No Banana

Banana has not made the expanding menu cut.   Little man has had banana in the past, the symptoms have not been clear enough to continue or cut it, but last summer after 3 days, he had bright red flecks in his diapers so we didn't continue.    

A day a week or so back, we were at the Good Food store picking up some more maple syrup and peaches and his brother wanted banana's, he got little man excited about having banana's so I agreed to buy the banana's.   He couldn't wait to get home to eat a banana!  He was so excited, he ate most of a half of one!! Gobbled it down!  All was well and good until 4hrs later he had made a "stinky" diaper, and changing it revealed what looked like raw meat- blood mixed in, and a lot of it.   Sigh, same reaction as last summer only much quicker.  Yep, banana needs to be shelved.   Ten minutes later, another very large diaper with even more blood.  Shoot! And not even ten minutes later, another one!  Crap!! Is this going to get worse?  Are we going to see vomit?   Thankfully no, we did not but loose stools after eating banana is not ok and blood is most certainly not a good sign.   

This was actually right before the GI appointment a few weeks back and when we decided to add back in some of the Alimentum- because with the blood in his stool, he was going downhill fast again.  He had such adverse responses to the Alimentum- again making me think more of it being tied to his dissacharide (carbohydrate intolerance) deficiency because it is worse with a "flare".   After seeing the GI and getting the go ahead to stop the Alimentum, we did for a few days only to have him sink down again- low energy, pale color, disturbed sleep - not reflux but the same type when his iron is low, where he can't get to sleep or is awake in the middle of the night, insomnia).  We were planning on leaving for a long weekend (Memorial Day) and we don't want to be admitted to the hospital before that so we weigh the risks vs. benefits and decided to give him a few days of a few ounces of Alimentum again.  Back and forth and round and round and we still get the same symptoms- paleness, blue around his lips, then behavior issues, large volume stools, then disturbed sleep.  We just don't know what to make of it....clearly something isn't right... We hope he can make it through the weekend, through the travel.  We take along the Alimentum just in case....  

It's Proprietary....

....means the company does not need to release the information of the ingredients.   This very often means we can't take the risk of using their products.  Unfortunately this appears more often than you would expect- especially with allergen acts and labeling laws to help protect individuals.  The sad thing is, I'm not asking for someone else to protect my child, or change anything about the way they do things- but I do need all the information to be able to protect him myself.   The answer "it's proprietary" means Russian roulette to us.  It means I can do the research as far as it will take me to know what the ingredients are typically derived from and take the chance, or avoid altogether.   

Well, this reaction wasn't even from FOOD.   It turns out the lubricant (Surgilube)....which doesn't have a label law since it's not a food but being a chemical, it has what is called a MSDS (Material Safety Data Sheet)    The ingredients are Hypromellose and Propylene Glycol.   I recognize propylene glycol immediately- that is a-typically-derived-from-corn ingredient.   Hypromellose is a substance made using ethanols- and what are ethanols a gas of?  You guessed it.  Corn.  

Of all the things you think you never even have to think about.  But this is the 3rd time he has had reactions to lubricant.  Once was when we used it to help with his severe dry and cracked lips the first time he was on TPN (he was dehydrated on TPN?  Yeah, well...).   Anyway, the next time was the NG tube.  And his scope at the time of his G tube placement showed inflammation - we suspect due to the darn lubricant (that we didn't even realize was being used until the 3rd replacement of his tube; a tube replacement without it resulted in no symptoms and better tolerance of his feeds).   

I am still kicking myself for not registering that she was using lubricant to replace his Gtube last Friday.  Yesterday afternoon was the first day he started showing no further symptoms- since Friday,he's run through the symptoms of flushing of his cheeks, loose stools causing an itchy, sore diaper rash (acts yeasty but doesn't look yeasty), decreased appetite,increased whining, decreased play, and some sleep disturbances, 5 day of symptoms from a tsp or less of lubricant!  Lesson learned, finally....no more lubricant.    

I called the NP from the surgeon's office and spoke to her about it, verifying that she did indeed use a small amount of lubricant.  She was baffled that this would cause symptoms but assured me that she would add this to his list of allergens on his chart to alert others to help us avoid this in the future.  But like my hubby pointed out- she wouldn't have checked allergens before using lubricant anyway!  It will be there, for verification and alerts but we still will have to remain vigilant.   Corn allergy really, really sucks- we aren't just avoiding foods....

Either way, we're past it.  His tube site looks much better!!  He isn't acting like it is as tender but it is as if he is just now starting the true healing process.   We haven't used it in 2 weeks, or longer? So discouraging, but just trying to hold out hope that if we let him guide us and let it truly heal- we will be able to use it soon.   He is drinking 2oz. of the Alimentum with every 4-5oz.bottle of hemp milk so he is getting some of it everyday- which is really good because once we are able to use the tube, we won't have to do as many "feeds" and yet will still get the benefits of the feeds and using the tube to keep him thriving and safe, and his quality of life and ours, increased.  We still hold out hope to get there anyway....

Pressing through trials, more pineapple and on to potato...

With a few days into pineapple looking like a go (our new plan to move through trials), we moved on to potatoes.  Little man has had it twice before- bites (nibbles) of a baked potato. He isn’t terribly interested and I had picked up some Ruffles Naturals potato chips. Ingredients: potato, sunflower oil and sea salt. I ate them when I was on the elimination diet and nursing him a lot (a lot of chips have soy ingredients in them); although we didn't know anything about his corn allergy and I wasn't finding his baseline on my elimination diet- I simply remembered them as a potential friendly snack.   I just thought it would be fun for him to have a fun snack to get him excited about eating, thus making the future trials easier for him. Potatoes are very starchy, and he doesn’t have very much enzymes to digest the starches so that is another big reason for the chips- hoping it could help him stay within his threshold (a threshold recently discovered with pineapple) so we wouldn't have those symptoms confusing us for FPIES symptoms (adds to the complexity of the situation for sure).   

The first day went ok, he had them twice even- asked for them at bedtime! I started to see some subtle symptoms and the all-too-familiar- 4am crying in his sleep; but I was hopeful it was just a coincidence (when will I learn coincidences are rare in FPIES).  

The 2nd day he had his chips twice again, and he was GREAT all day. That night I also got him to take a few nibbles of a baked potato.   We were feeling optimistic that the random coincidental looking symptoms were just that- coincidence, and feeling empowered about finding him a fun food!

The 3rdday he had them once around lunchtime, and I was baking him some fries for dinner and had gone downstairs because he was starting to throw some tantrums and was quickly declining...and then he vomited. Ugh. No more potato. Darn it. It was one large vomit and he seemed to be doing ok.  He had a poor color (is he grey?) look to him a bit later but he took a bottle and his color came back, before falling asleep (early) for the night.  

My mind did not shut off all night, was this dissacharide or FPIES? If it was dissacharide, he could have small amounts- such as in baking powder that is made with potato starch and not cornstarch; and we can retrial it again sooner and simply find his threshold that his body can digest at one time.   If it is FPIES, anything related to potato needs to stay out. Then thoughts go to:  is it FPIES to potatoes or was it sunflower oil or contamination of the chips themselves? Corn-tamination?  Or is it FPIES to pineapple?   We've been continuing the pineapple and he's had it off and on (juiced, 1-2tsp in 6oz.of water).
The next morning he had the all-too-familiar-smell-it-from-the-next-room blow out diaper. Sigh. Crap. Literally. FPIES for sure, not dissacharide deficiency; an e-mail to the potato company indicates it is not corn-taminated although it could still be the sunflower oil.   

Recovering from this reaction has been hard on his body, even with the TPN we are noticing signs of dehydration, he is refluxing which he hasn't done in so long, and I wouldn't be surprised if his hemoglobin and iron stores fell again as he is so very tired (although has insomnia), he is chewing on things, fingers in his mouth, and very pale.   He is only eating about half his formula intake during the day (again, thankful for TPN overnight!), and isn't interested in any of his safe foods. 

Finally, almost a week later he is beginning to look better, act better and eat much better and I encourage him to drink his pineapple juice again.  One evening last week I made him some of his Millet Merry Muffins, he hasn't had those in awhile (since zucchini fail, he associated muffins with food that hurt his tummy).   He was excited about them again and actually ate a whole one!  (mini muffin, in crumbs, over the course of an hour).   Six hours later, vomit.  alot, repeated.  We get him cleaned up and settled down into bed, he is tired.   The next morning, he seems fine.   What was that about?   Is this pineapple?  Is this random?  Is this his gut still inflamed from last week's reaction? 

