2

Hindsight is 20/20: What I’ve Learned About Chronic FPIES

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Over the past couple of weeks I’ve written blog posts that I liken to the first two chapters of E’s FPIES journey: leading up to the (IgE) milk allergy diagnosis and from then until we eliminated grains from E’s diet.  My goal in writing those posts was to give you a glimpse into what that time was like for us.  I don’t think words can express the degree of anxiety, exhaustion, desperation, frustration and helplessness that we felt, but maybe I provided a little insight.

Many of you have asked me if these posts were difficult to write.  They were.  It is difficult to relive that period and it is difficult to pull the snippets of memories into a coherent story.  It is also hard to remember what we knew when and what we felt when.  But I think it’s important to tell our story, so that hopefully someone else who relates can learn from it.  It is also cathartic and helpful for me to try to iron out all of the details and emotions. Honestly, I usually publish the posts feeling a huge sense of victory and pride.  After remembering where we were, our current situation is amazing!  If you have seen E lately you know that he is thriving.  We are conquering FPIES every day.  But it was quite a journey to get here and I’d like to continue to share that with you.  If for no other reason than for you to be able to truly appreciate and join us in celebrating how far we have come.

 

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 April 2014

I thought that this point in our journey would be a good time to take a step back and relate what we were experiencing to what I have since learned about FPIES.

Looking back at those first 7 months of E’s life, knowing what I know now, it is clear to me that he was experiencing chronic food protein-induced enterocolitis syndrome (FPIES).  Chronic FPIES results from the chronic ingestion of an allergen.  It is a group of symptoms that continue pretty much constantly because the allergen, which is causing them, is eaten over and over.  The classic initial presentation of FPIES is usually chronic because it is to one of the staples of the diet – cow’s milk or soy. The milk (often in the form of a baby formula) is the primary (or only) food that the infant is eating and it usually isn’t clear at first that it is related to the symptoms, which include chronic diarrhea and profuse vomiting.  The vomiting and diarrhea can lead to failure to thrive, low blood pressure, and shock (6,108,110)*, which may require hospitalization.

E certainly had the vomiting!  It makes sense the he was vomiting all the time because he was constantly exposed to his allergens.  He was only three days old when we started supplementing his nursing with a couple of ounces of (milk-based) formula.  Newborns eat every two hours and the timing starts at the beginning of a feeding.  E ate slowly (probably because his little tummy was always upset) so we were pretty much feeding for an hour at a time, taking an hour break, feeding again, and so on.  He didn’t sleep (again, probably because he was so uncomfortable), so he didn’t really have any time to get the allergens out of his system before I gave him more.  The vomiting was constant with no obvious pattern because the feeding was also fairly constant (or at least it felt that way).  It’s not clear if he was reacting to allergens in my breast milk (from my diet), which is possible but rare (6) but we followed every feeding with a couple of ounces of formula.  So he was definitely getting milk (or soy for a few days) every 2 hours or so.  Obviously, I had no idea that I was essentially poisoning him every time he ate, but I still feel incredibly guilty.

And then there was his weight.  I’m not sure if anyone ever officially classified him as “failure to thrive” but it was definitely a phrase that went through my head more than a couple of times after learning that he was less than the 3rd percentile in weight and height at his two month well-visit.  At the time I think the pediatrician assumed it was because my breast milk supply was low, and that may have been the case.  However, I am also convinced it was because he was vomiting so frequently and in such large quantities that he wasn’t digesting the nutrients and calories that he was taking in.  The poor kid was starving, despite the (seemingly constant) hour-long feedings.  Keep piling on the mommy guilt, and now add the heartbreak of realizing how sick and hungry your little boy was during his first two months of life.

I don’t think E ever had the diarrhea, which is one of the things that originally gave me pause about the FPIES diagnosis.   I’ve since learned that only about half of the children with FPIES have diarrhea (105).  In addition, E rarely had any bowel movements.  We were convinced he was constipated (a logical conclusion based on his lack of poop and apparent discomfort), but in hindsight it seems more likely that he didn’t have enough food being digested to make waste.  It wasn’t that he couldn’t get the poop out, it was that there wasn’t any in there to begin with.

Finally, I have learned that about one-third of patients with FPIES also have atopic diseases such as eczema (6, 110), which explains E’s skin issues.  I will say that as awful as the vomiting was and as upset as I was about his failure to thrive, I am so grateful that he never had any of the more severe symptoms such as extreme lethargy, hypotension, and shock, which occur in about 5% of patients (105).

