Rare Diseases Day: FPIES - Jen's Story


Guest Post by Jen from www.chapmanbaby.blogspot.com

Hi! My name is Jen, and I'm the parent of a child who used to have something called FPIES. FPIES stands for Food Protein-Induced Enterocolitis Syndrome and is pronounced "F" then "PIES." FPIES is a rare food allergy-like syndrome that causes inflammation of the bowel and small intestines.  The most common FPIES trigger foods are milk and soy, but children with FPIES can react to any food (including breast milk).  There are no tests to determine what a child with FPIES will react to, as with regular food allergies. IgE prick testing and blood tests can only rule out or give results for regular food allergies, but will not help with diagnosing FPIES. Typical reactions to a trigger food are vomiting and diarrhea, which often happens within 2 hours of ingestion of the trigger food. Reactions like this can lead to lethargy, dehydration and shock.  The only way to know if a child will react to a certain food is to have the child ingest the food. This sort of test is called an oral food challenge, which is commonly done in a doctor’s office or in the hospital.

My son Bronsen had his first reaction was when he was one day old. I was a nervous new mom; I thought he was hungry and I was upset. The nurses encouraged me to give him a supplement of formula while I waited for my breast milk to come in.  Minutes later he vomited. The nurses suggested he drank it too fast. Later that day the nurse made a comment to me about how my son was the most lethargic baby she had ever seen.  My breast milk came in and I continued to nurse him. At 4 weeks of age he seemed hungry all the time, so I gave him another supplement of formula. The vomiting happened again, and so did the lethargy.  2 weeks later it happened again. This time he was so lethargic that he slept for 9 hours following ingestion of the formula.  We decided a visit to the pediatrician would be appropriate. We made the appointment and went in to discuss our concerns. By this time he was "fine" and our pediatrician thought he might have just had the flu. I knew there was no way my 6-week-old baby had just had the flu 3 times in his short life. We went on with life as usual and I continued to nurse him. I did think about the pattern that had taken place, how every time I gave him a supplement of formula he projectile vomited and became lethargic.

A couple weeks went by before I had to think about it again. He was 10 weeks old and I remember nursing him over and over. He just didn't seem full.  I decided I would give him a supplement of formula. I gave him a different kind of formula so I thought maybe it was just that. I gave him a 3 oz supplement and 2 hours later I heard him gurgling in his co-sleeper. When I first saw him he was covered in vomit from head to toe.  I picked him up and took him into the bathroom. I had my husband get the number for the pediatrician. The nurse suggested we go to the hospital to have him checked out, but she assured us that he was probably fine.  By that point he was vomiting bile and struggling to hold his head up. He was choking on his vomit and needed to be suctioned every couple minutes.  His color was gray and I knew at that point that there was something wrong with my baby.  We rushed him to the ER, where we were told by the entire staff that no one knew what was wrong with him. They were treating him and said he was very sick. On a scale of 1-10, 10 being the worst they rated him an 8 and told us that in a few minutes we would know if he would respond to their treatments.  Luckily for us, he did respond and within minutes he was smiling and flirting with the nurses.  We stayed in the hospital for 4 days, tests upon tests and we didn't ever get a definite answer as to why this was happening. On the 4th day we were told we would see an allergist the following day.  We checked out, went home and made it to our appointment the next day.

I'll never forget walking in, introducing ourselves and hearing the doctor say, "Your child has FPIES."  I had never heard of FPIES and I had no idea what it meant.  All I could remember the doctor say was we don't know if your child will ever be able to eat certain foods.  He might grow out of it but he might not.  I went home and started researching FPIES.  I had little or no support, no one knew what FPIES was and I didn't know how I could raise a child who may never eat real food.  I stayed awake at night, holding him, feeding him and crying. I was worried he would never go to school, he would never enjoy food, he would never eat food.  As I continued to research FPIES, I was able to locate a couple of other parents who were going through what I was going through. They walked me through it, along with our doctor. We didn't supplement formula after his reaction and diagnosis at 10 weeks.  At 12 months of age we added Nutramigen, a formula that our doctor thought would be okay.  He drank breastmilk from 10 weeks to 12 months, we added fruits at 8 months and skipped cereals altogether.  At 15 months he was admitted to the hospital, an IV was set in place and he was given small amounts of rice flour mixed in applesauce.  He passed his first major food challenge. I knew then that if he could never have anything else, he would eat rice products and fruit.

Over the course of our FPIES journey, we had several atopy patch tests. This test has not been proven to show what a child with FPIES will react to, but for us the results were right on. For this test small round discs that contain different foods are placed on the child’s back for hours. If a reaction occurs under or around the disc, it could indicate that a child with FPIES might react to that food if ingested.  We did that testing because it was the only test that might give us some sort of results.  Over the next couple years we did food challenges in the doctor’s office. We added foods in one at a time and since Bronsen was not reacting we were able to do some food challenges at home.  We would find out that Bronsen had a regular food allergy to milk, which did complicate matters.  At 3.5 years of age we did a prick test, to see if Bronsen had outgrown his milk allergy. After passing all other foods we challenged, milk was the last "big" item on the food list. We weren't going to give it to him if he had a regular food allergy to it, so we checked that first.  We did an in-office food challenge that lasted around 6 hours, and Bronsen passed with flying colors. After a 24-day challenge of adding in milk, we called it a pass. We still haven't allowed Bronsen to eat fish.  We haven't pushed him to eat foods he isn't interested it, because honestly, I'm worried he might have an FPIES reaction.  We skipped over things like avocado and some vegetables.  We feel that when the time comes and he wants to try those foods we will let him, but for now he is doing well and we wouldn't want to take a chance and have him react to something he doesn't need in his diet.

Our doctor has explained to us that his FPIES is probably completely gone, as Bronsen has passed the challenges to the major foods.  For us it's a completely different life. We went from packing all of his meals, to purchasing an in-car cooler to keep foods cold, to wanting to eat out every night of the week, as a family, just because we can.  It's been a long road and an amazing journey. Throughout the journey I've met so many wonderful, amazing people who have children with FPIES.  I've gained friendships and I've tried to educate people about FPIES whenever possible. I feel like FPIES happened to us for a reason, and that reason is because I am someone who will talk and raise awareness.  Because FPIES is so rare, it is commonly misdiagnosed.  I hope that by spreading the word about FPIES and providing resources I can help someone who may be walking in the shoes I once walked in.

Over the past few years I've met so many wonderful, amazing parents who are going through what I went through. Recently, a fellow FPIES mom created and established the IAFFPE (International Association for Food Protein Enterocolitis). The IAFFPE is a non-profit corporation and organization that provides education, support, and advocacy for individuals with FPIES and their families. The IAFFPE partners with leaders in the medical community to develop evidence-based research with a focus on early detection and new treatment options. The foundation’s research efforts are coupled with emphasis on education and advocacy to positively impact the lives of patients and families coping with FPIES. Ultimately, the IAFFPE represents families and medical professionals united by a common focus: finding a cause and a cure for FPIES. I've been lucky enough to join this foundation and take on the role as the Family Advocacy Co-Director. Please visit our website to learn more about FPIES.  There are many resources for those families who are just getting that FPIES diagnosis. Support makes all the difference in the world!

Find us on facebook and "like" our page! http://www.facebook.com/IAFFPE

Find us on the internet! http://www.iaffpe.org/
Feel free to send us an email with questions or your need for support.  We will help in any way we can.contact@iaffpe.org