Food Protein Induced Enterocolitis Syndrome: Diagnosis and Prognosis for FPIES

I always enjoy finding further updates, as well as medical and personal insights, written on FPIES.   The perspectives of this little known clinical diagnosis are always interesting to read when you have a child with FPIES.  View two well written articles here on Helium: Food Protein Induced EnterocolitisSyndrome: Diagnosis and Prognosis for FPIES.

The first, written by Dr.Nichole Evans is an informative synopsis of the the Up To Date article reviewed and updated in May 2011 on Food protein-induced proctitis/colitis, enteropathy, and enterocolitis of infancy and written by Dr. Alan M Lake, Associate Professor of Pediatrics Johns Hopkins University School of Medicine. 

Dr.Evans outlines the Up To Date article nicely.  "Food protein induced enterocolitis syndrome, also known as FPIES, is the most severe of all food protein-induced gastrointestinal diseases in infants".  She goes on to outline the diagnosis of FPIES, based on recognition of symptoms and by monitoring response to elimination of offending proteins as well as endoscopy to rule out other conditions that may cause similar symptoms in infants.  She concludes that "FPIES is an uncommon allergic disorder diagnosed by clinical features, endoscopic investigation and response to dietary management".   

The next, by Dr. Pandula Siribaddana, provides a summary of the diagnosis and prognosis of FPIES utilizing two comprehensive references:
1). In 2008: "Enterocolitis Syndrome: Consensus Recommendations for Diagnosis and Treatment"  written by the Japanese Research Group for Neonatal, Infantile, Allergic Disorders.   Which I also, personally, have found to be a useful resource of information. 
2). In 2009: Food Protein-Induced Enterocolitis Syndrome (FPIES) - a review from the UK Current Allergy & Clinical Immunology.  Which happens to be one of my personal top resource articles on FPIES. 

Dr. Siribaddana's article starts strong with "If ever there were a disease, which has been extensively under diagnosed and poorly managed, food protein induced enterocolitis syndrome (FPIES) would be right up in the list".  He goes on to highlight the "possibility of serious complications, if not managed properly" and that "it's pathological basis is a svere cell-mediated-hypersensitivity reaction (non-IgE mediated) within the gut mucosa".  His review provides the five-step process for diagnostic criteria set forth by the Japanese Research Group for Neonatal, Infantile, Allergic Disorders; which covers the symptoms, differential diagnosis, appropriate milk, body weight gain, and food challenges.

He concludes with a pivotal message to physicians, "although the paper was a consensus statement, it does not necessarily mean all affected infants and children should be managed in a similar manner.  However, following the guidelines would reduce the chances of missing FPIES, which would bring long-term benefits and improved prognosis for sufferers". 

I suspect my child has FPIES....

I suspect my infant/child has FPIES, now what? 

FPIES is a rare illness where little is known, many doctors do not recognize it.   If you have come across information on FPIES while searching for answers to your child’s extreme reactions to food and feeding intolerances, if you have read What is FPIES here and other sources (KFA, Baby Center, Facebook)- you have found information put together by FPIES families.   What can you do now?          

1.    Start a food journal, whether breast feeding or formula.  You will also want to strongly consider stopping all foods that are not previously assured as safe.  Often reactions happen with first food introductions, so this is not as difficult to do as if/when there is a larger diet in place. Following a reaction, the gut needs time to heal; any foods that are causing even small symptoms could impede this healing process.  It is safer for your child to stop foods and continue formula or breast milk only for ~2weeks (sometimes longer, rarely shorter). 

2.    If breastfeeding, consider an elimination diet.  Eliminating the food that the child reacted to initially, and if symptoms persist (or were developing prior to full blown reaction)- you may need to consider a Total Elimination Diet where the top 8 allergens are eliminated, as well as other FPIES allergenic foods such as corn, rice, oats.  If unable to continue breast feeding, consider elemental formula (will need Rx for from MD).

3.    If formula feeding, strong considerations for hypoallergenic and elemental formula’s. 

4.   It will be important to the health of your baby that other gastrointestinal disorders and allergies with similiar symptoms of feeding intolerances are ruled out.  This can be done with a Pediatric Allergist or GI.  Other conditions include Eosinophilic Gastrointestinal disorders and Celiac Disease.

5.   You will then need confirmation of FPIES diagnosis and treatment plans.  There is no guarantee that the doctors will know what to do, or have even heard of this diagnosis.  Although awareness is being raised, so it is helpful to call ahead of time and find a doctor that is familiar with treating FPIES.  If you are not able to locate a doctor who has treated FPIES patient, find one that is willing to help by learning.  Of course we would want doctors to have protocols already in place and know exactly what to do with food trials and reactions but there is not enough known yet for protocols to be in place in most hospitals; but that does not mean the doctors can’t help.  If they want to learn, they can help. 

6.    You may want to print off articles on FPIES, as well as 2010 Guidelines for Diagnosis and Management of Food Allergy in the US and Non-IgE Food Allergy to take with to your doctor’s appointments to ask them to review for considerations for differential diagnosis if your child is having these feeding intolerances. (also see my top FPIES articles on front page).

7.  Join an online support community, such as: Baby Center, KFA, or Facebook for additional resources and support.  Families sharing experiences can be a gold-mine of information and a lifeline of support.

Tests and Labs

NOTE: This post is under construction


Tests that may be done FPIES is suspected:

Upper Endoscopy

Colonoscopy/Sigmoidoscopy

Gastric Emptying Study

Motility study

X-ray

Upper GI study

IgE (RAST or SPT)

Stool studies

CBC

Tests that may help with identifying food sensitivies:

Atopy Patch Testing

What is FPIES?

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 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.