The next day, he doesn't want his muffin but he is willing to take a few sips of very watered down pineapple juice at dinner.   We go to bed, and again, 6hrs later he cries in his sleep, sits up and throws up everywhere, repeated, choking, vomit.  What is this?   Does he have a virus?   Is his stomach upset from the pineapple?   Is he failing pineapple?   Was the potato reaction actually an initial pineapple reaction?   He's been so off all week, was he recovering from potato or chronically reacting to pineapple?    We decide the game plan would be to stop pineapple for a few days, give his gut time to heal more, be sure this isn't a virus and move on in the following week.   He improved dramatically over the next few days.   Something is off, but what is/was it?    We have to reintroduce pineapple, or should we retrial potato?    As strange as it may sound, we may have to let him decide because he isn't wanting to try anything now again....he's a quick learner- which is not good for a strong oral aversion.   

"Mommy, I need you"

Little man started this about 1-2hrs. before the vomit the other night.  It is new.  Holding out his arms, he stood by me and said “mommy, need you”.   

From there, the symptoms continued to build, whiny and clingy and then throwing things and crying, and then the vomit.   Potato now goes on the shelf. 

As I try to decipher through his reactions and symptoms (symptoms we may have missed before getting to vomit).   My thoughts also go back to when Little man had the sepsis in his PICC line- how the symptoms came on suddenly, the chills/fever/rapid breathing; followed by vomiting….for little man, this happened twice, and then shocky symptoms appeared, after the third time came the full shock reaction.  The body could not expel the toxin because it was in his blood.   How similar is this to FPIES reactions?   The toxin is the food, that is in the GI tract (and “leaks” into the blood) and the body works to expel it (the vomit/diarrhea).   But what happens if the body doesn’t expel it?  Little man doesn’t get immediate to shock symptoms (acute FPIES) from foods; my theory is that they are septic in his body before/instead of that.  The reaction from the food-perceived-toxin is a sepsis like reaction; labs done during this time show that his body is struggling with what looks like an infection.  Continued ingestion of the trigger food “builds” this toxin in the body, thus further weakening the body under this sepsis.  The first time the body succeeds and expels the food, it is a sepsis response; continue this and the body becomes more efficient at expelling the toxin and the sepsis quickly becomes shocky symptoms.  Each food can be different set/order of symptoms to this reaction, as the body struggles to either accept it or reject it.   The mechanisms and reasons to why his body accepts some foods as safe and perceives others as toxins remains a complete mystery.

Fluids help the body cope with a sepsis reaction, they are part of the treatment for it- the hydration helps keep the body out of shock as the sepsis affects every organ. Little man is on IV fluids every night with his TPN, I really have seen how this has helped his body cope through these reactions the past few weeks. And I am grateful. 

And where does the pain come from?  What kind of pain is it?  Recently, I had a touch of the flu- my body was fighting a toxin and it ached all over.  I didn’t have a fever, I just ached everywhere.  I wonder if this is similar to what it feels like for him.   His body is attacking a perceived toxin, so is this how he feels every time?  When he was under a year, I used to give him some motrin to help with the pain- between the teething and the chronic FPIES symptoms, he lived in chronic pain, so when there was a spike in this pain- I would treat it with some motrin.  Unfortunately the motrin only helped some- he would get pain relief for ~2hrs., and then he would be in worse pain than before.  I thought that was odd since ibuprofen was supposed to last ~6hrs!  Once we got him compounded ibuprofen, it made a world of difference!  Pain relief that lasted 6hrs.+ and didn’t cause more pain!    I also remember thinking (and saying) that it seemed to me to be pretty significant pain, that if an adult was experiencing it- we would surely ask for a morphine drip! And yet, my son’s only comfort is occasional ibuprofen.  Well, ibuprofen and daddy or me; and so he says, "Mommy, need you".  

Pressing forward & Pineapple Pass!!

We’ve been pressing forward with offering Little man a variety of foods: 
1). Strawberry he ate one, next day had red-rashy cheeks all day.  Refused to eat anymore strawberrys.  Juiced some one day, coaxed him to take a sip- which he did and then looked at us like we had fed him poison, pushed it away and refused any more.  That afternoon he was a complete bear.   Enough, he doesn't want them, coaxing him to take some made him miserable.  Shelve it.
2). Pork- ate 2 bites, got them down but struggled with the texture, refuses to eat again. 
3). Potato- ate only a few nibbles and the following day had significant behavior changes- he has very little enzyme it requires to digest starches so unsure how far we can push this.   We're trying again  (now) with natural potato chips. 
4). Cauliflower- refuses completely
5). Beef- refuses completely (won't even allow the plate to be next to him)?!
6). Pineapple- juiced, 1-2tsp in 6oz. of water and he loves it.  No noticeable symptoms on first days! We have continued it and we do see some bloated belly, explosive stools; but feel this is related to his dissacharide deficiency.  For now, we will call it a pass! We will rotate it and limit his amounts but feel good that symptoms do not build upon reexposure.  
7). Eggs- was enthusatic about, but despite offering in a variety of ways, gets less and less excited about and has building symptoms from less and less nibbles (mostly behavior and sleep). We had been continuing egg all last week, he wasn’t interested in it as much as we had hoped he would be…in the past, for little man, that is a sign that he isn’t feeling well after eating it- symptoms that he can’t communicate to us but maybe include stomach cramps, achiness, mouth/throat itch; all confirmed after about the 8-10th exposure, he wakes up early one morning to fill my kitchen sink with vomit.  It wasn't till bile, but it was significant.    We don't call anything "FPIES fail" if it isn't to bile/diarrhea/dehydration....would it be so if we continued?   Maybe.   We're just not going to find out.  We'll give it at least a year before we try it again.   We're bummed about eggs, we had such high hopes for eggs- and it certainly increased the baking recipes! 
8).  Coconut - significant behavior symptoms from the flour, maybe it is just the fiber in the flour so I tried oil.   I snuck it into one bottle (about 1/4tsp) but the next time I tried to sneak it in he noticed and then refused his bottle.  I can't take that chance so tried it another way- coconut ice cream!  He doesn't like it!  We've tried coconut before- this spring, we got to day 4 with NO symptoms and then day 4 hit and he developed symptoms fast but since they were accompanied with a fever, I knew we had to try again since it could've been something else.
9). Quinoa- made him his "cookies" with quinoa flakes, he tried it one night but won't try it again.
9). What's next on the list?   Carrots, kale, try pork again, try beef again, try quinoa again, ???

We need to keep introducing foods, it is helping collecting data for his varied reactions so we can assess if he has something else on top of his FPIES.  The behavior symptoms are difficult as they can be from his dissacharide deficiency or his FPIES building.   What behavior symptoms do we see that are concerning for FPIES?   As the trials progress, he eats less and less but gets further and further away from baseline/ his “normal”- his tantrums increase (and get more irrational, any tiny thing will set him off), he has started hitting (hard!), he throws things (not good!), and he screams!  his sleep is disturbed as well, and so is his appetite.  He is just not our Little Man, he isn’t like this at baseline, he isn’t like this when we are not trialing foods, what is this beast inside of him? Why can’t food NOURISH him??! I can’t even begin to describe how this feels, to a mom….to not only not be able to nourish him from basic foods, but to have basic nourishing food turn him into this unrecognizable child. 

What if?

The other day I wrote about  trying a new plan, I am hoping to catch up a few posts on how we got to it and how it is going, so far.  

What if?  What if Little man could tolerate foods in a rotational way? Or with shorter trials? Not putting any extra stress of a drawn out delayed reaction trial on his body?  For the past 2years, I have been studying, documenting, logging and evaluating little mans FPIES symptoms and reactions.  

FPIES is a syndrome- meaning that it shares common symptoms grouped together.  FPIES has the common shared symptoms of profuse vomit, diarrhea, inflammation, dehydration, immune response.  But there are also a host of other symptoms, the chronic FPIES noted in some medical literature sheds some light to these other symptoms.  In FPIES not all symptoms are seen in all children although there are main features shared.  For some (many) FPIES kids, they have 1-2 “triggers”, where others have multiple triggers and other non-IgE reactions;  and a few only have a few ‘safe’ foods.   Little Man isn’t the only FPIES child at 2 ½ with 5 safe foods, he isn’t the only one needing supplemental nutrition (although many kids are on G-tubes vs. the IV nutrition he gets).

