Allergy and Asthma Today - Spring 2015

Rare Disease

Gary Fitzgerald 2015-01-30 12:54:35

FPIES – It’s Not Your Typical Food Allergy Amanda Bofferding was excited to start her 6-month-old son Matthew on solid foods. It started well … until she fed him some rice cereal. Two hours later, Matthew started vomiting every few minutes. “An after-hours nurse told me to give him sips of water every five minutes until he could hold it down,” Amanda said. “After two hours, he slept for the rest of the night.” The next day, Matthew was fine except for diarrhea. A week later, Amanda tried feeding Matthew rice cereal again. Two hours later, the vomiting returned. “I was worried and knew something was wrong,” she said. Amanda googled “vomit rice cereal” and discovered a disease with the acronym FPIES – Food Protein-Induced Enterocolitis Syndrome – that mirrored Matthew’s symptoms. She took him to see an allergist and the diagnosis was confirmed: Matthew had FPIES, a severe type of food reaction that affects infants and young children. How does FPIES differ from food allergy? FPIES is a non-IgE-mediated reaction to a food protein that occurs in the gastrointestinal system, typically during the first year of life. Any food protein is potentially an FPIES trigger, but the most common are milk, soy, rice and grains. Some children react to just one food, while others experience reactions to multiple foods. It’s also possible for some infants to experience reactions to trigger foods via breast milk. Unlike most food allergies that produce immediate reactions such as swelling and hives, FPIES reactions are delayed and usually begin about two hours after ingestion of the trigger food. Acute FPIES reactions can include profuse vomiting, diarrhea and fatigue. In about 20 percent of cases, the child will experience a severe reaction leading to shock and need emergency intravenous fluids for rehydration. In rarer cases, intravenous steroids may be used to reverse the effects of shock. Chronic FPIES symptoms can include vomiting, diarrhea (sometimes with blood or mucus in the stool), weight loss, and in the most severe cases, an inability to gain weight. How is FPIES diagnosed? An FPIES diagnosis is only made after considering a patient’s history of symptoms and exclusion of all other possible illnesses, including IgE-mediated food allergies. Misdiagnosis is common. Symptoms might suggest a stomach virus and many pediatricians and emergency physicians remain unaware of FPIES. Standard IgE-based allergy tests usually come back negative, so the primary test for FPIES is trial and error and medically supervised oral food challenges. Is there a treatment? There is no cure for FPIES. Strict avoidance of the trigger food is necessary. Parents should work with a dietician to design a personalized dietary plan that ensures proper nutrition. A hypoallergenic diet can be an amino acid elemental formula or an extensively hydrolyzed casein or whey formula. Children usually outgrow FPIES between the ages of 1 and 3, although for some it lasts until 6 or 7; for others it is a lifelong condition. Update on Matthew When Matthew turned 3 years old, he had more than 30 safe foods, including some in the same food family as his triggers. When Matthew was 3 and a half, Amanda took him to an allergist for his first follow-up oral food challenge – dairy. He passed. “Matthew was so excited,” Amanda said. “It was an awesome moment. We are waiting to trial rice next, and hopefully he’ll pass challenges to all of his triggers in the coming months.” FPIES Resources • The FPIES Foundation: • International FPIES Association (I-FPIES): Reviewed by Prem Menon, MD and Fallon Schultz, President and Founder of I-FPIES

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