"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? 

Love you through this

The first time I heard this song, I cried.  It (obviously) isn’t about FPIES, it’s actually about breast cancer but it made me think so much of what it takes to be an FPIES parent.  I am by no means comparing cancer to FPIES, but I find any illness can identify with the lyrics of a beautiful song.   Many times, there isn’t anything else but love to get us through.    Get us through the dark and lonely nights, holding your baby who is in pain that you can’t take away; get you through the multiple appointments trying to figure out the complexity of your little man’s FPIES, hospitalizations, procedures and tests, food trials and reactions, hunts for safe foods, pulling them through and out of failure to thrive…

I write this as I am sitting with Little man while he takes his bottle, which I do multiple times a day.  My day is planned around it actually.  Much like when nursing an infant, I would nurse and then know I had 1-2hrs. before I would need to be in a place where I could nurse them again- a private, quiet place- not shopping or out running errands, or driving; but able to meet their needs.   To this day, we still make plans knowing how far we can go between bottles. He will eat while out now, just less- so we don’t have all day or hours on hours to be away from home or he is set back.   He eats better if I am sitting with him.  Maybe it goes back to our nursing days bond, maybe it’s just simply that I’m mom.  Maybe he’s just very routine orientated.  But it is what it is, it is what he needs, it is love, and I will love you through this. 

Even with TPN supplying ~800calories in his overnight feeds, he still needs to eat every 1-2hrs. through the day.  Recently we have discovered some hypoglycemia (more about that in a future post, if I can ever get caught up here); so it makes even more sense that he thrives better on these small frequent meals. 

My 3^rd little mudpie, who is 4yrs.old, eats non-stop throughout the day (ok, maybe not non-stop but it sure seems like it some days!).   I had a realization recently that this habit (that is currently an inconvenience because of there being constant exposures to food, reminders of food that little man can’t have, while he watches his big brother eat) will someday (hopefully soon) likely come as our blessing- that to get Little man to eat enough throughout the day (when he has more safe foods and post TPN),also to meet his hypoglycemia needs and his disaccharide deficiency, he will need small frequent meals throughout the day.  Likely, the bulk of these calories will still come from his formula and now the addition of his “juices”.   Currently he only nibbles even his safe foods- either he is just being cautious or he simply does not know that he is supposed to fill his belly; or he is self limiting so that he doesn’t go over what his body can break down in one setting (being that he lacks every digestive enzyme to break down sugars/starches).   But one day, nibbles will turn in to servings, and servings will turn into snacks and snacks will turn into meals.   One step at a time, one day at a time, I will love you through this.   

Trying a new plan?

Zucchini was a frustrating trial and "fail"; fail in quotes because stopping it before full vomit was needed as it was making little man too sick.  It is disheartening to try a food and not add it to his diet, just as it is a BIG celebration when we can ADD food to his diet.   

For awhile now, I've had theory's as to what little man needs for HIS plan, An Individualized treatment plan.   Taking him down to elemental nutrition enabled us to see what he was still reacting to, corn fail and removing him from the corn syrup in the elemental formula helped to confirm that corn was a chronic FPIES trigger for our little man.   Removing that from his diet enabled us to add hemp, safflower, arrowroot and shortly after peaches and millet.    That is where we have been stuck.   Following his patterns, we learned that our little man has chronic FPIES- that his body doesn't react severely on the first exposure although it does act as a toxin in the body, removing the trigger food, removes the symptoms; but his body struggles to get back to a "baseline" (depending on how far we pushed what was toxin in his body).  Why would so many foods be toxic to his body?  Food that is meant to nourish not only doesn't nourish him but is toxic to him. 

Much of my research, and following little man's symptoms, reactions, baseline, his involvement in Dr.J's research, response to probiotics, anti-fungals, and antibiotics keeps steering me that his gut flora guides his reactions or tolerance.  Gut flora is involved in immune mechanisms in the body, I think this is where little man's FPIES is tied.  Influence and guide his gut flora, influence his tolerances?     When I was building his formula, I didn't trial each ingredient for days on end, we didn't have time to- I added them every few days.  He never had any issues with any of these ingredients.   Peaches were also an easy pass.   Millet puffs were easy, the millet flour was up and down and it did take awhile to settle that it was not going to FPIES but just finding a tolerance level for him.    Removing the millet and reintroducing it helped clear that.  

Little man is a "build reactor", but his safe foods have been safe from day one.  But we've also had foods that look like they could be safe and then they gradually get worse- each dose becomes less and each day symptoms build.   So, what if we shorten the the trial?  Moving forward before his body builds the reaction, before his gut flora changes enough to induce that response? 