In a recent communication with Dr.J (a research  immunologist researching Non-IgE allergies in different populations), whom we saw in consult last spring; she relates that she has theories of there being a difference in kids’ responses to the elimination/elemental diet - some respond very well to it, benefit from the gut rest and time for healing and complete nutrition; while others have different responses- that their immune systems respond differently.   What if that is why so many kids are like our little man and struggle to find safe foods after taking much needed breaks?

I keep thinking that when Little man was on foods, as we were restricting his diet more and more- some things were getting better and some were getting worse….I keep wondering if we had just identified the corn trigger and moved away from that, if we would have been able to keep his other foods, or go back to them much quicker.   But because we were lost for so many months, and his system kept getting more sensitized, stopping foods meant long healing times; but then what?   Why have we been unable to add foods back in?   Why does he seem to do ok on a food for a little while  before he starts to show us sure signs that his body is struggling, or has a full blown reaction?   Why does he have this “honeymoon” period?    Why did he respond so well to probiotics and why did he respond so well to antibiotics?  I feel like for him, the answer must be in his gut flora.  Our gut flora even has roles in our immune responses.   So, is this why he has delayed responses?  From the tie to his gut flora?   His body accepts the food initially, or fights too- it has the mediators it needs to  fight it (vs. full shock in the first bites), but then it comes back around and attacks it….is this the gradual change in gut flora from the new food in the gut environment?   It builds to a level that then pushes the body over its coping ability?  The attack methods then win?   What if??  And more importantly,  what do you do about it?  

What if we re-introduced a few foods at once?   What if this worked?  What if it fooled the immune system and we gained a new food or two?   What if he reacted?   Well, he runs the risk of reacting any way we do trials.  So why not try it?   Why not use Little mans body as the guide and push forward- listening to his body signals of intolerance, stopping if he isn't tolerating (whether FPIES or not), making notes and pushing forward.    Ideally, this was the scenario for the in hospital trials (recommended by CHOP) to help us identify what else is making his FPIES complex but also to gain a few foods into his tiny menu.   

So, the next step was to develop a plan, a plan for a rotational trial.  Little mans daddy and I discussed it and we decided on:  3 foods for 3 days, rotate to 3 new foods for 3 days, rotate again and then rotate back.    We picked 9 foods, including beef and eggs from a corn-free ranch 3+hrs away that his daddy went to pick up (that took him all day- that’s a dedicated daddy!).     Healed from zucchini, we were ready to implement a new plan for little man.

Little man decided that his first day was going to be strawberries!  He loved them, ate almost a whole one.  The next day had red-rashy cheeks but otherwise was fine so we offered them again- he refused; and has refused every day since- despite multiple offers.   The next day he did eat 2 bites of pork, he struggled with the texture but I had unknowingly cooked up a stringer cut so I am hoping he will try again with chops.   The following day was potatoes; he barely took nibbles and the next day was an “off” day with lots of clinginess; was it the potatoes or is this too much for him?  Noted, moving forward.  The following day was the eggs- which he was enthusiastic about but each day has eaten less and less.   We tried coconut (flour) and got some all too familiar behavioral symptoms.  Coconut in the form of coconut flour may be too hard for him to digest so I will retry that with coconut oil, then came pineapple which we juiced and combined with some water and he LOVES!   We have also tried beef (hamburger) and cauliflower- neither which he will even touch or want near him!  We will have toddler finicky- ness to deal with I have no doubts but with his relationship with food already being negative and us having no idea if the food is going to cause him to be sick- I am NOT about to make him eat it, or force him or even coerce him.  If he wants it, great; if he doesn’t, fine.   We’ll just keep moving forward.   What if? 

We need a new plan

Zucchini caused a lot of symptoms and we stopped before getting to full FPIES vomit-till-bile session with dehydration.    Little man suffers for weeks recovering from those reactions, we know from his past all too well. Slow food trials are intended to avoid that, to stop at first signs that the body is not tolerating a food….for whatever reason-.  If his body cannot tolerate a food, it shouldn’t be in his diet causing problems and preventing him from expanding his diet. Unfortunately, for our little man that has meant a very limited diet.   He isn’t the only FPIES kiddo with a very limited diet still at 2yrs.old. It may be because that he has other things going on alongside his FPIES (one being a disaccharide deficiency, others unknown), or it may just simply be his FPIES.
  
We didn’t get to full FPIES vomit till bile (although we did see vomit);and yet we still had weeks of recovery time- weird diapers with mucus and blood and undigested food, terrible irrational behavior, decreased appetite, not trusting his safe foods, is he reacting to his safe foods?, whining, tantrums….and we stopped before the FPIES vomit.     In the past, doctors not familiar with FPIES, or our little man, have previously advising us to push through until we get to the vomit (and not sure if we’d be advised to stop even then).   With Little man not on an elemental formula, and missing nutrients in his restricted-unable-to-expand-diet; some doctors are sure any symptoms and reactions we encounter surely do not outweigh his needs for the nutrients those foods would supply. I know, from the outside looking in, it seems quite bizarre. It IS bizarre, that his body would not just reject food, but attack it in the way that it does!! It’s very bizarre. But that doesn’t make it not real, a very scary, exhausting reality that his daddy and I live with day in and day out.    Unfortunately, we feel it is poor advice to tell us to push through symptoms that make our little man sick, especially without factoring in his illness complexity.  There isn’t many other ways to put it- it is poor advice to not consider his allergic mechanisms when advising us to treat other symptoms.  I have explained it this way to people- this is the logic we face….it is like telling someone with celiac disease that they need the vitamins in fortified bread so they must eat the bread, even though it contains the very gluten protein they are allergic to. The allergy isn’t anaphylaxis, so it will be fine. NO! It won’t be fine! A person with celiac ingesting gluten is doing chronic, and often silent, damage to their system. It is vitally important for a person with celiac disease to avoid gluten- in any form and in any contamination of. That is how our little man's body responds to corn, and many other of his triggers. His body suffers internal inflammation, oftentimes inflamed before he shows us overt outward signs (FPIES vomit till bile); so we watch his other signs- other signs that tell us that something is causing inflammation in his system. Will his body be able to cope? Will the inflammation subside? Or will his body try to cope, only to exhaust every internal anti-inflammatory mechanism until it is too tired to continue? And then everything starts to go “haywire”. That is what happened with zucchini, and countless other foods we have had to shelve….his little body just gets too tired to continue fighting- so instead of making it, watching as he gets weaker and more off character and unrecognizable, we stop giving that food.     

We are now a few weeks past zucchini, ready for the next trials.  But what we did learn was that an in hospital stay for a trial isn't going to work for our little man.   The symptoms we see and the symptoms doctors are measuring are too different.   We are responsible for keeping him safe, keeping him from being sick and increasing his nutrients.....and we remain his voice during these reactions- FPIES or not.   Where does that bring us?    We need a new plan.....

Surviving not thriving....

I used to refer to Little Man as that- that we were just surviving the days, he was not thriving. This is what some foods do to him and I honestly just do not know why. Even before he is having overtly outward FPIES symptoms, it is the build symptoms we know all too well but it is these symptoms that his docs want to see if we can push through, that it is some sort of adjustment thing. Maybe if we knew it was just adjustment or some other intolerance, we could do it… IF I knew that on the other side of this torture was a new food! But, we don’t know that, and for all we do know (and for his history), it is a build to a full FPIES reaction that could be prevented if we stop early enough. And then his system gets so off (presumably from the inflammatory cascade it is fighting on a daily basis while we keep pushing a food his body doesn’t have the mechanisms to tolerate). I’m not sure how long it will take to come back to ‘normal’ (yes, he has a normal).


That is why were supposed to be getting labs throughout the trial. He has a PICC line, enabling us to watch what is happening in his body so that he doesn’t have to go through this additional suffering; so we can match his first signals to what is happening in his body – his body fighting for oral tolerance or his body building to a reaction and each day less and less of the anti-inflammatory mediators are “winning” and more of the inflammatory cascade is taking over.

