Food Protein Induced Enterocolitis Syndrome: Isaiah’s story
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As an infant exclusively breastfeeding, Isaiah was a healthy, thriving infant. At 6 months old, his mom, Cayla, began to introduce solid foods into Isaiah's diet. One day, when he was 7 ½ months old, Isaiah began to vomit profusely. He was lethargic, pale and barely responsive. At first, Cayla didn't know what had triggered the attack. He had eaten peas and pears — but that was hours before.
Cayla rushed Isaiah to a local emergency department where doctors were perplexed by his symptoms. They suspected he had viral gastroenteritis and that the vomiting had triggered a sudden drop in his heart rate and blood pressure, leading to lethargy and pale skin. But Cayla, a nurse practitioner herself, was suspicious of that diagnosis since Isaiah perked up as soon as he received IV fluids.
Perplexing symptoms
“I had a gut feeling it was food related because I couldn't think of anything else,” she recalls. As a precaution, she decided to keep peas and pears out of his diet for a month.
“He was fine and I thought I was being over-anxious,” she says. “So, I gave him peas and pears again and he had the same reaction a few hours later.”
Another trip to the emergency department followed. This time, doctors performed several tests including an upper GI series, thinking Isaiah might have reflux. Once again, Isaiah returned to normal quickly and left the hospital without an explanation about why he'd gotten so sick.
At a follow-up visit with Isaiah's family doctor, the doctor told Cayla he suspected Isaiah had food protein-induced enterocolitis syndrome (FPIES), a rare food allergy that affects the gastrointestinal tract. Cayla took Isaiah to a pediatric allergist and pediatric gastroenterologist in their local area for more information, but didn't learn much. Their family doctor encouraged Cayla to schedule an appointment with a specialist at the Food Allergy Center at Children’s Hospital of Philadelphia (CHOP), the only pediatric program nationally recognized for its expertise in diagnosing and treating all types of food allergies.
Decoding the mystery
Soon after, Cayla brought Isaiah to CHOP’s FPIES clinic, one of the specialized clinics run by the Food Allergy Center.
Isaiah was examined by a multidisciplinary team including Terri F. Brown-Whitehorn, MD, an attending physician in the Food Allergy Center; Gayle H. Diamond, MD, a pediatric gastroenterologist; and Amy Dean, MPH, RD, a clinical dietitian.
After an examination, hearing about his symptoms, and learning the family's medical history, the team agreed Isaiah had FPIES. Unfortunately, there is no one clinical test to specifically diagnose FPIES. Instead, diagnosis is based on a clinician's experience, the child's symptoms and medical history.
FPIES can be challenging to diagnose because it presents differently than other food allergies like IgE-mediated allergies that produce an immediate reaction after eating the trigger food. In FPIES, reactions typically take one to four hours to appear. In addition, FPIES symptoms can be more varied than other food allergies, and may include vomiting, diarrhea, lethargy and changes in body temperature or blood pressure. FPIES typically isn’t associated with reactions in the skin, mouth, throat or lungs.
Another hurdle in diagnosing FPIES is that it often does not show up on the traditional skin-prick tests used to check for allergies.
Coping with FPIES
While no one wants to hear their child has a condition that many in the medical community don't fully understand, Cayla says she felt relief to finally get a diagnosis.
“Having a doctor who could tell me why he was sick — even if not all the pathology behind it — helped,” says Cayla.
“I felt empowered knowing that it’s a real diagnosis and I’m at a place where they can help treat it. They had clinical experience with FPIES, which is hard to find. I had never even heard of it in my training as a nurse.”
With pediatric food allergies, parents bear the burden of not just caring for their child, but also managing the logistics of avoiding potential exposures and keeping track of foods being trialed. With this too, Cayla found help at CHOP.
“I’ve been so impressed by Dr. Brown-Whitehorn’s kindness. She really cares for Isaiah, but I feel like she cares for us too — things like how stressful it is to trial foods at home,” Cayla says. “Her availability to patients is amazing — she calls me directly to answer my questions. She has inspired me in my own patient care.”
At–home food trials
Once FPIES is suspected, treatment consists primarily of avoiding known triggers and treating any acute reactions.
To figure out Isaiah’s potential triggers, they would have to test each food one at a time. At the direction of Dr. Brown-Whitehorn, Cayla gave Isaiah a half teaspoon of the food being tested on the first day. Cayla would monitor Isaiah for a negative reaction, such as anaphylaxis or vomiting. If Isaiah reacted, the test would be over immediately and he would no longer eat that food. If there was no reaction, Cayla would gradually increase the amount — or "dosage" — of the food item being tested until he could eat a full serving without a reaction.
“It was really scary. I felt like we were playing Russian roulette," Cayla says. "I never knew when he might go into shock.”
Over the next few months, they discovered Isaiah had many food allergies. On his first birthday, he only had five “safe” foods: broccoli, pumpkin, apples, strawberries and lamb.
Despite his limited diet, Isaiah continued to put on weight. But Cayla worried about his growth, and spoke to dietitian Amy Dean, who helped strategize the order in which to test foods to give Isaiah optimal nutritional balance. The dietitian also gave Cayla recipes with just a few ingredients to make it easier to create nutritious, interesting meals for a child just learning to appreciate food.
“That was a big concern of mine because I’m not an amazing cook,” Cayla says with a laugh. “With babies, you want to introduce a lot of food textures and flavors to prevent them from avoiding foods later. Having things like a three-ingredient cookie recipe gave me a lot more confidence.”
Outgrowing allergies
Today, Isaiah is 2 ½ year old and thriving. He is a sweet, laidback toddler who loves singing and playing with toy tractors and trains. Best of all, he has a much more balanced diet of 35 foods … and counting.
Isaiah continues to do food trials. Every two weeks, Cayla introduces a new food to expand his diet and palate. All signs point to significant improvement: His last allergic reaction was a year ago.
Better yet, he recently passed an oral food challenge test at CHOP and can now eat pears — one of his original allergens. Some children with FPIES grow out of their allergies between the ages of 3 and 5.
Isaiah will continue to do in-hospital oral food challenges for his remaining trigger foods to see if they can be added back into his diet.
“When your child is first diagnosed with FPIES, it can feel dark and terrifying,” says Cayla.
“It’s helpful to know that you will see progress, foods will be added to their diet — and there is a chance they can outgrow it.”
In the meantime, she urges other FPIES families to rejoice in the milestones along the way. “I remember crying with happiness the first time he had a cracker,” she says.
“Take those moments in," she says. "Even if it’s a small step, you’re making progress.”
