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!

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