Rotation Diet, Protein Intolerance and FPIES?

FPIES is on the spectrum of Protein Intolerance.   Much like typical IgE allergy has a spectrum of reactions, from a scratchy throat, to flushing, to hives all the way to anaphylaxis.   FPIES is anaphylaxis of the gut.  It is a severe protein intolerance.   And, just as with typical allergies....some kids have just one IgE allergy and some have multiple.   With Protein intolerance's, some kids have just one (milk or soy or milk and soy- referred to as MSPI) but many kids have multiple intolerance's.   With FPIES, some kids have just one trigger- milk or soy, or rice and oats; but again many have multiple.   And then still, many kids have all 3- Protein Intolerance, FPIES, and IgE allergies.   The bridge where they all cross can be confusing.  

With protein intolerance's, you may be able to have a food if it is cooked or the oils or in low threshold amounts- the body can "cope".    Protein intolerance kids can often also handle a rotation diet.  A rotation diet can avoid a "build up" of the proteins while still keeping the foods in the diet for their nutritional value, and while also building up the bodies tolerance of the food- training it, with each small incremental dose that the food is foreign but safe.  With each dose, the body building more tolerance mechanism and less attacking mechanisms.

My question remains: is this safe in the FPIES child?   The FPIES child with protein intolerance that the body is already primed for FPIES trigger attack mechanisms.   Is the body so efficient at producing those mechanisms of attack...mechanisms designed for protection against viruses and toxins but attacking simple food proteins in the FPIES child....that every intolerance is vulnerable for FPIES? 

It is not possible to include known triggers in a rotation diet, but could it work for trialing foods?   Little man seems to build up intolerance's to foods at day 3-4 (or beyond); his body recognizes it as unsafe almost immediately (we can always look back at logs and see in hindsight that nothing was coincidental, that all symptoms eventually led to something) but we press through because we can't fail a food at the first signs of hiccups, or irritability or some disturbed sleep....when all these things can just be a coincidence and we would have no menu!   And yet, none of those symptoms have ever been a coincidence, and his intolerance's build until we have to pull the food as it is unsafe, causing too many off-baseline symptoms or behaviors, or it has caused a trigger reaction.   It would be unsafe to keep a food in the diet (even in rotation) if it is causing symptoms of off-baseline because the waters would get really muddy -- how would we know which foods were causing which symptoms and which foods are building symptoms and which ones are causing 'just' adjustment symptoms?  

With an FPIES diagnosis, you are given a set of instructions for food trials at home, there are variances to these schedules but they are all similar in that they are small doses over long periods of time.   The reasoning behind the slow introductions is to stop before the symptoms build to the full blown fail....the tricky part is finding that line of "are these build symptoms" or "are these body adjustment and/or intolerance symptoms?" Keeping a food and symptoms log has been essential for this; although many FPIES children react from the first dose of the food trial.   A second reasoning behind the slow introductions for protein intolerance is to teach the body "foreign but safe".

My goal has been, of course, to do this for Little Man.   To introduce foods in his diet (in his muffins that he enjoys so much would be ideal)  slowly, 1 tsp at a time....to build his tolerance while monitoring closely for symptoms. BUT- without a base diet first, or with missing micro nutrients....we aren't at that place yet to be able to execute this ideal FPIES scenario for food introductions.  

So, that got me to thinking about a rotation diet- would a rotation diet of trial foods help us to teach his body foreign but safe without overwhelming it and pushing him past his tolerance levels?   Would it make it less daunting to take breaks from a food beginning to cause symptoms?   Little man seems to do ok with foods for the first 1-3days, and then intolerance's really begin to add up.  Is this because they are closely related to the proteins he has had reactions to (grains and dairy?).   This is why I have been picking foods he hasn't ever had before, or that aren't related to his FPIES triggers.    Millet is a seed but it is in the cereals food family so we were taking a risk on it.    Millet is still a mystery.

