Myra's Diagnosis: Food Protein Induced Enterocolitis Syndrome (FPIES).

Some of you may know, some may not, that Myra has been struggling with some food-related issues. For your reading pleasure, I've divided this into a few parts: the history/symptom part, the diagnosis part, and the "what the heck is FPIES" and "how do you deal with it" parts. Many of you are already familiar with one or more pieces of this puzzle, so I'm trying to avoid boring you with redundancy. (Note: anything that is a link will take you to a site with more explanation/information since not all of these terms are self explanatory. For up to date information on Myra's food trials, safe foods, recipes, etc. click the "Myra's FPIES Foods" tab at the top of the page.)

The history:

Sleep in the early months was non-existent, and not the "I just had a baby and I'm exhausted" kind of non-existent. It was the no sleep, or maybe two 30 minute spurts a night for weeks on end, kind of non-existent.  She was happy during awake times, but when she was supposed to be sleeping she would always (ALWAYS) wake up 30-40 minutes later obviously in pain (a specific whiny cry, curling up as if her tummy hurt). Over and over. I could put her down "drowsy but awake" or, if she fell asleep in my arms, I could put her down and she wouldn't wake up right away.  She sucks her thumb so she wasn't waking for a pacifier. I was miserable and seeing the world, and my new baby, through awful, sleep deprived glasses. I didn't like my new baby (until I'd get a couple hours of sleep, then I was quite smitten).  The baby we waited SO long to have.  Then I felt guilty for having all these awful feelings, and so on and so on.  I took quizzes to make sure it wasn't postpartum depression.  Nope, I was just so. freaking. tired.  Don't get me wrong, it was certainly not easy on Trevor either, but I did my best to let him get sleep (very interrupted sleep, I'm sure) so he could be alert for his commute and school. And, of course, this affected us both in ways beyond the sleep deprivation.  After trying everything else (ok, except co-sleeping, but that's just not for us) and seeing Myra's doctor and a pediatrician who both said she seemed perfectly healthy, I decided to eliminate dairy from my diet (Myra is exclusively breastfed) even though Myra didn't show the "typical" symptoms of a dairy sensitivity. After three weeks (how long it takes for cow's milk proteins to work out of my system then Myra's), she was a different baby. She started sleeping 12 hours straight, no wake-ups, for six weeks!  I was really hoping it was a coincidence, so we tested dairy twice by giving her milk I had pumped before changing my diet. Bad idea, that confirmed it. No dairy!

(Believe it or not, this is the somewhat shortened version...) 

After those six weeks and a dairy exposure, she started waking at night again. The couple rough nights following the dairy exposure passed, but her sleep didn't return to how it had been. We thought maybe a growth spurt... teeth... milestone... then we started solids so maybe that. Finally we came back to "something is wrong...again." We had taken a casual approach with introducing solids (other than avoiding dairy), using baby led weaning and not following any specific timeline for each new food. Everything seemed fine, she was eating a variety of healthy foods, enjoying it, and we didn't really connect the dots with the night wakings and other more subtle symptoms creeping back in...yet. Early September we gave her a couple graham cracker sticks. After a few hours (3-4) she started vomiting. She threw up six times in two hours until she had thrown up bile and was ultimately dry heaving. She was, of course, exhausted afterwards and it was also bedtime. I got her to nurse a bit and put her to bed. She seemed fine the rest of that night and the next day. We figured it was either a coincidence or the graham cracker and we were hoping for coincidence.  Skip ahead through some rashes, sleepless nights, and swelling episodes and we figured she had some "traditional" (or IgE mediated) food allergies, so we took a step back and started over with 4 day food trials. I took her back to her doctor who did a blood panel which indicated she may have some allergies, and referred us to an allergist. We couldn't get in for that for 5 weeks, so we just kept plugging away. Things went okay, meanwhile her chronic symptoms started coming back (more on that below), but she did have three other vomiting episodes. None of those were quite as "bad" as the graham cracker, but once was from green beans. Seriously?! Green beans! 

These are the chronic symptoms we noticed starting in August and really intensifying in September:

Sleep disturbance (understatement of the year).

Big, loud farts that smelled horrible, like sulfur.

Constant diaper rash.  Constant.  Mostly only where her poop had contact.

Back arching at night time feedings.

Head banging (in her crib, sometimes elsewhere).

Body tensing/stiffening and clenching fists until trembling.

Coughing in her crib.

