About FPIES

From The FPIES Foundation

About FPIES

Food Protein-Induced Enterocolitis Syndrome (FPIES) is a type of food allergy affecting the gastrointestinal (GI) tract. Classic symptoms of FPIES include profound vomiting, diarrhea, and dehydration. These symptoms can lead to severe lethargy, change in body temperature and blood pressure. Unlike typical food allergies, symptoms may not be immediate and do not show up on standard allergy tests. Furthermore, the negative allergy evaluation may delay the diagnosis and take the focus off the causative food. Nonetheless, FPIES can present with severe symptoms following ingestion of a food trigger.  Read more.....

An individualized treatment plan....

Earlier this week, I read an article released on Medscape as part of a report on Challenging Diagnosis in Allergy and GI.  It features this article: “Recent Advances in the Treatment of Eosinophilic Esophagitis” by Shauna Schroeder, Dan Atkins, and Glenn T. Furuta. ( http://www.medscape.com/viewarticle/731955?src=ptalk ).   The Abstract reads: “First described nearly 20 years ago, eosinophilic esophagitis (EoE) is an inflammatory disease of the esophagus characterized by eosinophilic infiltration of the esophageal epithelium. Over 50% of the current literature on EoE has been published in the last 3 years, signaling both a rising incidence and increased recognition of this disorder. Treatment options available for patients with EoE include dietary management and/or pharmacologic therapy. An individualized approach to treatment is preferred, with an emphasis on patient–parental preference. The objective of this article is to discuss the current and future treatment options for EoE.”

I read this article, and am encouraged by such advances in the understandings of EoE treatments.  My hope is to someday (soon) be reading an article such as this about FPIES.  There has also been increased literature publication on FPIES in the past 3 years.  As advances in EGID’s (Eosinophilic Gastrointestinal Disorders) emerge, it is promising for FPIES research; the more definition behind this gastrointestinal illness brings further delineations in what needs to be researched for definitions to other gastrointestinal disorders, such as FPIES, protein-induced enertopathy, protein-induced colitis and proctocolitis.  The spectrum of these protein induced gastrointestinal disorders will all benefit from these advances.   FPIES reactions themselves can be classic or chronic, there seems to be a spectrum within these for those affected by FPIES and it varies in each child.   FPIES mechanisms are still not fully understood. Fortunately, there is some understandings of the potential mechanisms with advances in cellular research; but one thing that remains a standard in FPIES treatment as it does in EGID’s treatments is “An individualized approach to treatment is preferred, with an emphasis on patient–parental preference.”

A Case of Severe Atypical Food Protein Induced Enterocolitis Syndrome

I recently read this article: A Case of Severe Atypical Food Protein Induced Enterocolitis Syndrome (abstract here).

It is a short article published in Allergy journal in August 2010 about a case of a severe reaction to cow's milk in a 4yr.old boy.  "The article highlights the potential severity of this condition and its capacity to present beyond infancy".

This 4yr.old boy, with a history of developmental delays had been receiving his extensively hydrolyzed cows milk formula via tube feedings since infancy.  He had ongoing eczema and a history of GERD; but otherwise had no other indications of allergy past infancy.   He was given unhydrolyzed cows milk formula in an overnight feed, within 2hours he was showing discomfort and began to have frequent watery stools (every 10-15min), which progressed to bloody stools within 2hrs.,so the feedings were stopped, and he was also having significant shortness of breath so was taken to the ER.

The article goes on to describe how he presented in the ER without fever (actually had low body temp), had low blood pressure and also acidotic and significant shortness of breath.  Further labs also revealed increased methaemoglobin, neutrophils and platelet levels.  He was treated for his symptoms of presumed septicemia and remained in the hospital; although extensive work up could not find a source for his symptoms.  Follow up endoscopy was performed 3 weeks following this episode and biopsy findings revealed gastritis and eosinophlic infiltrate in the colon.  He was resumed on his tube feedings with an elemental formula. 