Through the zucchini trial, each day, he ate less and less and each day his symptoms build more and more. But his stools have evened out from all water/soaked in the diaper, to a thicker consistency but with some significant mucus in some of them. His symptoms are just all over the place. It reminds me of when we did the probiotic, and we pushed through and we got some great results (2# weight gain, increased appetite, happy playing boy) but then he hit a plateau on the probiotics; or he was building an intolerance to one of the inactive ingredients (which I am suspecting more now that he “failed” them after stopping them in August, and then re-introduction of them recently. And then my mind goes to- what if he was building an immune response to the probiotic ingredients all along and that is why he had so many complications this spring/summer? Why he had colitis with each food introduction, why he has had small intestine damage on the scopes, and why he has had persistently severe anemia. It was as if the probiotic was helping him with the gut flora but then hurting him with the intolerance to the extra ingredients. A change in probiotic to one with no added ingredients only made our picture so very messy as his system clearly did not tolerate those. And yet, my mind goes back to those beginning days on probiotics when we learned so much about how his body goes through so many FPIES-ish symptoms but that nothing was building or consistent, and it all evened off and we got great results (even if just for awhile). My mind keeps going back to where my original thoughts were, when he was just an infant….that there is something wrong with his gut flora- that any dramatic change in that alters his system and that too many times, his body can’t cope with those changes; because it doesn’t have the proper support. When he was breastfed, he had the support- again, another catch 22…where the breastmilk was giving him protective effects but it was still upsetting his system- and too much upset just tipped the glass over.

I didn’t WANT to think it is the zucchini itself. So, what is it then? Why the build? Why tolerate for a few days and then crash and roll? Why so many foods causing these symptoms and eventually (build) causing a reaction? Why can’t his body “win”? My gut says it’s his gut….

Zucchini will not be added to Little Man's safe foods....

We started zucchini last week.....we're doing in hospital trials for the first 3 days (not challenge, trial) to see if we can begin to put together more pieces to Little man's puzzle of multiple intolerances and reaction symptoms- is it all purely FPIES or are there other intolerances that are compounding his FPIES, giving us further foods to not include in his safe foods?   He does have enzyme deficiency- he lacks the gut level enzymes to digest sugars/carbohydrates/starches; so that will continue to make it more muddy for him.  The other question is does he have a metabolic component- something we haven't yet tested for?  He recently had some incidental lab findings (when working up a low blood sugar) that may be clues to some metabolic issues compounding, we are getting a work up with a metabolic MD now so will update more on that later.  The other component we are looking for is an auto-immune.  Does his body attack itself because of the FPIES and then not be able to turn it off?   Does his body attack itself from not tolerating a food and then turn on his FPIES?   We simply don't know.  The goals of food trials right now is to collect more of this data so we can analyze better - if there is something else we can be doing to help him thrive through this diagnosis.  Are we missing anything?   There is a good chance that we are not.  There are a few handfuls of FPIES kids who do not have more than a dozen foods until they are 4-5, or older.  This could very well be simply what is happening to our Little Man as well.   The complicating factor is his severe and sensitive corn allergy, and that he cannot tolerate the elemental nutrition many other children survive and thrive on for these years while they wait for their body to heal.   

Is zucchini a full FPIES fail?  We didn't get to full FPIES-to-bile vomit so we won't ever know 100% sure but he did have vomit, he did have diarrhea, he did have dehydration.  Bottom line, he simply got to sick- from such little amounts of food, that he was increasingly refusing to eat....less and less ingestion causing more and more symptoms:  


The first day- he had about 1/2tsp, he had some off symptoms but nothing that i could definitively identify as a reaction symptoms build as they were muddled with him being 2, and in the hospital.

The next day, his body was having some low blood sugars (before we even did the zucchini); and then he went into acidosis (low bicarb and continued low blood sugars) after the zucchini; and had a full blown "episode"- full tantrum where he was irrational and inconsolable, and he was drooling (not ok, not normal); and choking. He took about 45min to calm down (this is a classic type tantrum when his body is struggling). The next morning, his neutrophils were declined and his hemoglobin fell an entire point. All of these labs were written off to be related to something else; so we were encouraged to push forward. Although Sam was not interested in eating any more zucchini. So, we switched to baked in form.

Day 3- he ate only nibbles but seemed to be ok; no repeat of symptoms from the day before and his labs were all re-cooped. It appeared, at this time, that his body was trying to tolerate the food- and winning. We were optimistic. We went home the following morning.

Day 4- he had some "cookies" (zucchini/millet/oil/peaches) at home, did fine but was having some "poop soup" diapers but not really any other symptoms- and was just happy to be home.

Day 5 - things start to get a little muddy, poop soup diaper again, starting to get whiny and cranky off and on.

Day 6- I give him a Tsp of boiled, purred zucchini again- thinking if he was building to a reaction, we would see it from this controlled amount (vs. baked in). He had no concerning or building symptoms that day, he slept well overnight- doing well overall.

Day 7- eating zucchini/millet flour/oil biscuits and loving them- doing well all day; gagged on a piece of zucchini (insisted on holding a piece of raw zucchini I was cutting up to prepare to freeze and bit a teeny-tiny piece off)...or at least I think it was from the zucchini. It was enough to cover his plate but he was fine after. Although he has choked before on foods (due to texture) and not thrown up like this, so….

Day 8- slept well overnight, poops are no longer loose but do have some mucus in them (but that isn't so out of the normal for him that it would alarm me)- was doing well in the morning but started to really get "off" today- lots of whiny/cranky, looks so tired, and pale.

Day 9- eating less and less - of these baked millet/zucchini biscuits, whiny and clingy, more mucus and smell in his poops, seems to be having low blood sugar episodes,....

Day 10- rough day, decreased appetite, decreased wet diapers, looks terrible (purple around eyes), disrupted sleep (crying in sleep a lot- for naps and night). Had some millet/peaches waffles (no zucchini- made biscuits but barely nibbled them)- loved the waffles!

Day 11- same as yesterday but worse. Each day he eats less of the zucchini but gets worse. He is declining. Sleep is disrupted. Mood is terrible, whiny and angry- hitting and sensitive...

Day 12- today is day 12 and he's a mess. There isn’t' anything significant happening in his diapers. He had this weird drooling this morning, put his hand in his mouth, gagged and threw up (caught me off guard as he doesn't do that when he's at a baseline) but don’t' know what to make of it. Feel he had low blood sugar episodes again today. He hasn't played AT ALL today- he's been in bed watching movies all day, or in my arms, or sleeping. He is taking his bottles better today (had been declining for a few days).

Day 13: He slept ok through the night, except when he woke up at 5am, crying, I notice that his diaper is dry- completly dry.   He has been hooked up to IV nutrition for ~8hrs, and he has not urinated.  I calm him down and we go back to sleep (he does not want to drink anything).  When we woke up at 7am, he had soaked through his diaper....something caught up and "clicked"...whew.    But that was weird, he is always wet through well before 5am- between drinking 30-40oz./day and getting IV nutrition through the night; he is an overly hydrated little man.  So, any signs of dehydration- even temporary, concerns me.  
This morning is his weekly lab draw.  This lab draw will help me decide if we will continue to challenge zucchini or if we need to move on.   If his internal body shows me the signs of struggling that we are seeing externally, we will need to stop.  If his body isn't showing any signs of struggling, we will challenge with a full dose today and measure labs 4-6hrs. later and the next morning so we can get a more clear answer. 
I keep him hooked up to his IV nutrition until the moment the home health nurse draws the blood.   The home health nurse takes a few notes and then takes his blood to the lab to be analyzed and we wait.  I am nervous about "challenging" him today with zucchini as he is just a mess.  He is whiny and clingy and clearly not feeling well.  A few hours later, we get the labs and they reveal a very low neutrophil count (white blood cells)- a pattern for Sam following a reaction that we have observed in the past is this neutropenic look to his white cells. His hemoglobin is also falling again.   And, most concerning- his blood sugar is quite low (45).  His body is struggling to maintain his blood sugars- and that is while he is getting nutrition infused into his blood stream!  His platelets are on the rise but not out of range of normal, although we have never seen them too high since starting TPN- but he gets heperin in his line to keep it from clotting over, so I would imagine we won't get true readings with that; so trends of increasing is what we're watching for.   Another lab that we would see if his body was struggling, another one that is in his pattern, is his bicarbonate levels- he becomes clinically dehydrated BEFORE the vomit/diarrhea.   I suspect it has something to do with the sepsis state his body is in while "fighting" this reaction.   His bicarb is not low....but I am susicipious this is being masked by the IV nutrition (thankfully!) -- remember the dry overnight diaper at 5am?   That tells me more accurately what the IV nutrition might be masking.  

