A Rare Food Allergy Doctors Know Little About

Med Watch: A rare food allergy doctors know little about.

Another FPIES family in the news.   The family's story is something that hits close to home for many FPIES families...."I googled baby vomits rice cereal"- wow, did we all do that?  Because who in the heck is allergic to rice?  FPIES kids are, it's bizarre but very real, and scary.   This mom's account of her daughter's reaction is an emotional account for any FPIES parent, as her daughter is one of the numbers of FPIES that went into full shock with the ingestion of rice.  The statistics show that ~20% of FPIES children do (although I would dispute that number to be much higher based on what is shared in support groups).  The interview is a window into an FPIES families world. 

The news report includes an interview from the family physician who helps manage their daughters care, with emphasis on the need for ruling out other conditions, and the symptoms caused from an FPIES reaction. 

Thank you to Ginny and her mom for a great awareness story on FPIES!

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". 

FPIES makes National News on Good Morning America!

Parents Limit Child to less than 10 foods is the title of the Good Morning America segment dedicated to raising awareness of FPIES through a family's story.  This family, also has started a research Fund, I have mentioned here before:  FPIES United Family Fund.   

The title is deceiving but eye catching and eye catching is awareness.  We need to continue to raise awareness, that from the outside looking in, despite how it looks from the outside- our children are sick and our families are struggling because of the lack of awareness and treatment plans. 

Take a moment to view the video, if you have not already watched it.   Good Morning America did a good job in covering several of the aspects that FPIES families share.

"In truth, question everything"....

This blog post on an allergy blog I follow: Grading the Severity of your Allergy: Sensitivity vs. Reaction Level, gives good advice on how to decipher your reaction levels, your severity.  What do you (or your child) react to?   Everything in the air?  Cross contamination?  Trace proteins?  Food families? Through breastmilk?  Through feed from the animal contaminating the meat?  Through formula?  On the skin?

With Non-IgE allergy, just as with an IgE Allergy, even the blood and skin tests will not tell the severity of an allergy; it will only tell the positive antibody production and the probability of reaction.   The only tried and true way to tell a person's reaction level is by testing it with elimination and challenge.  Eliminate the food from the diet, if symptoms subside, there are good chances you are allergic and need to eliminate that food, and all derivatives of it from the diet.  Some reaction symptoms are obvious enough you don't have to, nor would you want to, challenge the food.   A challenge would consist of a re-introduction of the food once suspected symptoms have resolved.  This challenge can also help determine severity of the allergy; as addressed in the above blog article.   Some people react to any and all minute derivatives or cross contamination of the allergen, some can handle derivatives or oils, etc.  The only true way to know is to challenge, and in the meantime- question everything.

Key Points from The Guidelines for the Diagnosis and Management of Food Allergy in the United States: Summary for Patients, Families, and Caregivers.

Guidelines for the Diagnosis and Management of Food Allergy in the United States:  Summary for Patients, Families, and Caregivers.

A publication by the National Institute of Allergy and Infectious Diseases

This publication was developed for patients, families and caregivers based from, The Guidelines for the Diagnosis and Management of Food Allergy in the United States: Report of the NIAID Sponsored Expert Panel, released in December, 2010 and written to provide medical professionals with the most up-to-date clinical advice on how to care for patients with food allergy.    A panel of experts developed the Guidelines based on recent scientific publications of food allergy.   An outline of this document was covered in a previous post here: New Guidelines for Diagnosis.

Key points from this publication specific to Non-IgE- mediated food allergy are:

Introduction:

·         The Guidelines include definitions of food allergy and related disorders, with recommendations for diagnosis and management.

Food Allergy Prevalence and Associated Disorders:

·         Defines Food Allergy “an adverse health effect arising from a specific immune response that occurs reproducibly on exposure to a given food.”

·         Definition of Food Allergen, “parts of a food or ingredients within a food (usually protein) that are recognized by immune cells”.  It goes on to clarify that most food allergens cause reactions even after they have been cooked or digested but that some allergens (most often fruits and vegetables) can cause allergic reactions only when eaten raw.  And oils (such as soy, corn, peanut, and sesame) may or may not cause an allergic reaction, depending on how it is processed.

·         Defines IgE and Non-IgE.  “Non-IgE mediated- the symptoms are the result of interaction of the allergen with the immune system, but the interaction does not involve an IgE antibody”.

·         Conditions that can occur with food allergy: “if someone has food allergy, he or she is more likely to have asthma, eczema, eosinophilic esophagitis (EoE), or exercise-induced anaphylaxis”.

Diagnosis of Food Allergy:

·         Food allergic reactions may be IgE-mediated or Non-IgE-mediated, or both.  The chapter, on page 9 gives some guidelines for when your healthcare provider should consider food allergy diagnosis.

·         Table A: Symptoms of Allergic reactions caused by food is on page 10.  You may notice similar language to the PIC article written for Non-IgE Food Allergy as The Guidelines are keys in our references for that information and table provided.