To execute a rotation diet, it would need to be closely calculated out  - trial a food for 2-4days, give the body a break (3-5days)- reintroduce....teaching the body- foreign but safe (building the Th3 response to override the Th1 response mechanisms?). Similar to oral immunotherapy that is being found successful for some allergies?

A few important FPIES considerations:
1. It would be critical to not go too long between re-exposures or the body will 'forget' it's tolerance mechanisms.
2. Also critical to not push if reaction symptoms persist or build (body is building attack/Th1 mechanisms instead of oral tolerance/Th3). 
3.  Essential to stay at dose tolerated (or less) if symptoms begin to build.
4. To return to ONLY safe base diet during the "break" if there are any off-baseline symptoms noted (and not rotate to new trialed food). 

In my head, on paper, this works....with Little man it hasn't, yet....

Little man and his Mudpies

Mudpies = big brothers.   Little man has been enjoying his big brothers, even more now that he is not in pain or too fatigued to play.   Big brothers appreciate being able to have their little brother at home (and not in the hospital).   Little Man had an appointment follow up with the GI doctor yesterday, his biggest brother was aware of it and also was aware that it might mean a hospital stay for him.   He was pretty happy to see little man be at home yesterday when they got home from school!  

Little man may need an alternative form of nutrition if we can't get him over this hump we've been stuck in for months.   Moving away from the amino acid formula's stopped corn from assaulting his system and took away his daily pain and random vomiting.  He has been eating better (going from 20-30oz.Neocate to 50-60oz Hemp milk formula) and we have been able to give him a few safe foods (millet puffs and peaches) to practice his oral motor skills and give him a "snack" while we are eating, so he feels included, and we can enjoy mealtimes as a family a little more again.   We only give him his millet puffs and peaches in very small amounts, we have had to build up to even these very small amounts.  We are wanting to push forward with more food trials to build him a tiny menu....but we keep hitting roadblocks.   The biggest roadblock right now has been that he needs more nutrition to round out his current formula -- micronutrients.   For now, we have checked all his blood/serum levels of these mirconutrients and his body is holding.  However, the upper endoscopy biopsy results from last week show that he now has villous atrophy, along with continued/re-flared patchy inflammation in his intestines.   Inflammation will lead to poor absorption/utilization of nutrients but villous atrophy will lead to zero absorption of nutrients.  Despite these findings, Little man has been doing well since his release from the hospital 12 days ago.   He has had a few nights of some minor sleep disruption and a 3 mornings of some fussiness (he is cutting more new teeth), but otherwise he is maintaining a better baseline than we have seen in months!!  He amazes and mystifies the doctors all the time.  He is sick looking and acting, and his labs do not match; then his biopsy says he is sick and his demeanor doesn't match.   He is a puzzle.

With villous atrophy findings, there is even more urgency to be assured he is getting all his nutrients -- not only getting them but absorbing them.  Yesterday, his weight was up a gram.  This is good....he's not losing.   His demeanor is good, he plays actively, sleeps ok, eats well.....but he needs a turn around point.  If soy was the culprit to the inflammation and atrophy; we have removed that 16days ago and he is healing.   If the atrophy is from something else -- a slow effect of a trace protein (natural flavors ingredient in hemp milk? VitE derived from sunflower oil in protein powder? millet puffs?).   Villous atrophy is most common in Celiac Disease.  Celiac disease is a severe gluten intolerance where one crumb of gluten protein will cause this atrophy to begin.   FPIES sensitivity is the same as Celiac- where a crumb or a trace amount will set off inflammation; although atrophy is new to Little Man.   Atrophy could provide another clue to why little man's FPIES is so complex- it may indicate a protein enteropathy that is co-existing with his FPIES....because of his very sensitive and immature gut.   Villous atrophy also can come from malnutrition.   His labs have all looked good- indicating he currently is getting adequate nutrition (although we know ideally we need to improve it).  Malnutrition does not mean what that it comes from what is taken in, but also from what the gut is doing to the body (malabsorption of nutrients taken in, despite the diet being adequate).   We may never fully know what has caused this and the main goal right now is to stop it.   Only time will tell.    If his gut heals (he gains weight and labs continue to look good) - we can hopefully assume his atrophy has healed, although he may have to have another scope to verify before going to next steps.   If he does not have good weight gains over the next week, we will have to consider alternative nutrition (parental nutrition, feeding through a vein).  TPN is appealing in that it would finally provide my little man with every nutrient his body needs -- nothing would please a mothers soul more than to know her child is being nourished...it is the basic need of a mom.   TPN would mean gut rest.....a wipe-the-slate-clean sort of thing.  His gut could rest, heal, and his body would be nourished.   However, it runs it's risks....infection at the site being the biggest risk, having to re-teach him to take his bottle after a minimum of 2weeks not being able to (except for water) and then ending up with a G-tube if he does not is a very real risk.   With these risks in front of us, and with Little man playing and happy beside us -- we elected to give him just a little more time.    He appears to be healing. 

