An Infant With Acute FPIES: The New Guidelines

Stephanie A. Leonard, MD


August 09, 2017

Discussion: Management of FPIES

Management of acute FPIES should be based on the severity of the patient's symptoms, with priority given to rehydration. Oral rehydration in mild acute FPIES may be sufficient, whereas moderate to severe acute FPIES may require multiple intravenous fluid boluses. Two recent case series have supported a role for ondansetron in shortening the duration of vomiting and therefore presumably preventing subsequent symptoms, although more studies are needed to understand the drug's efficacy. A single dose of systemic steroids may be useful for presumed gut inflammation, although data to support this are also lacking. Epinephrine does not treat FPIES reactions; therefore, autoinjectors are not prescribed for patients who are not at risk for anaphylaxis.

The following medical treatment of acute reactions is recommended. For mild FPIES (one or two episodes of vomiting and no lethargy), oral rehydration with breast milk or clear fluids may be attempted.

In moderate FPIES (three or more episodes of vomiting and mild lethargy), seeking medical attention in the emergency department is advised, and if the patient is > 6 months of age, an intramuscular dose of ondansetron (0.15 mg/kg; maximum dose, 16 mg) may be administered. IV fluid boluses (20 mL/kg) may be considered, and vital signs should be monitored closely. The patient can be discharged home if oral intake is tolerated.

In severe FPIES (three or more episodes of vomiting and severe lethargy, hypotonia, or ashen or cyanotic appearance), emergency services should be sought and IV fluids and ondansetron administered. IV methylprednisolone (1 mg/kg; maximum dose, 60-80 mg) may be considered, and vital signs and electrolytes should be monitored and corrected as needed. Intensive care unit admission may be needed in the case of persistent or severe hypotension, shock, extreme lethargy, or secondary respiratory distress.

Long-term management of FPIES includes strict avoidance of the trigger food, dietary advancement, education on how to respond to reactions, and monitoring for resolution.

Long-term management of FPIES includes strict avoidance of the trigger food, dietary advancement, education on how to respond to reactions, and monitoring for resolution. The patient in this case reacted to goat's milk, which cross-reacts with cow's and sheep's milk; therefore, it was recommended that she avoid all of these milks. Most infants with FPIES do not react to the trigger food in breast milk; therefore, maternal elimination of the food from her diet is not recommended if the infant remains asymptomatic and is thriving. In the recent Australian population study, only 5% of infants with FPIES were reported to have reacted during exclusive breastfeeding.[3]

In cow's-milk FPIES, hypoallergenic formulas are recommended as supplementation or in formula-fed infants. Extensively hydrolyzed casein formulas are tolerated by most patients with cow's-milk FPIES; however amino acid-based formulas may be needed in 10%-20% of infants.

Nutritional guidance should be sought in infants with FPIES, because they are at risk for nutritional deficiencies owing to dietary restrictions and delays in food introduction, which may also lead to feeding difficulties later on. Recommended foods should meet nutritional needs and enhance developmental skills, such as acceptance of a variety of textures and motor skills. Weight and height should be regularly monitored.

The new FPIES guidelines have detailed dietary recommendations based on the age of infants and risk for FPIES to common foods, as well as alternative dietary sources of nutrients when avoiding certain foods. Overall, it is not recommend that introduction of complementary foods be avoided beyond age 6 months in infants with a history of FPIES. If early-introduced foods, such as fruits, vegetables, and red meat, are tolerated, then the diet can be liberalized. Tolerating one food from a food group (eg, grains) is a good sign that other foods in the same group will be tolerated.

Supervised oral food challenges to encourage introduction of foods should be considered if parents are hesitant to start solids in infants with a history of severe or multiple-food FPIES. Such challenges could involve individual foods or a mixture of several foods that, if tolerated, could be given in gradually increasing serving sizes at home.

The resolution of FPIES varies depending on the age of onset, type of trigger food, and nationality. Overall, it appears that cow's-milk and soy FPIES resolve earlier than FPIES to grains and other solid foods, as was seen in this case. Tolerance to cow's milk ranges from 85% by 3 years in an Israeli population to a median age of 5.1-6.7 years in US populations. The average age at the time of soy tolerance is 12 months, with a range of 6 months to 22 years. The average age at the time of grain tolerance is 35 months, and 42 months for other solid foods.

The heterogeneity of the data on tolerance does not support clear guidelines on when to evaluate for resolution. In the United States, it is suggested that children with FPIES be reevaluated every 12-18 months. It is not known whether FPIES from seafood or egg in older children or adults resolves, but periodic monitoring is also recommended. Specific recommendations on conducting oral food challenges to assess for tolerance are available in the new guidelines.

In summary, FPIES is a non–IgE-mediated food allergy affecting primarily the gastrointestinal system and, when severe, the cardiovascular system secondary to hypovolemic shock. FPIES is a clinical diagnosis, and it is important to rule out other potential causes. Management should include guidance on avoidance, with special attention in infants to nutrition, growth, and developmental feeding skills. Patients should be monitored periodically for resolution of FPIES.

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