·         “How should your healthcare professional diagnosis Non-IgE-mediated food allergy?” is covered on page 16.  Examples given: Eosinophilic GI diseases (EGID)- clarifying that EoE is a mixed IgE/Non-IgE-mediated disorder; Food Protein Induced Enterocolitis Syndrome (FPIES); Food Protein-Induced Allergic Proctocolitis (AP); Allergic contact dermatitis (ACD); and Systemic contact dermatitis.  The coinciding guidelines, within The Guidelines, is given with brief descriptions of these disorders here.

·         Table C, on page 17, gives tests to diagnosis Non-IgE-mediated food allergy.

Management of Nonacute Allergic Reactions and Prevention of Food Allergy:

·         Avoidance of the food allergen following diagnosis of IgE, Non-IgE, or food allergy in combination with eczema, allergy, or EoE, is the safest way for management of food allergy at this time.

·         EoE, eczema, asthma may have influences not related to food allergy.

·         Children diagnosed with food allergy should receive nutritional counseling, growth monitoring, and training on understanding ingredient lists.

·         Know that there is a law, the U.S Food Allergen Labeling and Consumer Protection Act of 2004 that “requires food labels to list which of the eight major food allergens (milk, egg, peanut, tree nut, soy, wheat, fish, and crustacean shellfish) are present as ingredients in prepared foods.  However the law does not require or suggest wording for warning labels, such as “may contain trace amounts of nuts” or “may be prepared in a facility that also uses nuts”.  The inclusion of these warning labels is voluntary.

·         There are currently no medications available to prevent and treat allergic reactions caused by foods.   Oral immunotherapy is currently being studied.  There are active clinical trials: http://www.clinicaltrials.gov

·         Recommendations, including a helpful table, for vaccines and egg allergy on page 20-21.

·         Discussions on prevention of food allergy on pages 22-24.

Diagnosis and Management of Anaphylaxis Caused by Food

·         Symptoms and treatment of anaphylaxis (IgE) allergy, including a Sample Anaphylaxis Emergency Action Plan

Glossary

·         Listed on page 30 gives a glossary for the many terms used in this summary that may be new to the reader.

It should be noted that the Guidelines for medical professionals are “a resource to guide clinical practice and to help develop educational materials for patients, their families, caregivers and the public.  They do not override your doctor’s responsibility to make decisions appropriate to your circumstances”.

The Summary for Patients, Families and Caregivers can be viewed at the website below, and printed or you can request a copy to be mailed from NIAID.  I strongly suggest you read it and become empowered with the knowledge you need to manage food allergy for your family, in your doctor’s office, and in your home.   It is great progress to see these key points of Non-IgE allergy being recognized and discussed as important in the diagnosis of food allergy for our children.

The Guidelines can be viewed here: Guidelines for the Diagnosis and Management of Food Allergy in the United States

The Summary can be viewed here: Summary for Patients, Families and Caregivers

Food and Symptom Logs & Journals

Journals/logs are an important tool for the delayed reactions of Non-IgE Allergy.

Journals have been incredibly helpful.   The logging can be tedious but it is very reflective- which is incredibly helpful for seeing larger patterns to the chronic nature of FPIES.  It is particularly helpful, almost critical, to do them during food trials.  

Here are some examples of ones I have used:

Date:

12am

1am

2am

3am

4am

5am

6am

7am

8am

9am

10am

11am

Formula:

Probiotic:

Food:

Diapers:

Reactions:

Notes:

DAY	TIME	Food/Formula/Med	Reaction:	Notes:
	2am	8oz.
	3:30am	7oz.
	6:30am	4oz.
	8:30am

Date	Food Trial	Med/ Probiotic	Formula Total oz.	Calories	Reactions	Notes

FPIES: Video Tributes

FPIES is currently a clinical diagnosis based on symptoms.  There is currently no test for it.  The diagnosis has come a long way since this mother created this video: FPIES: Not your typical food allergy.  A video created by a mom desperate for help and answers to her sons violent reactions.  It is the graphic video of her son having a reaction.

Awareness has grown since this video was made, in large parts likely due to her putting the video on YouTube for others to learn and share.   I know it was educational for me and my family with our son's reactions because nothing could have captured it better than if we had recorded one ourselves.  Thankfully a challenge such as this, that would have been the diagnostic criteria a few years ago, is not needed with the increase in awareness and better understanding of this Non-IgE Allergy.   With an experienced MD, a history of symptoms and reactions is often sufficient for this clinical diagnosis.  Removal of the offending protein(s) with resolution of symptoms also confirms the diagnosis.    It is hopeful that, wth further research, there may one day be diagnostic tools available for this type of food allergy.

I was inspired by the knowledge gained from this mom's experience, I wanted to share our own FPIES story- in hopes that it too, could help another family along this road.   Where the above video captures an FPIES to shock reaction, our video highlights pictures of our son's first 2 years.  It outlines our struggles in our son's "atypical" FPIES responses.  It can be viewed here.