack of directions we seem to be receiving. Our latest visit to the GI at the U of MN was good, but she didn’t want to dx her as FPIES, she called it “multiple food allergies” instead. Our allergist dx’d her right away with FPIES but I don’t think he understands a whole lot becuase he’s given us no direction from there.
Are you aware of any physicians in the MN area that accept this as a diagnosis AND have the knowledge to treat it?
We are currently goign through an FPIES reaction to pears, otherwise it’s dairy, soy, oat and rice. Her only safe foods to date are corn and banana.
Thank you for the question. No, I am not aware of anyone in MN who works with this. As I have stated in my postings, my personal experience is limited to 1-2 patients and that is only over that past few months. My understanding comes from what I have read about the problem. My quest was to enhance awareness- it seems to have done this in some small way.
I think that there needs to be an interval of time to elapse with no symptoms and full avoidance. The culprit foods should be easily avoided. The food challenge helps to determine if the child has outgrown the reaction. Such a food challenge could be done through anyone who has this experience in a clinic- GI, Allergy, or even primary care. A consideration could be made for a hospital stay for the challenge in severe situations. The people at Mt. Sinai in New York, have experince with the challenges.
I hope this helps,
FEL
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