Lymph nodes behind ears always swollen.

A few levels from complete blood count increased in April and September (when I brought her in for these issues and she wasn't sick with anything else), specifically platelet count.

Rash on the rest of her body, little dots, mostly on her torso and neck.

Who the heck knows what normal poops look like after all this, but hers were definitely not normal at times.

Crying and tensing with diaper changes.

Little, juicy burps when I would pick her up out of her crib.

Despite all that, a very happy baby! (Except at night/sleep times sometimes)

Each of those things in themselves could be considered a normal baby thing, but combined, they paint a picture of something else happening.

The diagnosis:

When our allergist appointment finally arrived, we went through the symptoms, timelines, etc. with him and discussed allergy testing options and he brought up FPIES (Food Protein Induced Enterocolitis Syndrome). Since her reactions were not immediately following ingestion and didn't involve respiratory problems, he seemed to be leaning more towards FPIES than traditional allergies. FPIES is a non-IgE mediated food allergy, so if that were the case, she would not test positive on allergy testing (although it is possible to have both). We decided to do skin prick testing for dairy, wheat, and soy and they were all negative. We then did blood testing for the same three things, also negative. He wanted to consult a colleague who had more experience with FPIES and get back to us. Meanwhile, Myra's chronic symptoms had gotten so intense I decided to try eliminating soy from my diet and we took her off all solids until we could figure this out. Both the allergist and Myra's doctor were aware of these dietary changes. .....Three weeks (and two emails from Myra's primary doctor) later, we heard back from the allergist we saw. He spoke with his colleague and she "did think it could definitely be FPIES." We were to avoid feeding Myra dairy, soy, oats, wheat, and rice. That's it. (Remember, she vomited after eating green beans and that wasn't even included in her no-no list...) Thankfully, we had already decided to move forward with other options and made an appointment with an allergist in Minneapolis who has experience with FPIES, Dr. Stillerman. He's actually listed on the FPIES Foundation's website. Also, Myra's doctor gave us a referral to a pediatric GI specialist, Dr. Grothe, to rule out any other GI problems (and/or confirm FPIES). 

When Myra and I met with Dr. Stillerman (allergist with FPIES experience), we almost exclusively discussed her acute symptoms (vomiting mostly), and only touched on her chronic symptoms. He agreed with the two allergists that it, indeed, did sound like FPIES. He sent a letter (below) to her primary care doctor and also gave us a copy to keep with her at all times (more on that later). We discussed a plan for reintroducing solids and discussed different formula options since Myra seems to react to things through my breast milk. To be clear, Dr. Stillerman did not suggest I stop nursing. (Also, to be clear, I have breast fed Myra exclusively for 10 months now, but I have absolutely no issue with people using formula no matter what the reason.  Science is a wonderful thing, too.) "Normal" formula most often has either a dairy or soy base, and contains the full protein chains as-is. Some more sensitive formulas (Nutramigen or Alimentum, for example) have partially broken down those protein chains to make them easier to digest. Elemental formulas (like Neocate or Elecare) have completely broken down the protein chains into individual amino acids.  Since FPIES has to do with food proteins, the elemental formulas are the safest bet when choosing a formula. There are certainly babies who do well on others depending on how sensitive they are and what triggers they have. Cost is most certainly a factor in determining which to try.  Together, we developed a plan for starting solids again as well as an emergency plan if Myra reacts to a food.

A few days later was our appointment with Dr. Grothe, a pediatric GI specialist. We thoroughly reviewed Myra's symptoms, both acute and chronic, beginning with the sleep disturbance and dairy elimination from early on. She explained that if there were some sort of anatomical GI issue (reflux, a twist in her intestines, etc.), Myra's symptoms wouldn't change so drastically based on diet, touché. Based on Myra's history, Dr. Grothe agreed with the diagnosis of FPIES and stated she would document it as such in Myra's chart. It may sound strange, but I can't describe the relief I felt hearing she would have an "official" documented diagnosis!  Dr. Grothe was supportive of the plan we had in place as well as the switch to an elemental formula. (By now we had decided we would gradually do the switch after our first food trial which we had already started. Formula will need to be a trial in itself.) She made sure we had access to Mayo's patient portal so we could easily contact her if anything changed.

