I came across this article tonight and thought it was an easier explanation of FPIES to understant.
This is by Dr Frederick Leickly from Riley Hospital for Children in Indianpolis.
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.
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: 5.5 months (range 3-7 months)
■Age at resolution: 24 months (range 14-44 months)
The Milk/Soy FPIES profile was the following;
■Age at onset of the reaction: 1.0 months (range 2 days to 12 months)
■Age at resolution: 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.