FPIES Trends and Insights from the Patient Registry

FPIES Trends and Insights: 
Caregiver/Patient Reported Data from the Global Patient Registry 
By Hilary Lagerwey 

For four years now we have been working on collecting data from FPIES patients across the world using our Global Patient Registry from Patient Crossroads, a leading registry platform among rare diagnosis.

First of all, we want to say a big thank you to those who have already filled out the survey with valuable information!  There are several good studies out there describing what FPIES looks like, and our goal is to supplement that research with up to date information gathered from an even larger pool of families that we are able to reach using this tool. We hope that this information proves immediately valuable to families and doctors who are managing this diagnosis on a daily basis. We also hope to provide some insights to researchers that might prompt further study.

Anyone who fills out the survey has access to all of the cumulative data so feel free to browse and analyze for yourself, but today, in honor of Global FPIES Day this month (Oct.14th!) we thought we would share some of the insights we’ve gleaned so far. We recognize that since we are collecting data by voluntary responses some of our information may look skewed as compared to a more random sample; however we know that these results are important to share and that they represent a significant portion of FPIES families.

If you haven’t filled out the survey yet, please do so! The more data we have the more we can learn. The more information we can give to doctors and researchers, the more we can advance research to benefit all families living with FPIES.  Here is a look at what our data says about some frequently asked questions:

Do FPIES symptoms typically appear with the introduction of solid foods and formula or is it possible to react through breast milk?

57.4% of survey respondents reported exclusive breastfeeding when symptoms began.

Is FPIES typically outgrown in early childhood?

Out of patients 3 or older in our survey data, 15.8% report outgrowing acute reactions. 1 patient reported is under 3 and reported already outgrowing acute reactions. Several patients well into their elementary school years state that they still have not outgrown acute reactions.

*Also out of patients 3 or older, 15.8% report outgrowing chronic reactions.

50% of those who report outgrowing acute reactions report still experiencing GI symptoms with previously reactive foods.

Do most kids only react to one or a few foods?

Out of those who report projectile vomiting with reactions, 51.9% stated this occurs with only 1 or 2 triggers vs. 48.1% who stated they experience projectile vomiting with more than 2 triggers.

26.9 report projectile vomiting with 5 or more triggers.

What symptoms are common with reactions?

81.3% report projectile vomiting

65.6% report diarrhea

48.4% report visible and/or occult blood with stool

Thank you to FPIES Mom and FPIES Foundation volunteer, Hilary Lagerwey for compiling this survey data. 

Graphic created by FPIES Mom and FPIES Foundation volunteer, Melissa Rice 

A conversation with Prof. Stefano Miceli Sopo on the importance of a Global FPIES Patient Registry

Prof. Stefano Miceli Sopo works out of the Department of Pediatrics at Catholic University of the Sacred Heart in Rome, Italy. He has co-authored several studies on Food Protein Induced Enterocolitis Syndrome including:

• “Clinical management of food protein-induced enterocolitis syndrome”
•  "FPIES, From Practice to Theory"
• “Food protein-induced enterocolitis syndrome (FPIES) and well cooked foods: A working hypothesis” 
• “Ondansetron for Food Protein-Induced Enterocolitis Syndrome”
• “Chronic Food Protein-Induced Enterocolitis Syndrome Caused by Cow's Milk Proteins Passed through Breast Milk”

Prof. Stefano Miceli Sopo and his colleagues are passionate about FPIES research and the need for a Global FPIES Patient Registry. We recently had the opportunity to speak with Prof. Miceli Sopo who works with a center in Rome that follows and studies FPIES children.

Prof. Miceli Sopo says the most surprising thing for him is that there is still very little research being done on FPIES.  He says even most of the studies that are being done seem to offer only descriptions of case studies and maybe some guidelines.  He says, "We read little about the search for new treatments, to better understand its pathogenesis."  

Prof. Miceli Sopo says one of the most interesting recent aspects of FPIES research has to do with the promising effects of Ondansetron, during the acute phase of FPIES.  Ondansetron is an anti-vomiting medication, typically used to help chemotherapy patients.  He says in twenty of his case studies Ondansetron was administered and seemed to work well.

Prof. Miceli Sopo has made it a goal to create an FPIES registry in Italy and says there is a great need for a Global Registry, like the FPIES Foundation Global Registry.  He says, "It would be a really good thing" to have a central platform that was functional and affordable to provide information to pediatric allergy centers around the world.  He says a global registry would help doctors get on the same page when it comes to diagnosing FPIES.  Prof. Miceli Sopo says, "I believe there may be some differences.  We believe that we need a greater unanimity of behaviors.  The registry will serve to highlight the differences; we will try to eliminate them."

Prof. Miceli Sopo says hopefully a registry can also shed some light of what appears to be a spectrum of FPIES patients who suffer from varying degrees of the condition.  He says, "Just through the registry maybe we can see if there is a phenotype that tolerates small amounts of food, maybe processed in some way, and those who vomit after eating only a few milligrams of the food culprit."

Research of course takes time so Prof. Miceli Sopo says until we have more answers patients should keep an open dialogue with their doctors.  He says if there is any suspicion of FPIES he suggests eliminating the food, even if the suspicion is weak, and to carry out an Oral Food Challenge soon as possible.  Prof. Miceli Sopo says, "We have heard stories of children, who had 6-8 acute episodes before arriving at the diagnosis, it does not seem right."

If you interested in learning more about the Italian FPIES registry write to stefano.micelisopo@gmail.com

To join the FPIES Foundation Global Patient Registry follow this link.  If you've already joined you can instantly see survey and data results by clicking “View Data” here. 

This interview conducted and article written by Victoria Warren.  Victoria is a television news reporter for the NBC affiliate in Boston, WHDH-TV.  Victoria is a parent volunteer with The FPIES Foundation Volunteer Advisory Board.  Follow Victoria on twitter @VWarrenon7.