We have always let him guide us for his best treatments through this and I have done my best to be his voice.  

So, coming home from the appointment was a good thing and his big brother knew that- he greeted him home from school yesterday afternoon with a big hug.   They all played together all evening and now this morning....they appreciate having him home.  Little man enjoys being home.   We strive to maintain a quality of life for Little man, and his brothers, mudpies. 

With the holidays approaching, I typically do a lot of baking.  My boys enjoy baking with me and it is an activity for me to share with them.   Every year we do a Christmas village, with graham cracker built houses and candy filled streets....each year I let them pick out the candy that will create this village.  It has become a tradition they love and look forward to (they have asked if they can do a 4th of July village too so they have a summer one too!).    This village sits on the table for a few weeks during the Christmas season, as a decoration and a snack!   It is filled with food.....food that Little man can not have....food that would be poison in his body.....what do we do this year?  How do we make this compromise so his brothers do not feel left out from their traditions while we keep Little man safe?  I have been thinking about it for weeks, trying to come up with a fun idea.   We discussed it last night at dinner.  Little man's big brother (mudpie #2) came up with a great idea!  A Lego village!  A non-food, safe, but still very fun alternative!!   I was so happy and relieved, but also so proud.   It didn't even phase them that they would make this sacrifice and compromise for their little brother.   We're in this together....

But I've never heard of a child reacting to an elemental formula....

First visit with Allergist #4 was yesterday, it went well and we now have an allergist on our team at home, this will help manage his illness from day-to-day.

Allergist #1 was sure FPIES was not what was ravaging our son's body because he had not ever experienced full shock- even though the research shows that "only" 20% of kids do go into shock.   Although, she also admitted that she has only diagnosed one case in her career, and that case is quite severe.  I have actually since "met" this mom (online) and yes- her daughter's case is quite severe in her reactions; but otherwise their FPIES course are similar.   So, on to a 2nd opinion.  I have to say here, that it is funny to me that doctors are surprised when you ask for, or seek out a 2nd opinion.   A good doctor would encourage it...would admit their humanity and that they simply can not know everything; some other doctors are challenged by it...what did they miss?  I respect the doctor who admits their humanity, that admits they don't know everything but are ready and willing to learn.  With their training, experience and expertise; they can catch up fast on something new.  I caught up fast- because I live this day in and day out....I am gaining more experience than any study or research article could ever quantify.

Allergist #2 had more experience with this condition,and conditions similar to it; and was able to recognize our son's FPIES immediately by symptoms presented in his history.   He was also able to recognize a likely corn trigger intolerance....because of his experience.   "There is a small percentage of kids who react to even the elemental formula's and it is unfortunate because it makes it very difficult"....I can attest to that! 

Allergist #3- confirms the FPIES once again and gives us a great empowering statement for this difficult clinical diagnosis.  "He has FPIES, this has been confirmed by two allergist, here is some information if you would like to learn more"..... So, on to Allergist #4....and back to the clinic we started at- in our home. 

Allergist #4 wants to be part of our "team".  I've spoken before about how FPIES is best managed by a team- GI, Allergy, Nutrition,Pediatrician, parent (a good social worker would help too- and I'm working on that).  I am encouraged that he wants to be a part of the team, wants to be a support for us and a resource of our pediatrician to help us manage this chronic illness that has become complex for little man. 