It's enough for me, the outward appearance and suffering of our little man and now his pattern of labs indicating that his body is struggling.   There is no reason to include zucchini in his diet, we won't be able to move foward with other foods, he's not thriving he's surviving.....no.more.zucchini. 

How is Zucchini going?

We started with zuchinni because it is an easily digestible food, low sugars, with nutrients he needs, and can be baked into the one food he loves- his merry muffin/cookies made with his 5 safe ingredients.


So, the first day we gave him a tiny slice of steamed zucchini; he had some symptoms that afternoon that were notable but nothing to write home about, but note and move through....so the next day I got him to take a tsp of boiled, pureed zucchini (that I pureed with some of the water I boiled it in- was that bad?) - he let me feed him a teaspoon and then would not take anymore. At the 4hr.mark, he had this terrible-terrible and classic-for-him irrational tantrum...the kind where you can't let them out of your sight because they may do something to hurt themselves because they are being so irrational (but he wouldn't let me hold him)- so he is having this tantrum in the bathroom (of the hospital) and he is drooling excessively (doesn't normally do that when he cries and he is 29mo.old so drooling isn't an everyday occurrence around here).....another FPIES mom helped clear this up in that it is uncontrolled reflux, which makes a lot of sense.  He isn't necessarily crying as much as he is screaming- no tears, just screaming...then he starts to choke (not on his saliva, almost as if he is throwing up in his mouth and swallowing it down- you know when you recognize that sound)...it goes on for at least 30min. The plan is to take labs if he is symptomatic- so I ask (ok beg for these labs) and they are taken and found to be "off". He finally calms down and then is ok for most of the day- until he has some cookies later and then about 5hrs. after that, he has another one of these "mini" tantrums- this time he has clear mottling and his feet are purple when being held (again, classic for him when he was an infant and symptomatic). But then, he recoups from that and is fine again, he sleeps ok that night. Then we did baked zucchini the next day-Friday (so boiled and pureed and then baked into his muffin/cookies) and he doesn’t' have any symptoms to write home about. We got him home yesterday and he had his cookies again at dinner, and 3hrs. later had a poop soup diaper; but no other symptoms- sleeping good, eating ok, has a red rashy spot on his right cheek, behavior is ok,.... The plan is to continue the food trial for a full week- now at home, getting labs as needed. Until poop soup diaper, I was feeling confident that we were going to be able to work with just baked zucchini into his diet....and not even 'test' the boiled zucchini again (why make him miserable?) but now I worry that all we're doing is drawing it out and going to end up making him sicker in the long run vs. just knowing.

He's been, the past 2 weeks (since probiotic/sorry board game ingestions/symptoms) a little on the slower side for dirty diapers, only going every other day, and it being more play-dough so now to have soup...it's confusing... The labs that were off were his blood sugar and bicarb- he fit the criteria for acidosis (which he has had before with reactions); and his neutrophils (white blood cells) took a huge dive (very classic for Sam following a reaction, and why he always gets a cold after- (he's susceptible because his WBC's are down), and his hemoglobin dropped a full point (and didn't recover)- again classic patterns we’ve (I’ve learned) for Sam. I'm nervous but we need to know.....it seems to me that his body is having these dips of problems so if we helped his body (by baking it)- he could tolerate it? But....this could be his body adjusting or it could be building to a reaction....I'm trapped because we are supposed to call it a pass or fail after 7days but if he doesn't get enough of a serving- it's going to be hard to call it, but if we do too much- and his body is just adjusting we risk pushing his limits versus teaching his body to tolerate the food (which obviously is the goal).
My theory is: what we are seeing now (and the labs that correspond with his symptoms), but then we see him re-coop. I am curious if this is his body training for oral tolerance or if it is his body teaching to react? So, do we push it so we know and not continue to make him more chronically ill; or do we take it slower so we continue to teach his body oral tolerance?

Halloween Screams and Probiotics

I am a little behind on the blog, so much going on!  Over the next few days, I am hopeful to begin filling in some posts to update as we now into a zuchinni food trial!  So there is much to share, and still so much to process. 

Little man was on a 10day course of antibiotics after being discharged from the hospital for the line infection. That course was set to finish Oct.26th. I began to think about how this long course of antibiotics, plus the multiple antibiotics during the hospital stay, would be robbing his body of bacteria- good and bad gut flora. Ridding the body of the “bad” gut bugs is a good thing, now how to ensure the “good” gut flora flourishes so that there is homeostasis in his gut: where the beneficial gut flora reigns, the “bad” gut flora remains to do its job keeping the fungal’s at bay.


We had stopped the probiotics he has been on since March, in August after a switch in brands caused some questionable symptoms and coincided with the scurvy he developed. The beginning of his probiotics last March was very favorable for him, he gained weight, his appetite increased, his labs leveled out, his hemoglobin was stable, he was at a baseline we hadn’t seen him at in a long time. However, he quickly hit a plateau so I set off to find a better fit- first I gave him the probiotic in 2 doses, a morning and an evening and that seemed to help; then I thought if that was helping, I wonder how he would do with the probiotic in his formula so he was getting a continuous “feed” of it all day, and so the probiotics were in his gut in an environment (with the food directly) that would support their growth at optimal levels. I can’t change his diet to encourage that (with foods referred to as ‘prebiotics’) so this seemed to be a good answer; and he again responded well to it. I don’t remember exactly when he seemed to be having a plateau or something again, but I thought he needed new strains- maybe the strains I was giving him was creating more dysbiosis within the beneficial bacteria. I tried adding a strain (bifidi) and he had too many symptoms to continue, I tried adding a new line (ProBioGold)and he developed symptoms, fevers and even an ear infection (his first and only!). Back to the CD-Biotic (3strain probiotic from Kirkman labs). Then, following advice from the Functional Medicine doctor we saw, he was concerned over this combination of probiotics and felt Sam needed custom probiotics; he had a guy (the “bug guy”) that did this very thing; so we switched to Custom Probiotics. That brought us to August when he had symptoms and then developed scurvy.

Now, the antibiotic brought us to a great baseline; all to see it slipping away with reintroduction of the probiotic? Does this make sense? Is this die off or is it a reaction (maybe one he was having chronically all those months) to the filler ingredients in the probiotic or even a reaction to the strains? Initially I started with the CD-Biotic, which is the one he responded so well to in March and the one we felt he did well on for a long time- even putting it in his formula mix. He had symptoms and I worried the long course of antibiotic was too much and 3-strain probiotic was too much to start him off with; so I switched to a single strain I had gotten to try and add to his 3strain a few months ago, or try in place of it since something seemed off. Dr.Jyonouchi, the research MD we saw in NJ (in Feb) had said he would need to be on a low strain (single would be preferable) as his system was so severely dysbiotic. After trying a few days of CD-biotic, with these symptoms of uncertainty, I thought I’d give it a try this time around….

Which brings us to Halloween screams. I had given it to him that morning for the first time (I worked over that weekend and I didn’t want to do anything while I wasn’t home). I thought it was attributed to him being upset that he couldn’t have the candy he saw us pass out to the trick-or-treaters. It was a bit irrational and extreme; when he had it the next night over not letting him dump water on the floor- was starting to wonder if we were seeing a pattern? That something simple was setting him off because he was having some underlying pain/inflammation/whatever-happens- in- his- body- during- a- reaction. I skipped the next day and he was fine. So, I gave it one more time- somewhat reluctantly…and, like clockwork, ~8hrs after he consumed the probiotic, he is screaming and screaming and screaming. Irrational, inconsolable. Other things I notice is that he is sweating (from the fit) but cold, also he is gagging every few minutes- kinda like you would hear from a typical kids’ crying fit when they are sobbing so much they begin gagging on their snot…only he doesn’t have snot and he’s not crying as much as he is just pure screaming. And the gagging is more like he is throwing up in his mouth and then swallowing it.  And even his brothers recognizes the screams and the pattern, and say “first it was the Halloween candy, then the next night over the water/ice, and now this? What is going on?” Also, in the night on these nights- he is crying in his sleep and even crying out “help”, it takes a lot of rubbing his rock hard belly and patting his back to get him consoled for sleeping.

