From Dismissed to Determined: Creating the Roadmap to Solve FPIES

Imagine feeding your baby rice cereal or avocado for the first time. Fast forward hours later to episodes of vomiting so severe they leave your baby pale, lethargic, showing signs of shock. Is it stomach flu? Something else? Then perhaps the scenario repeats, eventually sending your child to the ER. What can you feed this baby? This is how Fallon Schultz’s FPIES advocacy began 18 years ago when she founded iFPIES (The International Food Protein-Induced Enterocolitis Syndrome Association.) Where has her advocacy led? Find out...

As a new mom, I knew fear, panic, frustration, grief that my motherhood journey wasn’t anything I expected.

I gave birth to my son in 2008 and right away something seemed very wrong. He was a breastfed baby and was covered from head to toe with bleeding eczema. He would vomit every time he ate. His diapers were bloody. My heart was breaking.

It took two years for my son to get an FPIES (Food Protein-Induced Enterocolitis Syndrome) diagnosis; at the time no one really knew anything about the disease. Those two years were fear-filled and frantic, trying to navigate a rare disease without a roadmap, without a community, with providers doubting I was telling the truth about my son’s symptoms, and desperately fighting for insurance coverage to go to Children’s Hospital of Philadelphia.

I remember the day my son was diagnosed.

I said, "ok, so what’s the treatment?"

"We don't know. We don't have one."

"So what do I do?" I asked.

"We don't have any interventions. You're just going to have to work through it by trial and error."

That wasn’t good enough for me. I just couldn't accept that this was the path for us and other FPIES patients, the way we had to exist in this world – trialing foods, becoming horrifically ill, going into hypovolemic shock.

Getting answers became my life’s mission. And when chasing answers?

I can be relentless.

What is FPIES?

When people think of allergic reactions they think of hives, breathing issues, even anaphylaxis. But there is actually a full spectrum of food allergy disorders that don’t present with those specific symptoms.

FPIES is one of them.

FPIES is a non IgE-mediated food allergy and reactions affect the gastrointestinal system. The reactions can be violent.

With FPIES, profuse vomiting, sometimes 20 to 30 times over hours, along with bloody diarrhea, starts two to four hours after eating a trigger food. In about 20% of cases the vomiting can become so severe that patients become significantly dehydrated and can go into hypovolemic shock. This is a serious medical emergency often requiring medical resuscitation to replenish fluids and resolve symptoms.

Literally any food can trigger an FPIES reaction. In 2008, milk, soy, rice and oat were the high risk, common triggers. Over the last 18 years these triggers have morphed and seem to occur more broadly with the first foods introduced. This has been most pronounced in recent years with the adoption of the 2017 early introduction guidelines for IgE-mediated food allergy. Prior to 2017 there wasn’t a single documented case of peanut FPIES. Today, we are seeing an alarming increase in peanut FPIES, and egg as well. Trigger foods can be the most unlikely foods you could imagine, like bananas or sweet potatoes, avocados or chicken.

A longitudinal cohort study published by the Children's Hospital of Philadelphia found that most patients either react to 1 or 2 foods, or 6 and more.

When my son was born, back in the ‘dark ages’ of FPIES, patients and parents were told that all patients outgrow it by age 3. But to date there have not been any studies to confirm this. My son will head to college soon with five FPIES trigger foods, including peanut and dairy, down from the 32 foods he once avoided.

FPIES prevalence is estimated at approximately 2.3 million children and adults in the U.S. and 10.5 million globally. This data, collected from a prevalence study by Dr. Ruchi Gupta (et al) of the Center for Food Allergy and Asthma Research was further confirmed by the implementation of the ICD-10 (diagnosis) code for FPIES which went into effect in 2013. I believe the numbers are significantly understated because hard data always lags actual lived experience.

If you’d like to dive deeper into FPIES, here is the largest cohort study to date on FPIES, representing 441 children, with significant and alarming findings on the demographics, foods avoided, spectrum of reactions, and variety of medical management provided through the lens of the caregiver.

