Food Protein-induced Enterocolitis Syndrome: Dean's Story

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Dean was only minutes old when one of the delivery doctors told his parents, Dina and Frank, their son’s sugar was low, a common condition and easily treatable. When the medical staff suggested the first-time mom supplement her breast milk with formula for Dean’s initial feedings, she agreed. “That first night he was so fussy,” recalls Dina. “He was crying. But I felt like he was crying more than a newborn. I felt like he was in pain.”

Dean What Dina now refers to as her intuitive “momma gut,” was right. But it wasn’t until weeks later — when staff at Children’s Hospital of Philadelphia (CHOP) diagnosed Dean with food protein-induced enterocolitis syndrome (FPIES), a rare food allergy that affects the gastrointestinal tract — was the family able to put Dean's rough welcome into the world behind them.

Two-year-old Dean is now a thriving, happy kid. Dean's parents attribute their son's relatively normal life to the quick diagnosis and comprehensive care and treatment from CHOP and its staff including: Terri F. Brown-Whitehorn, MD, attending physician and food allergy expert; Amy Dean, MPH, RD, clinical dietician; and Andrew B. Grossman, MD, and Gayle H. Diamond, MD, pediatric gastroenterologists.

Children's Hospital of Philadelphia and its Food Allergy Center have led research efforts worldwide into FPIES, which is often misdiagnosed as a severe stomach bug because the symptoms are so similar. Typical symptoms of FPIES include severe vomiting, diarrhea and dehydration, and can lead to blood pressure changes, lethargy and failure to thrive. Unlike most food allergies, symptoms of FPIES do not begin immediately after eating and can take hours for severe symptoms to begin.

Such was the case with baby Dean.

‘He constantly wanted to be held’  

Dean’s fussiness that first night was still on Dina’s mind when she spoke to the pediatrician the next morning. When she expressed her concerns, the pediatrician recommended she switch formulas and begin feeding Dean a sensitive formula that is easier to digest.

For the next few days in the hospital, and even after as the family was discharged and settled into their home in Deptford, NJ, Dean seemed to improve. But it wasn't long before Dina noticed new troubling symptoms: Dean seemed congested constantly, had explosive diarrhea, and would wake screaming every two hours when sleeping in his crib.

“He constantly wanted to be held,” Dina recalls. “I would swaddle him and just hold him. He would sleep on me.”

At 3 weeks old and still feeding on the sensitive baby formula, Dean was having multiple bouts of diarrhea a day. Dean's pediatrician suspected he had a milk allergy and instructed Dina to begin a new formula.

The same day she made the formula switch, Dina took Dean to visit relatives. “Both my mom and aunt looked at Dean and said, ‘He doesn’t look right,’” recalls Dina. Her concerns were heightened.  

That night, when Dean was still experiencing diarrhea and refused his bottle, Dina took him to CHOP at Virtua Voorhees in New Jersey, where clinicians from Children's Hospital of Philadelphia collaborate with Virtua to treat babies. Dean was treated for severe dehydration; blood tests were performed and he was admitted.

After two days at Virtua Voorhees, Dean's condition worsened. He was transferred by ambulance to CHOP's pediatric intensive care unit (PICU) in Philadelphia for additional testing, treatment and monitoring.

Classic case of FPIES

“There was so much going on with Dean,” his mom recalls. After running a battery of tests, doctors ruled out metabolic disorders, cystic fibrosis, meningitis and bacterial diseases.

After three days in CHOP's PICU, Dean was transferred to a step-down floor. Though he was accepting a bottle, he wasn’t sleeping. A few hours after Dean drank a bottle with formula, Dina left him with a nurse to use the restroom. When she returned, Dean was gray and lethargic.

“What just happened?” Dina’s recalls thinking.

Again, Dean needed IV fluids, and again his formula was temporarily restricted. By now, Dina was extremely frustrated. But after 2½ weeks in the hospital, Dean appeared to be better and was sent home.

Dina believed there was more to her son's illness and made an appointment with CHOP pediatric gastroenterologist Dr. Grossman at his office in Voorhees.

After Dr. Grossman reviewed all of Dean's symptoms, test results and his parents' concerns, he diagnosed the 7-week-old baby with "a classic case" of food protein-induced enterocolitis syndrome (FPIES). He referred them to the FPIES Clinic, part of the Food Allergy Center at Children's Hospital of Philadelphia.

Soon after, Dean and his parents met with Dr. Diamond and Dr. Brown-Whitehorn from the FPIES Clinic. They agreed with Dr. Grossman's diagnosis: Dean had FPIES.

Treating FPIES

Every child’s FPIES reaction is different. For that reason, FPIES treatment must be determined on a case-by-case basis and customized to meet the child's unique needs.

Treatment for Dean included switching to an amino-based baby formula. His body reacted well. When Dean turned four months old and he began solid foods, Dina was instructed to introduce fruit and vegetables one at a time to see how Dean would react. First on the list: apples. Each food trial lasted a week.

Dean reacted to carrots, butternut squash and white potatoes with bouts of vomiting and diarrhea. All three were immediately stopped and he would return to eating only formula for a weeklong period of gut rest.

“I just kept introducing new foods,” Dina says. “It was a long process.”

At the suggestion of Dean’s doctors, Dina joined an FPIES support group for moms on Facebook, which she has found to be very helpful. Not only has she found new recipe ideas, she’s reminded that her family is not alone.

Steady progress

In early 2019, doctors gave Dina approval to try adding rice, and later soy, to Dean's diet to determine if the foods still caused him to have allergic symptoms. To his family's delight, "he passed both rice and soy," Dina says.

Today, Dean can safely eat poultry, pretzels, pumpkin, apples, rice, soy and his favorite — turkey hot dogs. Dean's family must continue to restrict him from all foods that contain cow's milk — a common FPIES food trigger — but the 2-year-old is a healthy 30 pounds now.

Dean’s doctors are “very happy” with his progress, Dina says. For many patients, FPIES is not a lifelong condition; some children outgrow it by the age of 3. Dean’s parents have already seen some positive signs of this, and remain hopeful he will outgrow all his food allergies.

But, Dina says:

no matter what happens in Dean's future, we know CHOP will be by our side.

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