How Do I Know if My Child Has FPIES?
What is FPIES Syndrome?
Food Protein-Induced Enterocolitis Syndrome (FPIES) is a food allergy disorder that affects the gastrointestinal tract and sometimes causes severe reactions. It is an uncommon disorder that most often affects infants and young children and occurs following the ingestion of a food trigger. Because the symptoms of FPIES can mimic those of other disorders or illnesses, it is often misdiagnosed and can leave parents frustrated and unsure where to turn.
FPIES produces an abnormal immune response to a causative food trigger, resulting in GI inflammation. FPIES primarily affects young children, particularly during infancy with the introduction of food into the diet.
Diagnosis can be challenging since symptoms are often delayed and standard allergy tests are ineffective in reaching an FPIES diagnosis. Furthermore, symptoms of FPIES can mimic other medical conditions, and a negative allergy test can delay diagnosis, further confusing the relationship between symptoms and the causative food.
Since FPIES is a non-IgE food allergy, it cannot be diagnosed with traditional food allergy tests such as the skin prick test, or blood test that measures food IgE antibodies.
An FPIES diagnosis is usually made based on consideration of the history of the symptoms the child has experienced, and exclusion of other illnesses. A doctor, allergist or gastroenterologist may use additional tests such as blood tests that are taken during a reaction, or a medically-supervised oral food challenge. It is important to note that although an oral food challenge is one of the most definitive tests, it is not necessary for diagnosis and is often avoided due to the risk associated with an FPIES reaction. Atopy patch testing is sometimes used when FPIES is suspected but is not considered a valid test for an FPIES diagnosis.
Other illnesses that could present similar to FPIES include dehydration, food poisoning, local or systemic infection, metabolic diseases and GI disorders. It may require more than one episode before the causative food can be identified.
Common Symptoms of FPIES
While each child is unique and the type of symptoms and intensity of symptoms can vary, there are typically two ways that children who have FPIES present with symptoms when seeking medical attention.
Diagnosis can be challenging since numerous conditions can mimic symptoms of FPIES, including eosinophilic gastroenteritis, viral gastrointestinal illness, proctocolitis, food protein-induced enteropathy, sepsis, sucrase-isomaltase deficiency, various metabolic disorders and Celiac disease.
Traditional food allergies can be distinguished from FPIES by common symptoms including asthma and skin issues such as hives and rapid swelling of the skin. FPIES can also be initially mistaken for certain medical conditions involving the intestines.
First, an FPIES reaction can occur when an otherwise healthy infant or child develops symptoms shortly after drinking or eating. Acute FPIES often presents in infancy with repetitive vomiting approximately 1-4 hours after ingestion of the food trigger. In acute reactions, children often appear sleepy, pale, blue, and/or unresponsive, and may also have delayed diarrhea. Severe reactions may lead to dehydration, change in body temperature, and/or blood pressure.
Children who have acute FPIES can react to any food with common triggers being cow’s milk and soy. Many children also experience symptoms with introduction of solid foods, including grains such as rice and oats. Recent research also suggests that other common triggers can include, poultry, peas, green beans, sweet potatoes and squash.
Your child may appear ill and sleepy and may even become pale. A doctor can determine if your child has low blood pressure or dehydration, and they may also check for sepsis. Because of the similarity in symptoms, some infants who have FPIES may initially be suspected to have a severe infection or sepsis.
FPIES Symptoms usually resolve within about 24 hours of food ingestion and with proper nutrition management, these children often have normal growth.
Chronic FPIES occurs in infancy (usually < 4 months of age) and occurs when the food trigger is ingested on a regular basis, usually daily; such as infant formula. The second common FPIES reaction occurs when infants ingest a causative food on a regular basis. Children with chronic FPIES will often have an acute reaction if the food trigger is reintroduced into the diet after a period of avoiding the food. The most common triggers for chronic FPIES are cow’s milk or soy formula.
Chronic FPIES Symptoms typically resolve within a few days after removing the offending food from the diet. Common FPIES symptoms include chronic/intermittent vomiting, watery diarrhea, and poor growth.
How is FPIES Diagnosed?
FPIES can be difficult to diagnose because there are no specific procedures or tests for diagnosing FPIES. An accurate medical history and a description of the reaction are the most important tools for obtaining an accurate diagnosis.
When describing the reaction to your healthcare provider, it is important to include details such as all foods that were eaten and the time they were consumed, as well as the specific FPIES symptoms, when they occurred, and how often they occurred.
Traditional testing used for IgE-mediated food allergies, such as skin prick tests or blood tests are typically negative and may be used to rule out other types of food allergies and further clarify the type of reaction.
