FPIES (F-pies) stands for Food Protein Induced Enterocolitis Syndrome. It is a Non-IgE Food Allergy. It can be described as a severe, delayed food allergy of the gut. Anaphylaxis of the gut is another way it has been described. It is understood to be a T-cell mediated response in which food is considered a foreign invader which the body attacks, like a virus, until it can violently expel it; although the exact mechanisms are still not well understood. For more information on IgE vs. Non-IgE Food Allergy read further here.
FPIES is a clinical diagnosis (based on history and symptoms), there is currently no test to confirm the diagnosis. Diagnostic tests are done to rule out other medical conditions that can present with similar feeding intolerances in infants and young children (such as Celiac Disease or Eosinophilic Gastrointestinal disorders). For more information on these tests, read further here.
It is very important that your child is evaluated by an MD if they are presenting with these feeding intolerances. FPIES is a rare syndrome and currently not well-known among family doctors, pediatricians and subspecialists, if your doctor is unfamiliar, read further here.
Symptoms are delayed following food ingestion (~2hrs or more) and include: profound/violent vomiting (often to bile), diarrhea and/or constipation. These symptoms, and the immunological response in the body, can quickly lead to: lethargy, low body temp or fever, hypotension, and in severe cases, sepsis and shock. For more on shock symptoms, read more here. Treatment of these symptoms is symptomatic and includes IV hydration. If your child is experiencing these symptoms, they should be evaluated in the ER. For a sample ER letter your doctor can provide for you to alert ER staff to your child’s needs with this rare illness, click here.
Some children experience these above “classic” symptoms only, while many other children also experience the many discomforting symptoms of protein intolerance while the body attacks the trigger protein (commonly referred to among FPIES parents as “build” symptoms). Build symptoms can be recognized in a persisting pattern and these can include: persistent hiccups, extreme stomach pains/cramps, “colic”, gas, runny or diarrhea stools with or without mucous or blood or undigested food, acid reflux, rashes/eczema, sleep disturbance, and agitation. Each child can experience their own range and intensity of symptoms. FPIES can be better treated with individualized treatments, specific to the child’s genetic makeup, environmental contributors, and trigger foods. For treatment plan goals, read further here. FPIES is thought to be a severe end of the spectrum of Protein Intolerance.
FPIES common triggers are: dairy, soy, rice, oats, barley, chicken, turkey, sweet potatoes, green beans, squash, corn, eggs. Trigger foods are not limited to these foods, as any food with protein can cause a reaction, even foods considered to be hypoallergenic. These are merely a list of common triggers found- every child is different and can develop intolerances to any foods with this illness. Some children have just 1-2 trigger proteins and some kids have multiple. Foods need to be trialed in an specific timed dose manner.
Once a food protein has triggered the FPIES response, it must be strictly avoided for 12-24mo. This differs from typical (IgE allergy) where avoidance is often lifetime. This is based on current thinking of this being a T-cell mediated response. T memory cells will regenerate (and forget the antigen) after strict avoidance. The emphasis is placed on strict because any trace/minute amount will trigger a launch of the Tcell mechanisms for this allergy, which will thereby increase the recognition and response mechanisms to the trigger protein; and can result in chronic inflammation. Strict adherence to avoidance of trigger proteins is cruicial for management.
FPIES common triggers are: dairy, soy, rice, oats, barley, chicken, turkey, sweet potatoes, green beans, squash, corn, eggs. Trigger foods are not limited to these foods, as any food with protein can cause a reaction, even foods considered to be hypoallergenic. These are merely a list of common triggers found- every child is different and can develop intolerances to any foods with this illness. Some children have just 1-2 trigger proteins and some kids have multiple. Foods need to be trialed in an specific timed dose manner.
Once a food protein has triggered the FPIES response, it must be strictly avoided for 12-24mo. This differs from typical (IgE allergy) where avoidance is often lifetime. This is based on current thinking of this being a T-cell mediated response. T memory cells will regenerate (and forget the antigen) after strict avoidance. The emphasis is placed on strict because any trace/minute amount will trigger a launch of the Tcell mechanisms for this allergy, which will thereby increase the recognition and response mechanisms to the trigger protein; and can result in chronic inflammation. Strict adherence to avoidance of trigger proteins is cruicial for management.
FPIES is a rare diagnosis, but you are not alone. There are communities online that are sharing information daily on new ventures, experiences, doctors, tests, food trials, symptom management, breast feeding and elimination diets, formulas,….These communities are largest in Baby Center FPIES group, Kids with Food Allergy (KFA), and Facebook. This diagnosis is not fully understood or recognized, but awareness grows daily. New research will bring additional answers to mechanisms, awareness to physicians, support for families, and protocols for treatment. To learn more upcoming research, read further here.
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