A struggle we still have is the fact that we no longer have little man on an elemental formula, and will not be putting him back on it; instead we have him on a formula that I make.   It is adequate in calories, carbohydrates, protein, fats, and macro nutrients but it is lacking in some micro nutrients....nutrients that should be filled in by a diet.   But, we have yet to find a diet little man can tolerate in large amounts to contribute to his nutrition. The "easy" answer to the doctors is: put him back on the elemental formula....because after all, "I've never heard of a child reacting to an elemental formula".  

I am in nutrition- a dietetic technician, I am Registered- which means I must maintain a certain amount of continuing education yearly.   I also am practicing- which means I have a job, a job where I gain more knowledge from my experience.   I attend seminars on GI and nutrition and allergies.  I have special interest in GI, allergies, and pediatrics.  I had never heard of FPIES, and I certainly would not have suspected it to be such a severe and sensitive condition.....but that doesn't mean it didn't exist.  It meant simply that I had something to learn. 

Many will say that they have never heard of infants being allergic to breastmilk, and yet many infants are....for multitudes of reasons, some still unknown.   If a baby can be allergic, or sensitive, or reacting, or not thriving on breastmilk-- that is made from God....Why is it such a stretch that an infant can be allergic to something made by man?   There are many additives in formula.   The elemental are designed so that the proteins are broken down to single amino acid (something otherwise the body has to do).  So, instead the body does not have to break it down- giving it little, if anything, to react to. It simply absorbs it, providing the nutrition the body needs.  But, the formula doesn't just contain amino acids.  It contains fats and carbohydrates (in the form of oils and sugars).   My little man was reacting to the sugars - he has a sugar intolerance but it was more than that, he also has a corn trigger.   The corn syrup solids are strained down to remove the proteins but there is chances for trace proteins to remain.   FPIES triggers are highly sensitive and some kids can, and do react, to trace proteins....it is why my little man could not thrive on my breastmilk -- reacting to trace proteins.

Everyone following me understands this, right?  It makes perfect sense to me, I am living it - I have a perspective on it that I cant' seem to illustrate.   How do I get his doctors to understand this?  The same doctors that not requested I stop breastfeeding, but insist that I stop breastfeeding as it was clear he was reacting to my milk- despite my giving up all sources of dairy, soy, and gluten.  It wasn't enough, he was still reacting to something.  I knew that, I knew I had to stop nursing him- I knew something in my diet was making him stay sick; so either drastic measures needed to be taken or I could switch him to an elemental formula. He was so sick, we had to do something quickly.  He improved some on the elemental formulas- it helped some but again, just like when I gave up some of his triggers for breastfeeding him and saw some relief - it wasn't enough and he was still reacting to something.   In my diet, it was rice and corn, and maybe other things we have not yet identified.  In the formula- it was the corn and maybe even the soy oil.

I had never heard of FPIES or reacting to everything in breastmilk or reacting to elemental formula's either.... but I have now.

Honeymoon period

This is what Little Man's daddy and I have coined the time period when things look like they are going to work out and finally start to improve and "coast" on this rollar coaster ride for awhile.....right before we hit the wall and fall off the bridge.


Please,please,please dear Lord - I PRAY this is not one of those times. Little Man has been doing SO wonderfully the past few days....I lack the adquate words to describe the giddiness that I am having a more and more difficult time keeping a lid on! My little man is SO happy....could this be? Could we have finally found something to help him finally thrive? I can barely contain the anticipation of the next days- watching him be so happy, hoping for things to stay this way (and continue to get better so we can begin food trials).

Little man had a endoscopy in March, he just had the repeat one 2weeks ago. The first endoscopy, the inflammation wasn't quite visable but the biopsy's told the story...."non-specific inflammation", they kept saying. Now, on the re-scope- the inflammation was "patchy" and visable on scope (so yes, worse) and confirmed as worse on biopsy.