How is FPIES diagnosed?

Diagnosis is a journey.

The lack of biomarkers and diagnostic testing makes it difficult to confirm a diagnosis of FPIES. The gold standard diagnostic is an oral food challenge where an IV is placed, the patient is fed a small amount of a suspected trigger food under supervision, and then observed for several hours until they react, or not.

Initially, to identify trigger foods, patients introduce foods one at a time for several consecutive days and anxiously await the onset of symptoms. If a food is a trigger, patients may endure prolonged episodes of vomiting, which may be severe and require ER visits.

When triggers have been identified, it is recommended that patients undergo oral food challenges, either at home or in the clinical setting, and this can be traumatizing. What is even more challenging? Consenting to these procedures — feeding your child a known or suspected trigger and anxiously waiting to see if it will make them sick. These clinical appointments are often hard to schedule with most centers having 6-month to 2-year wait lists.

Due to the well-documented family anxiety and fear, and limited diagnostic and treatment options, many families avoid introducing foods to their child. Prolonged avoidance of food introduction carries additional risks. Patients can become nutritionally deficient, or develop feeding difficulties necessitating feeding therapy. Patients can also even develop secondary conditions from poor nutrition like growth delays.

This is equally challenging for patients with adult onset FPIES, who often do not have access to knowledgeable clinicians or awareness of emergency management for their symptoms.

As you can imagine, given the severity of the reaction, patients and families are not signing up super excited to do oral food challenges, or to repeat them to see if FPIES has resolved. I have been through 50 oral food challenges with my son over the years which has placed a significant burden on our family and caused a disjointed relationship with food.

The emotional and psychological burden of oral food challenges on the entire family is immense.

Who gets FPIES?

Clinically, the first documented cases of FPIES were made in the 1970's by Dr. Robert A. Powell who identified the persistent characteristics (i.e., chronic phenotype) in formula-fed infants who developed bloody stool that in some cases, progressed to vomiting.

But it’s not a disease just confined to infants and toddlers.

We now recognize that FPIES is a full age spectrum disorder, with both adult onset and, in some cases, unresolved pediatric FPIES that carries on well into adult years. FPIES can also run in families.

It is a common report amongst adults with FPIES that the onset was sudden, primarily after eating shellfish – mollusks (e.g., clams, oysters) and crustaceans (e.g., shrimp, crab) – they’d previously tolerated their entire lives.

Initially, adults often dismiss the symptoms as food poisoning. It isn't until they’ve endured repeated bouts of vomiting from the same food that they suspect something’s amiss and seek evaluation. Today, we are seeing more adults being diagnosed with FPIES as awareness of the disease grows through iFPIES' work globally.

What complicates the disease is that frontline providers, like emergency departments, internists, and pediatricians, are still relatively unaware of the condition, leading to poor medical management of it and often lack of emergency resuscitation in adult FPIES patients.

The emotional toll…

FPIES families experience a heavy psychosocial burden and some even experience PTSD. For parents it’s confusing and deeply challenging to figure out how to feed your child. The same goes for adult FPIES patients trying to nourish themselves without triggering a reaction.

How do you manage the day-to-day – three meals, two snacks every single day – safely? The stress level, degree of difficulty, and the risk of making mistakes is high. There's no escaping this disease. You walk into social settings, you sit at your dinner table, you send your child off to school – it's there, ever present..

At a low point, before my son was diagnosed, I even questioned if I was cut out to be a mom if I couldn’t feed my baby safely. FPIES made me feel powerless as a mother, like I was failing my son. There is so much guilt and sadness, so many tears, that go hand in hand with living with this disease.

Navigating Life with FPIES

FPIES may still live in the gray areas of medicine, but for the families living with it every day, every aspect of daily life is dictated by this disease. I want to leave you with some thoughts that I hope will give you reassurance as you persevere through diagnosing FPIES and navigating life with it:

(1) It's important to work with an FPIES-knowledgeable physician.

As a parent, trust your instincts and experiences and advocate for evidence-based medical care. Be relentless in your quest and if you need help, iFPIES can connect you to experts.