When I read these descriptions of chronic FPIES, it seems as though they are describing E.  It seems so clear that this is the correct diagnosis for him.  I only wished we had realized it sooner.  If had known and had done more research, I think the second chapter would have looked different.  If I had known the stats concerning multiple allergies and grains, I would not have given him rice and oat.  I now know that approximately 48%  of FPIES patients react to more than one food (110) and 30% react to three or more (105).  After milk and soy, rice and other grains are the most common triggers (105).  About a quarter of those who react to milk and soy also react to one or more grains (110).  Further, 40-50% of those who react to one grain, react to another grain (105, 110).  It has also been noted that  those with solid food FPIES are more likely to have eczema (110).  Because of these findings, one group of researchers (105) recommended caution when introducing grains to children who react to milk and soy.

I feel like we were led somewhat astray by the recommendation to add rice or oat to E’s formula to help prevent the “spit-up” (which, by the way, I am now sure was actually vomit as a result of an allergic reaction).  If I had known about the high rates of reactions to grains (with these two being the most common), I would have avoided those longer and tried to obtain a clearer baseline after eliminating milk and soy.  So, yeah, in trying to help our baby, we actually made him worse.  On the bright side, these data do support our decision to avoid all grains for a while.

It’s now clear to me that so many of the things E was experiencing – the eczema, the sleep problems, the poor weight gain, the general discomfort, and especially the vomiting – were the result of FPIES.  Thankfully, this also meant that once the allergens were eliminated from his diet, he started to improve.

By the end of March 2013 his only source of nutrition was an elemental formula (which contains no proteins and is, therefore, hypoallergenic).  We had removed milk, soy, and all grains from his diet.  In April (8 months old) the vomiting stopped, his skin cleared up, and he started taking two good naps and sleeping through the night.  And I could breathe again.

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When E was about six months old (which is the recommended time frame), we were given the ok to introduce fruits and vegetables.  I decided that it would be safest for me to make E’s baby food.  I had never even imagined making baby food but I wanted the control of knowing exactly what he was getting and be sure that any contact with his allergens was avoided at every step in the process.  We followed the four-day wait rule, that is recommended for all children (not just those with allergies).  We introduced one new food at a time and offered it for four days before declaring it safe and moving on to the next food.

E loved everything we gave him!  It’s actually fairly common for children who have food allergies or other food-induced ailments to have aversions to food and reject new foods.  Not E! Before we knew it he was eating pureed avocados, bananas, sweet potatoes, pears, apples, carrots, peas, and the list kept growing.  We even started to add meats and eventually baked egg (in preparation for his birthday cake).  As he got a little older it was tricky to find good finger foods (the typical first finger foods are usually Cheerios or wheat/rice puffs – all primarily grains), but he did great with small pieces of bananas, avocados, and sweet potatoes.  We eventually found Happy Baby Happy Creamies, which are coconut milk-based fruit and vegetable melts.  These sweet treats became a favorite snack.

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They didn’t always make it into his mouth…

I spent the end of E’s first year perfecting my label-reading skills, enhancing my creativity, brushing up on my cooking techniques, and catching up on much-needed sleep.  I remained in constant fear of an anaphylactic reaction to dairy but triple-checking to make sure we had the epi-pen before we went anywhere became second nature.  E’s diet was limited but he wasn’t having any reactions.  As we approached his birthday we did another round of skin tests, all of which (even dairy) were negative, as were blood tests.  This was great news and a step in the right direction but not enough evidence to know for sure that his allergies were gone.  At least two negative tests are needed and an oral food challenge to know that he has officially outgrown an allergy.  So we allowed ourselves let out a little sigh of relief but didn’t let our guard down, just in case.

By the time we made it to August (and E’s first birthday) we had a lot to celebrate.  We finally knew what was going on with our baby and felt in control of it.  E was thriving, his weight had exceeded the 90th percentile, though he was still a little short (we’ll cut FPIES some slack and blame my short grandfather for that one).  He was starting to walk and was generally an adorable, happy baby.  We celebrated with a Curious George-inspired, dairy-free party complete with dairy-free, grain-free coconut-flour birthday cupcakes.  E had a blast and loved his cake 🙂

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*I’m going to attempt to infuse some research wherever possible.  There isn’t much out there but I think it’s really important to acknowledge what we do know and let it guide our decisions in managing FPIES (and any other illness, really).  Clicking on the numbers will take you to the journal article page with the complete citation for each number.  I hope this is helpful.
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4

“Happy Spitter” – The Beginning of Our FPIES Journey

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I’m a worrier.  I worry – a lot – it’s what I do.  I’m pretty good at it.  I know that I’m a worrier and I work really hard to keep it in check (but I’m not so good at that part).  So, keep this in mind as I recount E’s first four months of life.