I didn’t give it to him yesterday and he was great all day; until he sucked on the Sorry board game when I wasn’t looking (or expecting)…and 8hrs (at 4am) came the screaming – that was fun. And today he is a little clingy. Most cardboard products like that have corn and potentially soy in them- and it becomes more clear that the probiotic is the same pattern of symptoms. His pattern of symptoms. Now what?

This got a little long, and complex- but this is my thought process as we go through the day(s)….everything needs to be trialed, anything could cause reactions. I’m tired.

Zucchini

Is our decided on first trial, next week when we are admitted while our GI is on hospital service, to do a food trial(s). During the care conference we decided on zucchini, based on my list of foods I have thought out: Zucchini, carrots, potato, pork, quinoa.

Little Man had never had zucchini, he has had carrots here and there, and he's had potato- before we took away food at 7mo., and recently I let him have a few bites of a baked potato.

Little Man is a delayed reactor. He falls into a category of chronic FPIES, where he does not have an acute reaction with first exposures of the food. Why - we don't know. But we do know it takes multiple exposures to a food for us to see measurable symptoms; and with how badly we want a food to pass- we have even gone too far with symptoms- although the doctors would not always agree. But that is what this hospitalization is about- to match the lab and vitals to the other symptoms we see when his body is not accepting a food; and hopefully learn form the corresponding symptoms to better guide future trials, at home.

11days ago, I let him have a few bites of some fresh, organically grown, boiled skinned, seeded zucchini. They were boiling in the pot and he called them a cookie and asked very sweetly for them. We had zero symptoms of concern for the next 18hrs or so. And then he had a liquidy diaper- ones we see when we know he is reacting. That same day, I had also started a new hemp protein powder- since the one he has been on for a year has been delayed in production, we needed to find a replacement. This one has plant extracts of sage and oregano- something he could potentially react to as well.

The diapers continued for 8 more day. Then, they finally stopped. The weird thing was that they weren't frequent- only once a day and for a few days, he wasn't even having one a day. And he didn't have too many other symptoms that would be terribly concerning- nothing building. So, my worry of it being the protein powder that he was still getting lessened and my concern that it was in fact the zucchini is growing. There is ONE other potential cause and that is a board book he sucked on on Saturday morning (2days after the zucchini). He's had full vomit reactions to board books before- I remember specifically because there were pieces of a board book in his vomit one time. But he didn't vomit, he had diarrhea for 8days....

The food trial next Monday will fill in the rest of this story...I hate that we have to, likely, make him sick but I don't know what else we can do at this point. We've tried everything else over the past year; and he not only needs a more varied diet, we need to know why this is getting worse for him instead of outgrowing. As with everything else, time will tell. And our Faith will guide us

Care conference and a plan

Today was the care conference. Long awaited, and worth the wait. It went well. We have a supportive team for Little man.  The main goal of the upcoming hospital stay is for food trials and to find Little Man a diet but also to see what is going on in his body as it accepts or rejects a food; what labs, stool tests, vitals, match to his crying/disturbed sleep/stomach cramps/irrational behavior.

The plan: Scope the week of Oct.10- check for intestinal healing from past reactions; as well as a repeat/follow up on his dissacharides (enzymes used to digest sugars in the body, last July this test revealed his levels as quite low).

Admission Oct.17 for ~3days while we trial Zucchini! He will have labs run throughout this process (before feeding, during, etc), vitals every 4hrs or so (blood pressure and temp), and stools will be monitored for signs. His labs will show that his body looks like it's fighting an infection if he is reacting to a food (we've seen this in the past but have not had a time when it is recognized as directly correlated). We will also have a chart to monitor the subjective symptoms; what we're watching for with those are a building pattern (as the body gets overwhelmed). Essentially- it's like he has the flu and is being asked to run a marathon - how would you feel? How would your body respond? That is the closest I can come to describing what I see happening to him.

We will stay in for ~3days, and then go home for ~5days (if he reacts, going home will be to rest; if he doesn't react, going home will be to continue the trial at home; having a baseline done at the hospital. We have no idea what to really expect and we have to start somewhere. So, this plan is where we will start.

Only time will tell and for now, we'll continue to enjoy this "down" time with him.

Admitted, what is the plan?

We go in for our planned admission.  We check in midday and the discharge planner has already been in - so that is all good signs...before we've finished admission paperwork, we're working how things will be lined up to get us out.

So, that is what this admission is for: to initiate TPN (and work through what he can tolerate of it), and then set up for home TPN. Our pediatrician reminds me it is not typically done but that it can be done (home TPN) but he is going to do the best he can to help arrange it for little man. 

While in the hospital, little man's daddy and I take shifts. I take the day shift and start after I drop the boys off at school, and Jeremy takes the overnight shift. It makes being there a little less hard. I miss the older boys when I'm here at the hospital and I miss little man when I am home. It aches how much I miss them, and our routine.   So, we are anxious to be able to go home. Our pediatrician has helped us with that, and agreed to help manage his care at home, while he continues on IV nutrition- in preparation for food trials. So we will be going home tomorrow morning! We will have access to 24/7 nursing care, but the goal is to teach us to do most of his needs- to our comfort level.

We will do the IV nutrition (he is still getting his hemp milk formula bottles as well- just less) for the next 2 weeks or so. The goal is to "beef up" his body, make sure that all his nutrients are at good levels, and his weight is solid, and his gut is healed. We will then be re-admitted in a few weeks, where he will have a baseline assessment done (that will likely include repeat scopes), and then we will begin food trials- monitoring his symptoms and labs. We will have a care conference and I have written up the discussion points, from the consult at CHOP.

I. Little Man's Classic FPIES:
1. Pallor
2. Blue lips and feet (cyanosis)
3. Lethargic
4. Body temp changes
5. Blood pressure changes
6. Vomit
7. Diarrhea

II. “Other” (not clear if FPIES building, or other; but concerning)
1. Mottling
2. Mouth itching
3. Extreme stomach cramps/pain
4. Disturbed sleep
5. Irrational behavior
6. Blood pressure changes
7. Body temperature changes
8. Significant changes in oral intakes (may be difficult to judge if on TPN)
9. Acidic stools (acid ring)
10. Inconsolable crying
11. Reflux/excessive gas


III. Labs:
1. Platelets elevate (although may be skewed with TPN due to heparin in line?)
2. White cells decline
3. Hemoglobin declines
4. Pre-albumin declines
5. Blood in stool (visible)
6. Change in body temp (high or low)
7. Changes in blood pressure
8. Diarrhea (green and mucus)
9. Constipation
10. Stool studies? Calprotectin, elastase?
11. Weight loss (may not be able to use if on TPN)
12. Scope (repeat scope at baseline for enzymes).


IV. Define parameters for:
1.Yielding food trial (a pause to step back and assess symptoms)
a. Worrisome and unclear symptoms, continue with ½ dose
b. Symptoms that are making Sam visibly sick (fever, cough, no sleep….), wait 3days and continue
c. Building symptoms – progressive and persistent

2.. Stopping food trial
a. Any presentation of classic FPIES symptoms (listed above)

3. Pushing through symptoms-
a. If symptoms align with enzyme deficiency, proceed with trial with Sucraid given with food.
b. ???

4. Pain relief during symptoms? Stopping a reaction?

5. Home or In-hospital??
a. Home TPN-ongoing but only between trials ( ½ strength)? To maintain Sam’s weight and safety during and between trials.
b. Food trial in hospital 4+days but go home in between
c. In hospital observation status vs. full admission (to allow for going home or at least out off and on??)

Those are my thoughts, from what I've learned about FPIES, what I've learned about Little man and where we are at in this journey. We are scheduled to discuss them in a care conference, before next admission for food trials.

Reaction? Vit.C deficiency? Anemia?

What is going on?  I don't even know what his body is struggling with right now. Is it his anemia? His Vit.C? the Vit.C pill we have been giving him since last week? The new beet root one?

He has been doing pretty well all week, gratefully and thankfully well. I don't even know what is going on but yesterday he was fussy a lot off and on, he wouldn't climb the stairs- just sits at the bottom and calls/screams for me. He took a really long nap yesterday and today (not a bad thing because earlier this week he wasn't napping at all!).  All day today, both Jeremy and I are noticing he is pale looking, purple eyes, and the most concerning symptom is this breathing- kinda a shortness of breath like he has been running around. He isn't gasping for breath, and his heart isn't racing- why is he doing that?

