"Due to the fact that FPIES is a rare yet serious condition, in the event of an emergency it is vital for the person to receive the appropriate treatment."
Food Protein-Induced Enterocolitis Syndrome or, 'FPIES,' is a non-IgE mediated reaction in a person's gastrointestinal system to one or more specific foods and is commonly characterized by profuse vomiting and diarrhea. 'Enterocolitis,' is inflammation involving both the colon and small intestine. FPIES is presumed to be cell mediated. Poor growth can happen with continual ingestion. After removing the problem food or foods, all FPIES symptoms a person experiences subside.
It is important to note that having FPIES does not preclude a person from experiencing additional allergies or intolerances with food. The most common FPIES triggers are dairy products and soy products. Any food; however, may cause an FPIES reaction, even ones not commonly considered to be allergens such as oats, rice, or barley.
A child with FPIES might experience what seems to be a severe stomach bug, yet the, 'bug,' only begins a couple of hours after the triggering food is consumed. A number of FPIES parents have rushed their child to the emergency room, limp from extreme and repeated projectile vomiting only to be told, 'it is the stomach flu.' The next time; however, they feed their children the same solids - the symptoms return.
'IgE,' stands for, 'Immunoglobulin E,' which is a type of antibody formed to protect a person's body from infection that functions in allergic reactions. IgE-mediated reactions are considered immediate hypersensitivity immune system reactions, while cell-mediated reactions are considered delayed hypersensitivity. Antibodies are not involved in cell-mediated reactions. Where understanding FPIES is concerned, you may disregard everything you know about IgE-mediated reactions.
FPIES reactions often times appear in the first weeks or months of a person's life, or at an older age for the exclusively breastfed child. Reactions commonly happen after introducing first solid foods such as infant formulas or cereals, which are usually made with dairy or soy products; infant formulas are considered to be solids for FPIES purposes. While a child might experience allergies and intolerances to food proteins they are exposed to through breast milk, FPIES reactions usually do not happen from breast milk, despite the mother's diet. An FPIES reaction commonly takes place when the child has directly ingested trigger foods.
Typical FPIES Reaction
Every person on Earth is very much an individual and the range, severity and duration of symptoms can vary from reaction to reaction. Unlike traditional IgE-mediated allergies, FPIES reactions do not manifest with hives, itching, swelling, wheezing or coughing. Symptoms usually only involve the person's gastrointestinal system and other body organs are not involved. FPIES reactions nearly always begin with delayed onset vomiting, usually 2 hours after ingestion, at times as late as 8 hours afterwards.
The person's symptoms may range from mild with an increase of reflux and several days of runny stools, to life threatening shock. In severe instances, after repeatedly vomiting, children often times start vomiting bile. Diarrhea usually follows and may last up to several days. In the worst reactions; approximately 20% of the time, a child has such severe vomiting and diarrhea that they quickly become seriously dehydrated and might go into shock.
Shock is a life threatening condition and may develop as the result of sudden injury, illness or bleeding. When a person's body is unable to get enough blood to vital organs, it goes into shock. The signs of shock may include the following:
Treating an FPIES Reaction
It is important to always follow a doctor's emergency plan related to your specific situation. Rapid dehydration and shock are medical emergencies. If your child is experiencing symptoms of FPIES or shock - immediately contact your local emergency services. If you are not sure if your child needs emergency services contact 9-1-1 or your doctor for guidance.
The most critical treatment during and FPIES reaction is intravenous fluids because of the risk and prevalence of dehydration. Children experiencing more severe symptoms might also need steroids and monitoring in the hospital. Mild reactions may be treated at home with oral electrolyte re-hydration or Pedialyte. Treatment of FPIES does not usually require epinephrine because epinephrine reverses IgE-mediated symptoms and FPIES is not IgE-mediated. Based upon the person's medical history, a doctor may prescribe epinephrine to reverse specific symptoms of shock such as low blood pressure.
FPIES is hard to diagnose unless the person's reaction has occurred more than once because it is diagnosed by symptom presentation. Usually, foods that trigger FPIES reactions are negative with standard skin and blood allergy tests such as SPT or RAST because they look for IgE-mediated responses. As mentioned - FPIES is not IgE-mediated.
'Atopy patch testing (APT),' is being studies for its effectiveness in diagnosing FPIES, as well as predicting if the problem food is no longer a trigger. The outcome of APT might determine if the person is a potential candidate for an oral food challenge (OFC). APT involves the placement of a trigger food in a metal cap, which is then left on the person's skin for a period of 48 hours. The person's skin is then watched for symptoms in the days following its removal.
Caring for a Child with FPIES
Treatment of FPIES varies depending on the person and their particular reactions. Many times, infants who have reacted to both dairy and soy formulas will be placed on hypoallergenic or elemental formula. Some children do well breastfeeding. Other children who have fewer triggers might strictly avoid the offending food or foods. New foods are usually introduced very slowly, one food at a time, over an extended period of time. Some doctors recommend trailing a single food for up to 3 weeks before introducing another one.
Due to the fact that FPIES is a rare yet serious condition, in the event of an emergency it is vital for the person to receive the appropriate treatment. Some doctors provide people with a letter containing a brief description of FPIES and its proper treatment. In the event of a reaction the letter may be taken with the child to the emergency room.
Because it's a rare, but serious condition, in the event of an emergency, it is vital to get the correct treatment. Some doctors provide their patients with a letter containing a brief description of FPIES and its proper treatment. In the event of a reaction, this letter can be taken to the ER with the child.
Many children outgrow FPIES by the time they reach the age of three. It is important to note; however, that the time varies by the individual and the offending food, so statistics are to be considered a guide and not absolute definitions. In one study, 100% of children with FPIES reactions to barley had outgrown and were tolerating barley by the age of three. Yet only 40% of those with FPIES to rice and 60% to dairy tolerated these foods by the same age.
Along with your child's doctor, you should determine if or when it is likely that your child may have outgrown any food triggers. Clearly, determining if a child has outgrown a trigger is something that needs to be evaluated on a food-by-food basis. APT testing might be an option to assess oral challenge readiness. Another factor for your child, you and your child's doctor to consider is if your child would physically have the ability to handle a potential failed food challenge.
When the time arrives to orally challenge an FPIES trigger, the majority of doctors familiar with FPIES will want to schedule an in-office food challenge. Some doctors, particularly those not practicing in a hospital clinic setting, might choose to challenge in the hospital with an I.V. already in place in the event of an emergency. Each doctor may have their own protocol, yet an FPIES trigger is something you should definitely not challenge without discussing it completely with your doctor.
Be aware that if a child passes the in-office part of the food challenge it does not mean a particular food is automatically guaranteed to be, 'safe.' If a child's delay in reaction is fairly short the child might fail an FPIES food challenge while they are still in the doctor's office or at the hospital. For children with longer reaction times it may not be until later in the day that they experience symptoms. Some might react up to 3 days later! Delay times can vary by the food trigger as well. If a child has FPIES to multiple foods 1 food may trigger symptoms within 4 hours. A different food might not trigger the child's symptoms until 6-8 hours after they have ingested the offending food product.
The FPIES Foundation
Food Protein-Induced Entercolitis Syndrome (FPIES)
What is Food Protein Induced Enterocolitis Syndrome
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