E was born about 3 weeks before he was due and about 5 weeks after we moved from Florida to Pennsylvania.  Luckily we had a wonderful team of family members who helped us unpack, build furniture, and stock the nursery.  We were pretty much ready for his early arrival, except for the fact that the carseat wasn’t installed and our last childbirth class was scheduled for the day after my water broke.  But we had a crib and diapers and lots of adorable little clothes, so we would be able to manage.  Like any new parent, we were super-excited and had no idea what to expect.

I’m sure you saw this coming, but even in my haze of overwhelming love and awe at the human that we created, I found something to worry about.  I was concerned that nursing wasn’t going well and that he was losing too muchweight.  To be fair to myself, this worry was the result of the fact that nursing wasn’t going well and he was losing too much weight.  These are facts.  It had been my goal to nurse E for as long as it was possible and realistic for us.  I tried not to have unrealistic expectations related to breastfeeding. Other women in my family had difficulties nursing and with supply and I was worried that I might too. But I was determined to try.  The first time I nursed, in the delivery room, E latched on immediately!  I was so relieved, which only led to more intense frustration hours later when the nursing stopped going well.  I worked so hard at it, I saw several lactation consultants and talked to some very patient postpartum nurses while we were in the hospital.  We were doing ok with the nursing by the time we were supposed to be discharged but E had lost a significant amount of weight (just over the acceptable 10% weight loss).  The hospital pediatrician wouldn’t discharge us until we confirmed that we could get an appointment with our pediatrician the next day.  At that appointment the pediatrician strongly suggested that I start supplementing with formula.  She also referred me to another lactation consultant who was great and supportive and had us doing all kinds of crazy things to support breastfeeding while supplementing with formula.  By his weight check at 4 weeks he was back to his birth weight.  We breathed a sigh of relief and stopped supplementing.  I could stop worrying about that (for now).

Which was good, because I had already found something else to worry about.  As a newborn, E had a tendency to spit up.  He spit up a lot.  Like all the time.  And not just a little dribble of spit-up, but an amount of spit up that often required an outfit change, for him and everyone in his general vicinity.  I never left the house without two or three changes of clothes for him and at least one change for me.  I still feel a little guilty because he spent about 6 months in one-peice pajamas.  He rarely wore those adorable little outfits that I was showered with prior to his arrival.  It just didn’t seem worth the trouble of getting him into a complicated outfit that would be drenched with spit-up within the half-hour.  My parents and in-laws learned to wear old clothes when they came to visit and cuddle with their new grandson.  It was a given that they would leave covered in vomit.  I didn’t get out much but when I did I usually had the pleasure of mopping spit-up off of the floors and furniture of our hosts.  I can keep going but I don’t think you’ll ever comprehend the amount of spit-up that came out of this adorable tiny creature.

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E slept in his “formal attire” one night after spitting-up on every other piece of clothing he owned.

I was a new mom but I knew that babies have a tendency to spit up.  Whenever I brought up the amount and frequency of the spit up, I was assured that spitting up was completely normal and we had nothing to worry about.   Still, it didn’t seem right and I started to worry.  When we reported it to the pediatricians they asked if he spit up after a meal.  Yes.  He spit up after a meal, and before a meal, and during a meal (which is great when your are breastfeeding).  He spit up all the time!  Granted, he was a newborn who was a bit of a lazy eater.  He nursed every two hours and usually fed for about an hour, so it was almost always after a meal. I was told (and read) that the amount of spit up always seems to be larger than it really is.  I was told that he was a “happy spitter,” a baby who spits up just because his esophageal muscles were still weak and that it wasn’t bothering him.  I was told not to worry.

I worried.  I worried when we put him in the cradle.  Is back really best for babies who spit up so much? Couldn’t they aspirate? I read study after study until I was convinced that it wasn’t a concern.  I still worried.  I didn’t believe that E was a “happy spitter.” I’ve taken a lot of psychology classes and read a lot of books on happiness and depression, and I was pretty sure that spitting up didn’t make my baby happy.  He cried a lot.  He arched his back.  It woke him up and prevented him from sleeping.  In fact, I started to recognize the pre-spit-up grimace that provided me with a couple of seconds of warning and a fleeting opportunity to reach for a burp cloth before the eruption.  If it didn’t bother him, why did he grimace? I worried that something else was wrong.