This is just one of those times when I don't even know what to do. Bring him in? Where- the ER? I go back and forth all day, I will look at him and just think - something isn't right and resolve to take him in/call in; and then he will start playing and then I feel like- maybe I'm just seeing things or over-reacting, maybe it's nothing at all....and than back again....around in circles, what to do?

And, of course, it is a holiday weekend...and he's going to be re-admitted this week anyway but will we even make it to then? Praying tomorrow shows me another sign of what to do....wait it out, or take him in, or what?!

Buckwheat

Buckwheat is a seed.  It is a seed of a flower in the fruit family.    It can be made into cereal and flour but it is not a grain, or in the cereals family.    I had high hopes for buckwheat... 

Even as I sit here and type- I am doubting the fail.   Why would I doubt the symptoms that were off baseline and adding up?   How can I doubt the vomiting that was choking him, and soaked through his clothes, including a winter jacket and carseat?

I was using a buckwheat flour to make his Merry Muffins that he has been enjoying (and being incredibly tolerant of me changing flours on).  The first day, he merely took bites of his muffin- which I was actually thankful for- so I didn't have to worry about taking it away from him when he got his "dose" for the day.   He mostly played with it, but I am ok with him playing with his food- sensory stimulation is just as important for him right now.   

Day 1: I began to see the RRD and spots on his bottom- I tried to think they were just residual from millet, or the orange pulp.    His cheeks got a little rosy and he was drooling within 2hrs of eating those crumbs, he also had some mottling and irritability.    I had a quick errand to run so I did that and he quickly fell asleep in the van -- it was only an hour or so before scheduled naptime so I wasn't worried about it but he woke up when we got home so of course he was moody for the rest of the afternoon, in fact he screamed for almost 2hrs straight- even inducing some vomiting from all the crying (or so I thought). But then he ended up taking a late nap, so thought we were getting back on track.  And then when he slept so much better than he has in months that night- I was hopeful.    He has been getting up every 2hrs. (not always to eat) and this night he slept a 5hr stretch!

Day 2: He enjoyed his muffins a lot more, and ate an increased amount (this is difficult to trial with muffins but just keeping his servings low and controlled),  he had some similar symptoms - but nothing worse so I was hopeful we'd get through all 4days of this trial, take the break we wanted to take, and then restart in a few days.   The afternoon resulted in another missed nap (not by my choice but his); but another late afternoon catch up nap.   And again a 5hr. sleep pattern (happy for sleep!)!  But only after some disturbed sleep in the beginning of the night, but nothing too out of his new "normal".  

Day 3.  He barely even touched the muffin,  I made him some muffin tops (cookies) and he took 1-2 tiny nibbles and then wouldn't touch it the rest of the day.   A morning diaper change reveals a very "shiny" diaper with mucous.  The symptoms again: more disturbed naptime, also- is he a tad hyper? Or is it the normal over-tired toddler behavior?   His mood is primarily happy but he is becoming less tolerant of things....a sort of Dr.Jeckyl and Mr.Hyde.....and I'm getting tired! :)  Holding him during an afternoon bottle (in another attempt for a nap)- I notice his look of pallor, he gets a distinct bluish/purplish tint to his upper lip, also his eyes start to have this same tint.  Afternoon goes on, and he is getting tired, well a 20minute nap for a 19mo. old will do that but he won't settle down for a nap.  His brothers had been asking me all day if they could go to the pet store...do a little window shopping for their new pet hamsters...it was getting to be a long afternoon so I thought a change of scenery would be a good idea, so I agreed.   

Little Man started his screaming tantrum right was we say we are getting ready to go...which is already odd because he LOVES to go "bye-bye"!  He proceeds to cry but we load up to go anyway- thinking maybe he'll calm down when we get moving.   The crying continues, and is curious as this is the 2nd time in 3days that he has had this crying episode in the van....these episodes he used to have ALL the time as an infant...so many days of SO much crying stress whenever we had to leave the house -- I simply dreaded going anywhere with him!  But it has been SO long since he acted this way in the van, in fact the van seems to have a calming effect on him....not lately.   I am trying to chalk it up to circumstance (crabby toddler from no nap), or mood/behavior (but he is actually a very mellow kid -- when not on food trials).  As I'm thinking and driving, the puking starts....then choking and more and more vomit.  He is covered.  I am still driving. No where to exit.  A mothers worst nightmare.  He does stop after 3-4 good heaves.  I immediately turn the van around and head home- not stopping to clean him up as it will only prolong the agony.  He's still crying, screaming.  We get home and I quickly get him out of his carseat and into the bathtub.  After getting him cleaned off, I got him a bottle and we sat and rocked- he calmed down. Finally.   He fell asleep after taking some from his bottle so feel he is doing good.   Was this a reaction?  Did he just choke from the crying?  Maybe.  I put the other boys to bed and I go to clean up his clothes and car seat.    His entire outfit was soaked, as if I had dunked it in his bathwater with him still clothed (I did not), his winter coat is soaked through, his car seat has a puddle still in it, and a puddle that leaked to the seat below.   The reality sets in.  I want to cry.  That is a lot of vomit.  This is the 2nd vomit in 3days, with such off baseline behaviors.   Buckwheat will have to be a fail, before it gets worse and we have a sick little boy.  

I am defeated and deflated.  We are missing something.  He should be able to tolerate more than just peaches by now.   I can't help but think we are missing something.    Is it his gut health?  Does he have yeast overgrowth?  And underpopulation of good gut bacteria?  Will a trial of a good probiotic be worth it, or will he fail that too?  What about the peaches I use to make the muffins?  They are a packaged peach product (Plum Organics)....I haven't called the company- just read the label.  But what if these are the culprit?   So many questions, still so many unknowns.  We have come so far and yet we are still spinning.   What are we missing?

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....

Multivitamin trial....the rest of the story

If you're tuning in to find out how the multivitamin trial is going....it's not.   We left off on Day 3, diaper blow out in the afternoon.  His formula intake was increasing and he was getting fussy but remained optimistic, until the vomiting....

Projectile, forceful, violent until emptied stomach contents...sigh....sigh...sigh.    This reaction reminded us a lot of when he was reacting while breastfeeding (trace proteins in my diet were likely culprits some days) because he was up for drinking his formula (he always was a comfort nurser too) and we feel this really helped him as he seemed to be doing ok.  Of course, he was tired but I wouldn't call it lethargic -- it's always hard to tell at bedtime because of course they want to sleep...it's bedtime and they just emptied the contents of their stomach...I'd be tired too.   But, his breathing and color were ok, and he drank a good amount of formula before going to sleep.  So, we were feeling it was a "minor" reaction (it was after all just trace proteins).   That is until the loose stools/diarrhea started....and they haven't stopped.   He was awakened 3x during the night for explosive diapers, and continued to have them all day Friday, but then they seemed to be starting to thicken up (he was eating really well on Friday).  So, things were looking good- his behavior was good and his color still looked ok. 

Things started to turn around nap time (what nap time?).  He couldn't settle in to sleep and I spent half the afternoon just trying to make him comfortable enough so he could find that rest his body was needing.   Little Man's daddy and I decide, we are done with the vitamin.   This was a "minor" reaction, from TRACE proteins and he is experiencing all of the all-too-familiar-going-on-and-on reaction symptoms.  Friday evening started to bring on concerns as his color started to look off.   We got stool samples to check for infectious sources of his diarrhea and we attempted to get a lab draw.    From previous lab draws around reactions, we have observed that he gets elevated platelets, and often has decreased leukocytes as well.  And then, there is always concern for his hemoglobin and his hydration.   No lab draw, all attempts failed and all we were left with was a very unhappy and tired little boy.   Having difficulty finding a vein, and difficulty drawing from that vein are very typical for Little Man around a reaction, also very typical among other FPIES children (from what other mom's share)....this is one of the reasons why an immediate IV for fluids is advised- not only for hydration but also because of the affects it has on the body only makes it more difficult to find that vein needed to start the IV fluids.   