The conclusion of the article highlights that FPIES is a "complex of vomiting, diarrhea, and systemic inflammatory response because of Non-IgE food allergy". The authors note that this case is outside of the published criteria for typical FPIES but that it illustrates the severity of this type of allergy and that it is possible to have an FPIES response beyond infancy.  The authors conclude the article with a very important message about FPIES, "FPIES is frequently misdiagnosed, and children often have several acute presentations before the diagnosis is established. In view of its potentially fatal clinical course and the recent increase in prevalence suggested by others, it is critical to consider the diagnosis in young children presenting with acute onset of gastrointestinal symptoms or shock".

FPIES: An Update on Natural History and Review of Management

Food Protein-Induced Enterocolitis Syndrome: An Update On Natural History and Review of Management

I love new articles on FPIES.   The above article was just published in August, and it is FULL of GREAT information.   It is a terrific synopsis of what has been studied thus far in FPIES, and how it is currently best managed.  Dr.Nowak-Wegrzyn has authored some great articles lately and it is exciting to see.   Most exciting to see is the table on page 2 giving descriptions for Chronic and Acute FPIES.   My son suffered from chronic FPIES which made his diagnosis more difficult to come to, as his acute reactions weren't happening as frequently as his chronic reactions.   It was a very muddy picture, it still is a muddy picture because his body reacts chronically as we seek to introduce foods- he "builds" to a reaction vs. having an acute shock reaction from the first 1/2tsp of food.  The acute shock reactions have been what has been studied over the past 20yrs. (rightly so due to their severity), now it appears groundwork is being laid to further study and define Chronic FPIES that so many other FPIES kids experience as well.

This is a great article, giving good descriptions for the history of FPIES and definitions of what is known about diagnosis and management of it.    It really is exciting to see so much being studied, and further recognized in the past year.  

An FPIES Toolbox

FPIES is a puzzle, it does not fit in a box.   The diagnosis can be complex, untreated symptoms leading to new symptoms...it can be chronic or classic.  No child seems to be the same- no triggers the same, response to treatments vary.  It is medically complex. 

Personally, I started my toolbox very empty, I began to fill it with: nuts and bolts (ie FPIES studies). Then came the nails that secures me in place with all the shared knowledge (ie FPIES families).  Then a few hammer's of varying sizes (ie to hammer in a point)- did you know kids can react to meat because of the feed that an animal eats? And that they can react to the proteins in mothers breastmilk? Then a few other tools to fill in the gaps:  Food Family Lists and Janice Vickerstaff guide to food introductions, as well as resources such as PIC and books like GAPS; all can fill in some holes here and there- used as tools to guide, taking bits and pieces of knowledge to apply to our puzzle.  Some glue has come along and proven to be quite effective already- ever use gorilla glue? (ie Probiotics).  Some screws were needed (diagnostic tests), not to test for FPIES but to rule out other related medical conditions (such as Eosinophilic Disorders and Celiac Disease.   A good variety of screwdrivers is very helpful to tighten up the seams (ie Allergist, GI, Dietitian, Pediatrician).  Every good household toolbox needs a flashlight, we found some light in research on Non-IgE food allergy to shed a good light on my thought process on individualization and the Immune and Gut involvement for the FPIES puzzle.  A good toolbox to keep all of this in is essential, FPIES kids don't fit in any square box but the corners of Functional Medicine makes a good bag to carry all of this in.   

I continue to look for new tools daily to fill my toolbox(bag) with, to help manage this chronic illness.   . If you need some tools for the journey you are on right now- there is an excellent toolbox filled already with some nuts and bolts (such as an ER letter, articles to bring to your pediatrician to help diagnosis, and food journals,...) here:       The FPIES Foundation

The FPIES Foundation

The FPIES Foundation is here!

"The FPIES Foundation is dedicated to overcoming the challenges of FPIES by offering tools for education, support, and advocacy to empower families and the medical community.... [It] is the collaborative effort of several FPIES families whose relentless journey has sparked the desire to help other families find their way. The FPIES Foundation strives to provide a credible and interactive support resource for this rare, oftentimes isolating diagnosis".

My entries on this blog have slowed down, in part due to my work as one of these families.  I am among the founding families of  The FPIES

The launch is official this week (refer to the press release on the website for details).  And, personally, I am so proud of what we have accomplished thus far, and we have so much more - so many more programs being developed to assist families and the medical community, to provide much needed support, and education.  We will be updating frequently, so check back often! 

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!