Corn fail has sensitized his system and it is almost so identical to when we started experimenting with supplemental formula bottles and I was still nursing- he was then reacting to trace proteins in my diet and we couldn't figure out why he wasn't gaining weight...and he just kept growing in his sensitivities....

Certainly makes a lot of sense to me, now to just move away from those trace proteins again.....they weren't ok in my breastmilk, why would they be ok in a formula? Clearly, it's not (for my guy who is sensitve to corn)....just have to clarify as kiddo's who are ok with corn thrive so well on the formula's and that is SO great but this just makes so much sense as to why our little man has been so super sensitive and so far away from a baseline for so many months. And why his scope had more inflammation after so many months on an exclusive elemental diet than before.

We re-initiated Neocate in his hemp milk formula, we began immediatly to see his inflammatory markers cascade of symptoms; we saw this plateau off - just right before we began to see blood in his stool. I keep logs of his caloric intakes (everyday since April), the past 10days since dismissal from the hospital, Little Man has been consuming an average of over 1000cal/day (he used to average around 600cal/day). He had a weight check today, he is down again. My theory of what is happening is that his system was assualted by the trace corn proteins in Neocate (once again, higher sensitivy after corn fail), it continued to assualt until it bled his intestines, now he is malabsorbing nutrients again. Time to make some changes....stay tuned....

Many faces of Little Man

The many faces of Little Man....having more good days than bad lately. He learned how to High Five this week- he even says it!! He loves to make all sorts of animal sounds. Loves to play peek-a-boo and clap his hands. He gives the best hugs and kisses. He is such a loveable little guy!

Despite having blood in his diaper yesterday he had a good day, he has not had any today but has had very loose stools all day...and he clearly isn't feeling good and now the detective game begins again....what is causing all this? The Neocate? The new changes with formula? A viral illness?

I called our Allergist right away this morning, he confirmed my thought process that the trace proteins in the Neocate are continuing to assualt his intestines and now has moved to causing colitis. He urged that we stop Neocate immediatly. We notified our Dietitian of our new curve ball and she worked all afternoon on finalizing his specific new formula- Hemp base, carbohydrate sources, complete amino acid source, vitamins and minerals and a little extra fats....my kitchen looks more like a science lab! But I am SOO excited at the possibility of continuing to see his true personality shine ALL day instead of just a few hours here and there, or not at all because it is buried underneath all the pain. Today has not gone as well for him, I suppose he just feels lousy (Allergist said he might -- encouraged me to give him lots of pedialyte today...well, unfortunatly Pedialyte is all corn derived as well). He seems to be running a low grade fever this afternoon, so trying to keep an eye on that. He just couldn't settle down for a nap although clearly tired. I gave him some of his compounded ibuprofen and that helped A LOT -- which is great but also confirms to me that he is in pain. The ibuprofen wore off before it was time to give him more, I tried everything and he was not happy - so finally gave him more ibuprofen and he finally gave in to sleep.

I do not like not knowing what is wrong, or worse- how to stop it or help him....it simply breaks my heart. Hopefully he will get good rest tonight and feel better in the morning.

Tapioca

The next step in building a formula for our little man is adding a carbohydrate source. We will then get a good amino acid protein profile added and FINALLY move away from Neocate and it's trace corn proteins.

Those trace proteins may be causing a new problem, for the first time tonight- his diaper had blood in it. :( Many experience blood in stools with allergic reactions, we saw it at 8weeks with our oldest son when he was reacting to milk proteins. It is called allergic colitis, it isn't necessarily a component of FPIES but many kids have it also. We have not, until now.

Tonight, I'm stumped- why now? Where is it coming from and should we be worried? How worried should we be? Little man has hyperplasia in his colon and the GI that performed his scope said it is common to see mucous and blood from the sloughing off of this from food intolerances. We felt lucky that despite seeing this in his gut, little man had not experienced the colitis.

And today was going well-- we are making little man a formula he can thrive on!!! We have an awesome dietitian who will help assure that he gets all his micro and macro nutrients, calories, protein, fats, carbohydrates. I spoke with our allergist's RN today and got good compliments on how we are handling things and moving forward.

