FPIES stands for Food Protein-Induced Enterocolitis Syndrome. It falls within the spectrum of food allergies, although it is a non-IgE mediated allergy, meaning it doesn’t cause immediate reactions such as hives or anaphylaxis.
Unlike skin-prick testing for Ig-E mediated allergy, there is currently no test to ‘prove’ the diagnosis of FPIES- oral food challenges are the ‘best’ option if the diagnosis is in doubt.
Usually though, the diagnosis is based on the clinical history.
Typically acute FPIES presents in infants- at the stage where solids are being introduced. Acute FPIES is typically characterised by repetitive profuse vomiting (90%–100%), pallor (14%–67%), and/or lethargy (3%–85%), typically occurring 1–4 hours after ingestion of the offending food. Diarrhoea may follow 5–10 hours later, in up to 30-50% of children. Low blood pressure in FPIES has been reported in up to 15% of patients (Michelet 2017).
A personal history of eczema and a family history of atopy are common. Siblings are not commonly affected (Mehr and Frith 2014).
Symptoms resolve with removal/avoidance of the causative food. The reaction usually occurs on first or second exposure. Often the diagnosis is difficult to make- things like viral gastroenteritis or sepsis (severe infection) look very similar and are more common than FPIES.
The most common foods for children to react to are:
Rice, oats, poultry (chicken), cows milk, soy and grains, some fruit and vegetables. Essentially any food can cause FPIES- most children (65-80%) react to only one food, but it is possible to have FPIES to more than one food.
In 2017 the first international guidelines/position statement about FPIES was released (Nowak Wegrzyn et al). They provide clear evidence-based guidelines as to the diagnosis and treatment of FPIES.
Treatment of acute FPIES includes ensuring adequate fluids- in some children, this may mean hospitalisation and intravenous fluid. Some children may benefit from medication to reduce the vomiting (ondansetron- Zofran), some children may benefit from steroids. The use of these medications will depend on your child’s clinical condition and all must be prescribed by a doctor. Sometimes you immunologist may provide a letter/action plan to carry with you in the event you do need to attend the emergency department.
The mainstay of treatment is to avoid the food that triggers FPIES. If the infant is still being breastfed and does not seem to react to feeds and is not failing to thrive, then there is no need to omit the food from the maternal diet (Nowak-Wegrzyn et al 2017). Generally speaking it is not routinely recommended to avoid products with precautionary labelling (the ‘may contain traces of…’ warning labels).
It is important to monitor weight and growth of children with FPIES.
It is also vital to continue to introduce new foods and textures under the supervision of an immunologist/gastroenterologist of a dietician who understands FPIES. Finding ways to vary foods offered, textures helps kids still meet their developmental milestones with food- so important in the long run to help reduce the risk of fussy eating/food aversions and restricted diets (restricted by the child beyond their medical restrictions)
Most often, children will grow out of FPIES. Generally speaking those with FPIES to cows milk or soy protein gain tolerance earlier than those with FPIES to grains (1-2 yrs vs 3-4yrs). This means it is important to keep checking in with your immunologist regularly to see whether it may be appropriate to book in an oral food challenge and re-test things under supervised conditions.
Some children with FPIES will develop IgE allergies, but not all. As with any restricted diet, input from dieticians to ensure you are meeting your child’s nutritional needs is so important.
Looking after yourself
Amidst all the stress of a new diagnosis, it is so important to be kind to yourself. Starting solids in infants can be a stressful time anyway, by adding FPIES into the mix it can become terrifying. Food can very quickly take over your life- the sourcing, preparing, feeding, cleaning, the worrying- multiple times a day- it is exhausting.
Please please please, don’t think you have to do this alone. There are some helpful online resources available for FPIES which I’ll link to below and if you are looking for recipe ideas, I’ll be developing some recipes and posting them in the coming weeks. Making time to create space for some self-care even for just 10 minutes a day is so important for maintaining your mental wellbeing. Reach out if you need to- don’t ever feel like you can’t ask for help.
Australian Information on FPIES
The international position statement on FPIES released last year. It is a scientific article, so not directed towards non-medical readers, but the table E3 might be a useful starting point when thinking about how and why to substitute foods for your little person with FPIES. This should be done in conjunction with a dietician though.