The new plan includes just that, trial a new food for 3 days, but not pushing through any building symptoms.  If Little man begins to show his body is struggling, we will shelve the food and move ahead; if he is not showing any signs- we move forward introducing new foods.   What if he builds a reaction to a food?  Well, we don't know that yet.  It's been over a year since we have successfully introduced a new food so we need to try this plan....an individualized to Little Man plan.  

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.....

Am I doing enough?

I appreciate this blog.   I am a talker (in case you hadn't noticed).   My posts are often simply reflective of our reality, of what I have learned from it, and sometimes just putting out what we are going through in hopes I can learn from others who read and have experience, knowledge and insights to share.   I process through my thoughts by talking them out.   They aren't always positive thoughts.  We're all human, read what Little Henry's mom has to write about that.   As I read her words, this mom of 4 catching up on her blog as well, updating on how she and Henry have been doing; I marvel in how closely united we all really are. 

Our FPIES world has been difficult again lately as Little Man now turns 2 1/2 and we were supposed to be "growing out of it" by now....and in many ways I feel we are being pushed back to the beginning.   Little Man is thriving on TPN (IV nutrition), and I am so.very.grateful for that.   He has gained 6#- we are about to hit 30# now, as the geneticist that we saw last week pointed out, it is encouraging to see that his body knows what to do with calories, calories that his gut does not have to absorb (or malabsorb in his case).  It IS good, it is all very good.  But he is now becoming dependant on this TPN, he has less interest in even eating- how do we do trials when he only nibbles?   He is 2 1/2 and has no positive relationship with food except to give food to his brothers and watch them eat.  And I am struck with the "what if".  What if he isn't outgrowing this?   What if we don't find him safe foods?  What if we find him safe foods that he refuses to eat, or doesn't know how to?   What if his body begins to reject the TPN?   What if there is something more going on that we are missing?  What if there is some metabolic disorder that is compounding his FPIES that we haven't tested for yet?  What if this new normal is our normal now?  What if?  

I trust in God, it is the thread keeping my peace....I have inner peace knowing that His plan is being carried out.  My worry remains with what part I am playing in that plan.  What does He want me to be doing?   Am I doing enough? 

FPIES Island

An FPIES diagnosis can be like being on an island. At first, it feels deserted with you standing alone, when you start to explore, you find out there are others on the island, they help find food, and nourish your survival knowledge and coping skills, and help to feel a little less alone in this new world. And you are assured by others that have been there that there are ways off the island; and you know you too will someday find your way off, and you feel less alone. Then you need something from off the island, and you find a bridge or two and you take them, only to be thrown back to that solitary place, on your island. Bridges to nowhere, bridges that don’t work, bridges to solitary islands themselves. So little is known about FPIES, and Doctors trying to help and yet unable to in this clinical diagnosis with misunderstood mechanisms and complexities, are also on an island- an island often with no bridge connecting to anyone else to help the FPIES patient/family.

Too many times on this journey, we have found ourselves alone on this island. In the beginning, we didn’t know anyone else lived on the island and we didn’t know to look for ways off or invite someone else on for help. Then it became clear that our little man needed more than what we could give him, his illness was becoming out of my scope as a mother. That instinct I had all along that something was wrong, was now a burning fire, and the need to find more bridges to connect our island became the mission. We quickly learned that a clinical diagnosis, was easier to file under “mom not coping” (ie mom is crazy) than to say medical science did not know, understand, much less be able to help. We tried so many times to connect bridges between different islands, but the complexity of little man’s FPIES needed a strong bridge to a specialist….which, after much searching and persistence, we finally located that bridge. We were happy to finally use it and didn’t know if we’d need any other bridges.

Unfortunately, it soon became clear that we were in need of other things for survival on our island so we went in search of other bridges - only to be quickly sent back to our island- our little man has an illness no one wants to touch…..even though he has needs for these bridges to work, they do not. A corn allergy with an allergic symptoms to even an elemental formula is too scary for most to deal with, so it becomes easier to simply send you back to your island, even though this little boy still needs help. It is left up to the parents to find that way, keep searching for another working bridge. Some really, really long bridges are found- but the cost is very high and the journey long to use those bridges; especially when the shorter, closer bridges should work. Last week we found a bridge that other FPIES families have been using, we hope to be able to as well….maybe we don’t have to travel on the long, long bridges; but maybe we still will……

I don't mind the island, I just want off this island long enough to get what is needed for survival….which bridge is that on?

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?