Raising Awareness during Food Allergy Awareness Week!

In honor of Food Allergy Awareness week this year, I contacted our local news station, after seeing that the same network had done a short story on our friends in Massachusetts, I inquired if they were interested in hearing more about living with this rare food allergy called FPIES.  They were more than happy to help us raise awareness and provided a nice short piece, spotlighting Little Man.  You can view it here: Rochester boy suffering from rare food allergy helps spread the word.

Trying not to get too discouraged....or, too excited?!

Today was some of the same symptoms as yesterday but not a build in symptoms; actually if anything- they were less. I am still confused by the symptoms at all, if this is going to be a pass but I am so encouraged that things didn't build. Maybe what we are seeing is left over from the Alimentum he had this weekend? It wouldn't be the first time a trial got muddied by being too close to a fail or just from a food that caused symptoms.
 He got 3 doses today (2tsp at a time, in 3 different bottles throughout the day). His symptoms to note are: crankiness when waking this morning and from nap....but I have to remind myself that is not abnormal for him- he really struggles with this transition and I really wish I knew why.
 But the good news, other than that- he was his normal mild-natured self all day. His cheeks aren't as rosy/chapped looking but it is waxing and waning still a bit. The concerning symptom of the blue coloring (cyanosis) around his lips and eyes is still there- there was a point today I just couldn't get over how terrible he looked.....but he was acting fine! He took a good nap, and he has fallen asleep easily tonight. His bowel movements have been great (yesterday and today)- no mucus, no blood, no increase or liquidy diapers, no diaper rash, no allergy ring; but there has been hiccups- twice. I know, seems silly right? Well, he's gotten hiccups before a fail before. He's also gotten hiccups randomly just like anyone else so who knows if this is a warning sign or not.
 Either way, we press on. We press on encouraged because his appetite was good today- he ate his millet "cake" for lunch, and he had snacks through the day and willingly drank his bottles, and had dinner even! I made mashed potatoes and he LOVED them! I mixed them with his safe oil (Safflower) and his hemp milk. He ate a few tablespoons, and then he followed that with gobbling down 3 mini millet muffins and a whole cup of peach juice (peach puree + water = his safe juice). He then had a bedtime snack of 6 mini muffins. 

The next day started out well, actually really-really well. The two little boys played SO well together! And then as the late morning wore on, we started seeing him crumbling...little things were upsetting him, he wanted mommy or daddy right next to him, he was getting obsessed over things (he has odd obsession behaviors when he isn't feeling good), he had 2 bowel movements - that although they didn't have any reactionary look to them, 2 in a day is out of his norm, plus that "allergy ring" was showing and some spots around his diaper area. What is this about? I actually didn't go right to the ProViMin in my head....

This is where it gets really hard....when you start seeing symptoms but you can't be 100% sure if they correlate with the trial or from something else....accidental exposure, cross contamination, etc. My head went to the Potato Stix he had eaten that morning. We initially found Pic-Nix potato stix at a Fleet Farm on our way up north last weekend. Everyone was getting a treat and I picked it up and read the ingredients: potato and cottonseed or palm, or safflower oil, and sea salt. Safflower oil is his SAFE oil! Right under the ingredients it states that it is manufactured on a dedicated line- no line cross contamination risk! The risk with it though is we're not sure if cottonseed or palm are safe....and actually have had suspicions that they might not be (from other foods with them). But, I took the risk anyway...we're traveling, he's not eating.anything. on the drive....I wanted him to have something to re-stimulate his appetite and was excited to give him a snack, just like this brothers were getting. I took the risk, for him. He ate the entire can over the course of the weekend, no symptoms to note. On the way home, we didn't go the same way but discussed that maybe we should have - since that can was seemingly safe, we should stock up!! Although we do have a Fleet Farm down the road from our house. I ran some errands earlier this week and decided to get some more, from our local Fleet Farm. I brought them home and he wanted some, took a few bites and then didn't want to eat them. Huh, well- maybe his "jag" is done and now that we're home, he wants something else "new"....or maybe it is a different batch with a not-so-safe oil and he can tell? My radar goes up a little. But, he has been asking for his "star cake" so I've been making that everyday (millet muffins made with a small star silicon pan). With the addition of pure maple syrup and ground CheeCha puffs (for potato starch/flour), the texture holds up better than his mini muffins ever did and he's gobbling them up- by the fork-ful! I'm amazed at how well this little boy is using a fork and spoon!