We have learned another lesson about Little Man and I am prompted to push once again for more detailed needs specific to his condition to be in his chart....we should have gone to the ER with that vomiting.  But it wasn't severe, and he drank right after....but his body needed that IV.  But I have no doubts that doctors would have disagreed- his color was still good, he wasn't lethargic, he wasn't dehydrated looking, and he was drinking....we would have wasted our time trying to convince ER doctors not familiar with our son and his very rare condition, that the IV would help him.     Unfortunately, this is part of the nature of this diagnosis.  The unfamiliarity of it leaves the parents making choices and decisions on their own that would otherwise be better guided by experienced medical staff.   Little man has had more minor reactions than full blown, we are fortunate in that he doesn't always experience full shock- his body has had times of shock symptoms and also acidosis but these are not immediate- only after days of "fighting" and his poor little body can't take anymore, does he start to exhibit these signs.   Would the immediate IV help?  The thought (and experience from other moms) is: yes,it does.  But, again- you can't just show up in an ER and expect doctors to insert an IV on a child that isn't currently in shock, or even to the point of dehydration...yet.    How do we change this for Little Man?  How do we change this for all FPIES children?  

Typical allergies, with IgE mediated immune response, children carry epi-pens so that parents/caregivers are enabled to help their children, while still seeking out medical assistance.   Non-IgE (FPIES) children carry an ER letter that describes an full blown attack with the recommendations for an IV and maybe even steroids-- but so many times the attack stops by the time you get to the ER, or the attack is seemingly minor (although I challenge someone to watch your baby vomit as violently as FPIES kids do and call it minor- we have classified it as minor because we have seen, and heard of, worse).   Vomiting 50x in one hour, and your child's heart stopping- that is worse, that is "full blown"....so my Little Man vomiting 4x in 15minutes until his stomach is empty and dry heaving but then stopping- that is "minor".   But both scenario's need medical assistance, need the IV (and maybe someday there will be even better treatment options to helping stop the reaction) but for now- getting the toxin out of the body is the body's only defense mechanism.   And maybe the IV not only helps with hydration so the body can re-coop better; maybe it helps to "flush" the system.  It would be nice to know if this is something that would help our Little Man.   We will look to try and get his chart updated, once again, to reflect his needs-- should this kind of reaction happen again; and given his history, it will....

Navigating through these murky waters.....

FPIES is a journey.  A journey in very rough waters - and that is on a good day.   Always worrying about crumb ingestion, reactions to foods, trialing new foods, ER plans, specialist appointments, hospital stays, doctors recognizing a little known, but very real, diagnosis (and not blaming you for their nutritional state of labs that are off and growth charts that are mountain ranges), coordinating all this care can be overwhelming.   I am always on the look out for ways to make it less overwhelming, to have any help I can get to navigate these rough and murky waters.   Navigating the journey requires coordination from all team members.  A good captain knows his crew and has the tools to delegate the navigation.   We have been lost in these murky waters of FPIES without a proper crew and insufficient tools.  It is difficult to be at the helm and in the "belly of the boat" at the same time.  And yet, that is where we keep finding ourselves- and stretched too thin.  Or, as Little Man's daddy so eloquently put it...."you can't rescue people from a burning building and put the fire out at the same time, (by yourself)"....you need to focus your energy at one task at a time; and then refocus to keep things moving.   We are constantly having to shift our focus, when we really want to rescue our son....putting out the fire can should be the doctors part of this illness. 

Navigating murky waters with the diagnosis and knowing what next steps to take, with the coordinated care team, and now with food trials.    We are on day 5 of plums but Little Man isn't feeling well.   Day 1 went well, he liked them and he ate them himself; there were a few small concerning symptoms but nothing that we could pinpoint just yet.  We have to keep pressing on until it becomes more clear.  Day 2 was a break day (I was at work-unable to monitor possible build symptoms).  Day 3 was on track and he did well, again with a few questionable symptoms that we are watching.   Day 4 was uneventful...until he got a crumb.  We took a day off, he had the all-too-familiar rotten egg (sulfur) smelling "reaction diaper" with a low - grade fever but he slept a good nights sleep (through the night), and no vomiting.   Today, offered the plums and he didn't want them.   Are we still spiraling from the crumb?  Or is it the plums?   I managed a swipe along his lips this morning, but was not going to fight him....he has enough negative associations with food already, he doesn't need to be forced to eat (and we've already tried this with terrible results in the past, in our desperation to get him to take/tolerate foods, not knowing or understanding what was really going on inside his little body).  He has already shown me he can tolerate textures, and he can feed himself with a spoon.  We will let him guide his eating patterns as we have let him guide so much of this journey.   I don't think he cares for a "breakfast" meal, he seems to do better with a noon-meal.  I put away the bowl and decided to try again later.   Within 30min or so, his screaming began....high agitation settled by little- not even a ride in the car to take his brothers to school was going to calm him down!   We got home and I gave him some of his compounded ibuprofen and his morning iron dose, and just held him for a while - clearly he isn't feeling good.   Once he noticed his daddy home (day off work), he went to him for a comforting snuggle where he fell asleep.  Again, another "off" thing- we've been fighting him the past few days to take a nap at naptime and now he falls asleep at 9am in the morning!  He slept for almost 3hrs, stayed clingy, fussy and not drinking very well all day.   The diaper he had this afternoon was suspicious but still not sure what it, and these symptoms are telling us. 

Navigating these murky waters.  Sure would be nice to have a map....

Pass or Fail?

I thought I would know by now if Millet was looking like "pass" or "fail"....unfortunately if I was to "call it" now, it would be fail.    Last night, little man was only awake once but he was in pain when he was awake- and it was all-too-familiar discomfort, but after almost an hour - I did give him some of his compounded corn-free ibuprofen and he then settled right down and slept another few hours, only to wake up too early and too grumpy. :(  But, here again, not enough symptoms to "call" it but things are starting to add up with too much familiarity and beginning to make me worry.   I then changed a diaper, lets just call it "weird", and "concerning" but not fully a reaction diaper.   He remained grumpy while I got his brothers ready for school and I got ready for work.  

Now, begins the guessing game - is this from the baking powder with corn starch? The crumb or two he may have ingested?  Could be.   Is it from baking soda or cream of tarter? Doubtful but could be.  Or, worst yet- is this an FPIES trigger to millet or an intolerance to millet? At that point- I'm leaning towards intolerance and taking a break to see what that does to his system and see if the picture can become less muddy over the next few days.  I work 4 days this week, so won't be home to monitor him anyway so a good time to just take a simple break.  

Little man had a lab draw set up for this morning.  We've been getting frequent checks on his labs, with his FPIES being a chronic illness- compounding situations have been sneaking up on us but we are learning more and more about FPIES and how things affect Little Man.    These lab checks take a lot out of him and this morning was no exception- especially since they had to try 4 times to get the vein!  Poor little man.  Results came back today and a short discussion with our pediatrician helped to calm my nerves between his labs being off and his questionable build reaction, with me being at work unable to do much about any of it.  His hemoglobin/hemotocrit and Iron are holding but not improved at all; although the good news is that his reticulocytes count is improved- showing his body is nicely responding to the iron supplement.  It will take awhile (6weeks or so) to begin to see a tiny bump in his hemoglobin/hemotocrit and iron levels from this though....it will be a slow process but we still feel this remains a better option than a transfusion at this time.  We are beginning to check other nutritional panel labs as well, to be sure his homemade formula is still helping him thrive (because as far as pain free and baseline and weight gain goes, he is thriving on it).    His other labs look ok.   

But that doesn't change the fact that he needs a tiny menu to round up his nutrition of micro nutrients.  We are fighting hard to get him this.  But in the meantime, he can't afford a set back- so we have to continue to proceed very slowly.   We remain between that rock and hard place.....he needs more nutrition in his diet, but he can't afford a reaction.  Does anyone have a crystal ball to see what we should even try next?   Do we continue with the millet?  I still feel like we should- I have been going primarily on instincts this whole time, and letting Little Man guide us along.  He isn't reacting during the day and in fact, he may even be improved- behavior, sleep, comfort, mood, etc.  But last night/this morning threw me enough of a warning sign to signal my instincts to take a break/slow down until the picture is less muddy (teething pain? cold? reaction? hungry?).  Maybe put millet to the side and get a fruit but then again- left in the same place we were last week- he won't eat a fruit...millet he was eating (another sign that his body isn't reacting to it- he wants it). 

Today, I'm left with FPIES=Feeling Powerless In Every Sense; tomorrow will be better.  It is day by day and sometimes moment by moment around here.  And today, I am reminded of that.