During an acute FPIES reaction, some lab values may be abnormal such as elevated white blood cell count, neutrophils, eosinophils, and platelets. In both acute and chronic cases, the child may also experience anemia (decreased red blood cell count) or low albumin (protein).
Management of FPIES
Strictly avoiding the causative food is the only treatment for both acute and chronic FPIES, but it requires vigilance in monitoring your child’s diet.
Since some FPIES reactions can be severe, it is critical to get prompt medical attention. The primary therapy is giving intravenous fluids to help stabilize blood pressure and treat dehydration, which can help avoid sepsis-like shock. Rehydration typically results in rapid improvement with complete resolution within two to three days. Steroids can also be used for the immune reaction.
It can be helpful to prepare a letter with FPIES information and a list of your child’s triggers to be taken with you to the emergency room in the event of a reaction. You can find helpful information on how to prepare a letter as well as guidance on emergency care for FPIES here
During a reaction, it is important to get medical treatment to stabilize blood pressure and treat dehydration. This may require giving fluids through an IV in a medical setting. Unlike a traditional food allergy, epinephrine is not helpful in treating an FPIES reaction. Going forward, it will be important to avoid any food triggers to prevent future reactions. Your healthcare team should provide guidance on how to stay safe while expanding your child’s diet to ensure adequate nutrition and development of good eating skills.
FPIES usually resolves over time, but your child will need to be followed closely by a healthcare professional to determine when FPIES has resolved and when it is safe to reintroduce foods into the diet. A supervised oral food challenge will often be recommended prior to introducing any food that previously caused an FPIES reaction. It is also recommended to meet with a registered dietitian to develop a plan for advancing the diet with safe foods, progressing with normal feeding skills, and ensuring adequate nutrition and growth.
Preparing for a Medical Appointment:
It can be helpful to share a detailed history of the reactions your child has experienced, including foods eaten and a timeline.
Questions a Healthcare Provider May Ask:
- What happened?
- What were the symptoms experienced by the child?
- What was the time course of the child’s symptoms?
- How old was the child at the time of the event(s)?
- How many times has this happened?
- Was it the same every time?
- What was the timing from the exposure to a suspected food to the onset of the child’s symptoms?
- What foods were involved?
- What was done to take care of the child?
- How long did it take for the child to recover?
- What advice were you given about handling future events?
- What tests have been done? Specifically, if your child had any labs checked at the time of the symptoms, can you get those results?
(Source: FPIES Foundation)
Keep a record with these details and bring it with you to your first appointment as this information can be critical in reaching a rapid diagnosis, identifying an appropriate course of treatment, and preparing for severe reactions in the future.
How Prevalent is FPIES Among the General Population?
FPIES is an uncommon disorder, although the incidence and prevalence is currently unknown. Over the last several decades, FPIES cases have been on the rise similar to many allergic disorders.
Several factors contribute to the lack of certainty on the prevalence of FPIES, including the variable nature of the disorder, lack of recognition within the medical community, and frequent misdiagnosis.
Is FPIES an Autoimmune Disease?
While researchers have not yet determined the immune system process in FPIES, they know that the disorder is non-IgE-mediated, which is different than traditional IgE-mediated food allergies. In fact, some researchers speculate that T cells may play a role in the development of FPIES-related inflammation in the intestinal tract but this has not yet been confirmed.
Furthermore, researchers have yet to identify genetic or environmental factors that play a role in FPIES. It may be interesting to note that a family history of atopic disease is present in about 40-80% of FPIES cases.
Can FPIES be Cured?
FPIES usually resolves over time, but your child will need to be followed closely by a healthcare professional to determine when FPIES has resolved and when it is safe to reintroduce previous food triggers into the diet.
A supervised oral food challenge will often be recommended prior to introducing any food that previously caused an FPIES reaction. It is also recommended to meet with a registered dietitian to develop a plan for advancing the diet with safe foods, progressing with normal feeding skills, and ensuring adequate nutrition and growth.
How an Amino Acid-Based Formula Can Help
Meeting the nutrition needs for children with FPIES can be a struggle, especially if there are significant food restrictions. Breastmilk or hypoallergenic formula is recommended for infants with FPIES and since FPIES does not typically resolve until the age of 3 years or later, many children may need a formula beyond infancy.
Amino acid-based formulas have many elements in common, but Essential Care Jr. is the only hypoallergenic amino acid formula for children over one year of age that is free of corn and artificial ingredients. Essential Care Jr. provides Low FODMAP tapioca as an alternative to corn syrup solids and uses natural monk fruit instead of artificial sweeteners.
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