Then there was the poop, or lack thereof.  E would go several days without pooping, almost a week.  And he just seemed so unhappy.  He was squirmy, like he couldn’t get comfortable and acted like he was in pain.  We assumed he had awful gas pains and was terribly constipated.  We called the doctor frequently.  I’m sure our name was on some sort of list by the phone.  But we were worried.  We didn’t know any other baby who pooped so infrequently.  The doctors weren’t worried, but I was.

2 months

E’s 2 month well visit was traumatic.  He was significantly underweight.  At that visit he was in the 0.37 percentile for height and 1.25 percentile for weight.  For the first time since we left the hospital, I felt justified in my worry.  I could tell the doctor was also worried.  I was devastated but I had a whole list of things I wanted to discuss with the doctor.  I was really worried about the lack of poop and the discomfort that he seemed to be in as a result.  I was worried about spitting-up.  I was worried about his baby acne, which had spread from his face to his neck and chest and seemed to be getting worse.  The doctor prescribed Zantac, believing the spit-up may be acid reflux.  He prescribed a topical steroid, explaining that what we thought was baby acne was actually moderate eczema.  He ordered an upper GI to confirm that the spit up wasn’t an indication of a blockage preventing him from digesting his food.  Most significantly, he suggested that we go back to supplementing and go back to the lactation consultant.

The upper GI was normal.  His spit up was determined to be normal.  Once again, he was labeled a “happy spitter.”  The lactation consultant determined that he was latching well but that my supply was low.  She recommended that we continue to nurse but also continue to supplement.  I’m still surprised that the lactation consultant was so encouraging of supplementation, but that isn’t the most important thing she said to me.  She told me that it seemed like E had a milk allergy.  She noted his spitting-up and his eczema and strongly encouraged me to explore the possibility of an allergy.

At that point I tried to limit my dairy intake, but I didn’t cut it out.  I didn’t really believe that milk was the problem.  But I brought it up at our next weight check (E was getting weighed every 2 weeks).  I also brought up the fact E was still spitting-up a lot.  This time we saw a different pediatrician in the practice.  She switched E to omeprazole, saying that Zantac doesn’t work for everyone.  She thought the lactation consultant may be on to something and suggested switching him to a soy formula.  By the way, he was gaining weight at this point.  phew.

By now I was getting really worried.  My little man was still spitting up a lot.  I felt like no one really understood what “a lot” meant.  That was when I remembered – I’m a scientist. I needed to quantify “a lot.” We started keeping track. I put a post-it on the coffee table and made a tick mark every time there was spit-up. One day we had 27 tick marks! We shared our data at E’s next pediatrician visit.  Finally, we got our point across!  The doctor admitted that this was an usual amount of spit-up.  We also reported that we had to stop the soy formula after a couple days because the spit up didn’t improve and E became really constipated. (We now understood that what we were seeing before was not constipation. The poor kid wasn’t pooping because he didn’t have enough in his belly, between my low breast milk supply and all the spitting up.)  The doctor heard us! She had us switch E to Nutramingen, a partially-hydrolyzed formula and referred us to an allergist.  In the meantime, I decided that it was time to start weaning E from nursing and focus on only formula.  He wasn’t getting that much milk from me anyway and I wanted to know for sure what proteins he was ingesting.

At the end of December 2012 we had the allergist appointment.  It was the most thorough medical appointment I’d ever attended, including the ones that I led (which I always thought were incredibly thorough).  The doctor took a detailed history, which suggested to her that he was allergic to dairy and possibly soy, but conducted a scratch test of the top 8 allergens to be sure.  I remember waiting for the results and talking to my husband about what a dairy allergy meant.  His cousin has a dairy allergy that had required hospitalization but we still didn’t understand the severity of E’s allergy in the moment.  We thought surely a dairy allergy wasn’t the same as something like a peanut allergy.  When the nurse came in to read the scratch test she initially thought they all were negative.  However, when she got out her ruler she realized that the dairy scratch was slightly positive.  The doctor came in to discuss the allergy, along with an emergency aciton plan and we were trained on how to use an epipen.  That’s when it hit us that our son had a life-threatening allergy to milk (of all things!).  The doctor prescribed an elemental formula, which doesn’t have proteins in it at all, just amino acids.  She told me I could continue to nurse but that I should cut dairy from my diet.  She seemed pleased when I told her that I had already decided to stop.  I felt validated in my decision to wean and, as scary as it was, we were glad to have an answer and a plan.

That’s the difficult thing about worry – sometimes there is a real reason for it.  These four months taught me to trust in myself and my gut.  To not work so hard to conceal and ignore my worry.  To believe that I knew what was best for my son, and to always remember my strengths and my ability to collect data.