Thankfully little man isn't having any GI distress -- so I am not inclined to think this is leading to an FPIES trigger reaction but I'm certainly not encouraged by this new appearance in his evening diaper change.

We press on....more on the FPIES rollar coaster ride!

We've been here before....

Little man has "colic" behavior in the evenings- he is upset and we can't pinpoint why....is he hungry, is he overfull, does he just want to eat some actual food, does his belly hurt, does something else hurt, is he overtired?

Since diagnosis, it is actually easier to deal with it all; and oddly enough there is comfort in recognizing that we've been in this same place before....he was reacting to trace proteins in my breast milk too and this is the same symptoms it was causing for him. Wish I would've known then what I know now...

He also has one nasty diaper rash, so I imagine that had something to do with his extra crankiness tonight -- poor little guy, it looks pretty painful but I've run out of idea's of what to put on it....he reacts to diaper rash ointments since birth (now know it could easily have been the corn/talc), he reacts to zinc oxide (thought I was crazy on that one but tried to put it on a face rash he had and it made it worse- so no, not crazy...just little man's sensitivity). Out of desperation for him, I put his eczema ointment on it yesterday -- seemed to help some but he was home with Daddy today and I neglected to tell him I was doing that. We've given him numerous baths....put petroleum jelly on it tonight. I can only imagine the burn it causes him...but, again- we've been here before....

Could tell his stomach hurt tonight too, can't wait to get the Hemp milk and try it!! I ordered a case of it and had it overnight shipped. If he tolerates it, I will request our local grocery store to order it in and buy it from there. I did contact the company yesterday to inquire about the Natural Flavors- knowing that this can often be a corn or wheat source....only to have my suspicions confirmed that it is corn alcohol! ((groan))....although she said that they have changed it and it is now corn free- as long as you get the ones produced recently with an expiration of Feb.11 or later. Say a little prayer that that is what is on it's way to us!!

I contacted Walgreens yesterday and inquired if they can make him a formula....they regret to inform us that it is "beyond their scope of practice"....translation- we don't want responsibility for something like that. Maybe if I had a recipe? Hmmmm....not giving up- yet....anyone who knows me knows I do not back down too easily -- especially not for my boys!!

If a Pharmacy can compound a medication....

Then why can't a nutritional company formulate a formula? My little man needs a dairy-free, rice-free, corn-free and I highly suspect soy-free amino acid elemental formula.

His current formula is an amino acid elemental formula....unfortunatly it has a corn syrup solid base. The company has assured me the proteins are extracted; but this would be similiar to separating an egg white from an egg yolk for a person with an egg allergy... (I speak from experience, our oldest son has an egg allergy and he can not have Culvers Ice Cream - a custard made from the egg yolk)... It depends on the sensitivity and severity of the allergic person.

Well, our little man appears to have a very sensitive case of FPIES - he currently reacts to everything.

He even reacts to the sand in our sandbox, we suspect the trace proteins from the Walnut tree in our yard because the squirrels have buried walnuts everywhere in the backyard. But I digress....
Back to the formula. Abbott Nutrition makes an elemental formula (Elecare) and a corn-free formula...unfortunatly they are not in the same mix. Alimentum ready to feed is corn-free (the base for this is sucrose and tapioca starch); BUT (and this is a big but)- it is not amino acid based proteins; it is hydrolyzed (partially broken down) cows milk proteins....which little man has reacted to small doses of in the past (and through my breast milk even).

So.....the question I posed to Abbott Nutrition (makers of Elecare and Alimentum) is: can you formulate a formula to meet my son's needs? The answer: not at this time.

Back to the drawing board. I do not give up easily. Hemp milk is what Dr.P at CHOP recommended, the first ones I have found have too many additives that little man can not tolerate but I'm not giving up. It is a possibility -- with the help of our great dietitian -- we may be able to make our own "formula" ourselves...it won't be easy but I know it can be done! Something that our little man can truly thrive on....just imagining seeing his true baseline makes me shiver...