Note on the formula: while I don't care how others feed their babies, I do understand that it is a very personal decision for many families.  There are moms who have found a way to work around FPIES, even with triggers through breast milk, and continue to nurse.  This is possible for us, but after weighing all the pros and cons, we have decided to trial a switch to formula and really hope it goes well.  Breastfeeding was hard at first, but it has become so easy.  I have found many ways to still eat the foods I like with substitutions when necessary, but the change does come with stress.  Considering the emotional toll as well as the effort it takes to get enough calories for a nursing mom each day, formula is the right decision for us right now.  That doesn't mean it will work and that doesn't mean it's the right decision for everyone, FPIES or not.  This can be a very hot topic in the parenting world (which blows my mind), so I thought I would just share our logic on the issue.  Do your homework then make your own decisions for your own family, adjust as needed-best parenting advice I've ever read!

Update: We did switch to Neocate Infant formula and Myra has done absolutely wonderful on it.  I continued to pump for a couple weeks in case it didn't work out, but that in itself was so exhausting (mentally and physically) it didn't last long.  Since there are no proteins in the amino acid based formulas and FPIES is a protein disorder, the chances of a reaction would be rare.  If she did react, we were prepared to find alternatives, but at that point continuing to breastfeed was not one of our options.  We noticed improvement within those first couple weeks and she continued to sleep better and show less and less of her chronic symptoms as time went on.  As of early February 2014, Myra has 4 safe foods.  I will keep a current list of her foods on the FPIES tab at the top of the blog page.  I do sometimes question if maybe we're just over reacting or reading into stuff so much, but looking back at how awful our nights were, the vomiting, screaming, zero sleep compared to now, it's night and day difference.  I have a hard time saying she's such a different baby because she was, somehow, still so happy all the time, but the sleep and other symptoms were absolutely present.  There is none of that now unless she is in a food trial that is not going well.  The big difference there is we know the cause and can stop it much sooner, even if it means stopping the problem food and waiting a day or two, that's a huge difference from months ago when we didn't attribute it to food at all.

What is FPIES:

FPIES, said F-Pies, stands for Food Protein Induced Enterocolitis Syndrome. It is a type of food allergy, but different from traditional food allergies. I'm not going to reinvent the wheel here. This letter is taken from the IAFFPE website and it is the same letter Dr. Stillerman personalized and sent to Myra's doctor. We also have to keep a copy with Myra in case of an emergency.

Myra has a food allergy called Food Protein-Induced Enterocolitis Syndrome (FPIES). This is a type of allergy that usually does not result in typical “allergic” symptoms such as hives or wheezing, but rather isolated gastrointestinal symptoms. The symptoms of this type of allergic reaction include repetitive vomiting that may not start for a few hours (e.g., two hours) following ingestion of the food to which the child is allergic. Even trace amounts can trigger a reaction. There is often diarrhea that starts later (after 6 hours). In some cases (~20%), the reaction includes hypotension and lethargy. The treatment is symptomatic and can include intravenous fluids (e.g., normal saline bolus, hydration) and steroids (e.g., Solumedrol 1-2 mg/kg) for significant symptoms. The latter is given because the pathophysiology is that of a T-cell response. This information is being given so that this could be considered in the differential diagnosis for this patient in event of symptoms. Of course, this illness does not preclude the possibility of other illness (e.g., infection) or even other types of allergic reactions leading to symptoms, so it is up to the evaluating physician to consider all possibilities. Similarly, the treating physician is encouraged to pursue any other treatments deemed necessary (e.g., symptomatic such as epinephrine for shock, antibiotics for presumed infections, etc.)

What do we do about it:

There is no cure or treatment for FPIES. It's unknown what even causes it. Management involves very cautious, precise food trials (two weeks per new food, every single thing-even things as simple as sugar or cinnamon), identifying safe foods and avoiding trigger foods.  Even the smallest amount can cause a reaction and reactions can be very severe (some vomit to shock). Many children outgrow it. There seems to be a fair number who outgrow at least some triggers by age three, more by age eight, and some who don't outgrow it at all. There is no way to know except food trials and, when deemed appropriate, challenging trigger foods, sometimes in a clinic or hospital. Dr. Stillerman really stressed the importance of calling for an ambulance if Myra starts vomiting since we don't really know how bad her reactions might be and we can't give her the care she needs while driving.  For food trials, we will likely only give her the new food for breakfast and/or lunch so we can monitor her since the reactions are delayed. Once she "passes" a food, we will include it in her diet for any meal. With this first trial, I only gave her a few bites in the morning for the first two days then increased the amount on the third day. If she vomits or has diarrhea, we will call it a fail and consider it a trigger food. If she shows some chronic type symptoms, we will give it a few days to make sure it isn't a coincidence before considering it a failure. We're keeping a detailed journal with times foods are given, sleep patterns, poopy diapers, and other relevant symptoms during trials. Hopefully, after a few trials, we can make sense of patterns so we can pick up on failures early. Or, better yet, hopefully she doesn't have many triggers and this all goes smoothly! For solids, we are starting with foods she has already had and to which she didn't seem to have major reactions. Often trigger foods are found after the child has had a break from it, so many people actually build a 2-3 day break into their trials. So, hopefully, we'll notice sooner than later if something isn't going well with these first few foods. If you've made it this far, you'll be glad to know you're part of the plan, too! Educating others, especially people who will be around Myra, is a HUGE part of our plan. People need to know how serious this can be, even from a very small amount of a trigger food. Myra can't have anything other than her safe foods (of which she has none right now) because we need to be in complete control of her food trials. If she gets her hands on a trigger food (or even an unknown food/substance) during a trial of something else, it will completely negate any progress in that trial, not to mention make her (and us) miserable. This isn't some paranoid, silly, new parent thing-it's very real and it's become a big part of our lives. We have to be careful even in our own home. Trevor washes his hands after eating and before touching Myra (I don't eat Myra's known triggers since I am still breast feeding, but I'm still careful, too!). We have to vacuum often so Myra doesn't eat leaves, clumps of dog hair, etc. (believe me, she tries!). We can't risk anything getting into her system because we have no idea if it would affect her. Of course, I'm human and wonder about things like "what kind of ingredients can we include in some sort of a birthday cake two months from now" and "will she feel left out at meal times," but those things will sort themselves out in time, so I just try not to get ahead of myself.  I've already heard a lot of "I could never do all of that," and similar statements. Just like military life (although that's more so a choice than this), you just do it. We play the cards we're dealt. It's not always easy, but it's not like Myra is naughty or a burden. She's a beautiful, happy, smart little girl with a big personality. And I never thought I would feel such joy being able to feed my baby FOOD, but since getting an actual diagnosis and starting solids again, feeding her feels so GOOD! Bonus--similar to military life, I've already met some totally awesome FPIES parents. They're full of information and experience, they ask what's going on and truly listen, remember when our appointments are and ask how they went, and offer excellent advice and support. Some have given us tips for working with our insurance company, navigating various clinics, etc. In some ways, yes this kind of takes over life some days, in other ways, it is what it is but it's just how we have to do food. With the right understanding from friends and family, it doesn't have to be a big deal.  The food trials and journaling probably sound intense, but that part really isn't so bad so far.  The biggest hurdle will be failures.  We haven't had a vomit to shock episode, and I pray that never happens, but let me tell you these chronic symptoms are HORRID.  Things could always be worse, it's all about perspective, but when you're on your eighth night in a row of a baby who screams no matter what you do and has a diaper rash so bad she scratches herself until she bleeds, you're not really thinking about "perspective."  

Truthfully, I could go on and on about the emotional toll this has taken, at times, on all of us.  Again, if you've made it this far, I imagine you can guess.  I'm not a label person, but this official diagnosis (and game plan) has already lifted weight off our shoulders.  Hopefully it will help others (medical professionals included) understand how serious this can be.  It will help us get the very expensive formula (that hopefully she tolerates) costs covered.  And, yes, it's validating that we're not crazy or dramatic or anything like that.  FPIES is a real thing and Myra has it, a real explanation for all these symptoms.  And if she ends up in daycare or still has it when she goes to school, an official diagnosis will likely be very helpful in those settings to ensure proper precautions are taken.

Please remember there is not a whole lot of "science" behind this, it's mostly observation.  The diagnosis is clinical based on medical history.  The symptoms and severity can vary greatly by food and baby.  While this may be helpful if you're running into some of the same issues, please don't use it as a replacement for working with your health care team.  It took bringing Myra in many times until the pieces of the puzzle started to make sense.  Blood work twice for no real reason, just to see if anything stood out.  And a lot of documentation on my part.  

Now, I'm off to relax and hopefully get a good night's sleep!  As always, any questions about this or anything else on the blog, PLEASE just ask.  I'm an open book.

 This was taken before we suspected any food issues beyond a dairy "sensitivity," but still an adorable food-related photo :)