(2) Don’t doubt yourself.

Don't ever question your ability to parent your child through this scary disease, even when the days are long, hard. Love for your child will carry you through as you peel back the layers and work towards a formal diagnosis.

(3) Know that the iFPIES community is here for you.

Our website offers resources and free support for navigating the disease, from an emergency action plan, an FPIES shock handout, and nutritional guidance, to free tele support and so much more. I find myself on the phone at 2:00 am helping families in the ER who are worried about their terribly sick babies. We’ve been where you are, and are here to help you through.

(4) Hold onto hope.

I believe we will have more answers, more tools, and more treatments in the future now that we’ve got the medical community’s attention and their support for solving FPIES.

What began as my relentless search for answers has grown into a global movement working to ensure that no family has to navigate FPIES alone. My hope is that one day, trial and error will be replaced with real answers, real treatments, and real hope.

“FPIES is the food allergy with a shade of gray...”

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iFPIES started in a hospital room in the hours and days following my son’s diagnosis. Like many other FPIES families, we had been through a long, confusing struggle to get a diagnosis. I was scared, worried for my son, and didn’t know where to turn. I was shocked to learn that there were no formal channels of support or information available to FPIES families.

With support and guidance from the Children’s Hospital of Philadelphia, we launched the ‘FPIES United Family Fund’ in 2010 to fund FPIES research and education. We soon saw the need to establish a broader international foundation to unite the medical community and FPIES families globally. iFPIES was officially launched in September 2011.

Our mission is to one day bring this non-IgE mediated food allergy on par with traditional, IgE-mediated food allergy by creating diagnostic and treatment modalities for patients.

We work with patients, providers, professional medical organizations like AAAAI and ACAAI, and the NIH and NIAID to bring this once overlooked condition into the light—so patients are believed sooner, treated more effectively and compassionately, and supported along the way.

We support over 2.3 million patients nationally and thousands of families internationally and have convened expert national and international medical advisory boards to advise on and spearhead many of the pivotal scientific advancements in FPIES.

We have taken the lead on important initiatives to build awareness of this disease and dimension the need for diagnostics and treatments. Over time, we have achieved significant milestones, building a solid case for more investment in research for diagnostics and treatments.

iFPIES offers resources for clinicians and patients, from the iFPIES Global Clinical Consortium, to our recently launched podcast, to a free tele-coaching platform where I work directly with patients and families to help them navigate all aspects of FPIES, connect them to experts, and bridge care. Providers and parents from all over the world reach out to us for information and support.

We are so appreciative of the support and generosity we’ve received from the food allergy community along the way. We could not do this without you.

Follow iFPIES on Facebook, IG, and LinkedIn, and visit our website to learn more about FPIES and find out how you can help.

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About the Author: Once a professional ballerina, iFPIES Founder Fallon Schultz has no fear of hard work and a deep passion for precise choreography — whether it's coordinating multi-disciplinary clinical work groups or convincing the NIH to dance a 'pas de deux' with iFPIES. She is deeply inspired in her work by her son Landon who was diagnosed with FPIES in 2008 after an arduous diagnosis journey and works closely with her to advocate for FPIES. Fallon leads the development and implementation of iFPIES programs, is a Licensed Clinical Social Worker focusing on health, mental health and disabilities, and provides counseling to food allergy patients and families in her private practice. She serves as an allied health and faculty member of AAAAI and ACAAI, as well as the European Academy of Allergy and Clinical Immunology.

Images: Courtesy of Fallon Schultz and iFPIES

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Additional Resources

1. International Consensus Guidelines for the Diagnosis and Management of Food Protein-Induced Enterocolitis Syndrome (2017).

2. A Slice of FPIES (food protein-induced enterocolitis syndrome): Insights from 441 children with FPIES as provided by caregivers in the International FPIES Association (2020).

3. Manuscript: Current status and future directions in food protein–induced enterocolitis syndrome–an NIAID workshop report of the June 22, 2022, virtual meeting (2022).