Ok, this is getting long so I better get to the point! Yesterday, he asked for his "potato chips" first thing in the morning. I hesitated- thinking of how he hasn't wanted them and what if they contain a unsafe ingredient and we're in the middle of the ProViMin trial,... but he has been wanting to eat before his morning bottle and I don't want to discourage that! So, I let him eat some- he didn't eat many and then he wanted his bottle, we decided to up the dose of the ProViMin yesterday- he has been SO pale and he needs the nutrients so his body can make iron if we have any hopes of not having to have another IV Iron infusion or blood transfusion. He took his 8am bottle and his 10:30am bottle well and was playing well all morning and then he had a full lunch of peaches, a potato pancake, 4 mini millet muffins, and some more potato stix. After lunch, he took another bottle and he started to get really crabby, we hoped he was just tired and an hour later he took another bottle and finally went to sleep. He did fine all evening, although his appetite was decreased and he was a little clingy to daddy. We got home late and he went to sleep easily and then he woke up around 3am SCREAMING!!! Yelling and screaming and thrashing and then hitting and kicking and screaming! He woke up everybody in the house, he was just crazy!! It's 3am!! We finally got him calmed down but he stayed awake for at least another hour. The next morning he was very crabby all morning- everything causing tears...all out of sorts. He seemed to be getting better as the afternoon wears on, so I am still hoping my instincts are right and that it was the potato stix and that will just have to work it's way out of his system (the can went into the garbage!) and not the increased ProViMin (went from 2tsp/bottle to 3tsp/bottle- upping his intake to 1/4cup/day (end goal being 1cup/day of ProViMin). I am also worried about his anemia and fear we have to face it and bring him in for an evaluation soon- he is so pale and has been for at least 2 weeks now, and this disturbed sleep (sleeping but not acting rested and then being up in the middle of the night) are familiar anemia symptoms. Also, his tube looks terrible!! I'm ready for it to just be taken out...we can't use it and he's now complaining again of it hurting during the day, and who can blame him? It's so red and inflamed, it looks like it is going to push itself out!! Time to address it. Surgery has given their recommendation - to pull it out, that it is causing more pain and adverse symptoms than it is helping (for sure!). His doctors are not sure if we are there yet with it and still hope to utilize it. We are just not sure. We continue to pray that the ProViMin will work out.

It's gonna be a bumpy ride...

ProViMin is started and, as suspected- we are seeing symptoms. At first this morning, I was hopeful it was just because I am watching so carefully- to notice that he woke up crabby and only wanted to sit and watch TV and didn't want me to leave his side, or that his cheeks were a bit dry looking and quite pink, or that he wasn't wanting his bottle or his waffles.... We started the ProViMin last night at 6pm with 1tsp of the powder mixed into his hemp milk bottle, he then got another one at around 8pm, with the same 1tsp. powder mixed in. He woke up around 11pm crying and uncomfortable but we hoped it was just because he was hot as he had fallen asleep covered up on the couch. He woke up at around 4am but a bottle (plain hemp milk) put him right back to sleep. He was awake for the day at around 7am and finally got him to take a bottle at 8am. I put 2tsp of ProViMin in this bottle. The goal is to give him 6tsp. in a day, which is equal to 1/2 a serving a protein. Once we are sure he is tolerating this amount, we will move up with the end goal being 1cup of ProViMin mixed into his formula and foods throughout the day. I suspect 1cup will be hard to get to (A LOT of powder) but we'll cross that bridge when we get to it. For now, we need to convince his body that it does not need to fight this. It is disheartening to see symptoms- however subtle they may seem today- as his safe foods have been safe from day one (no matter the quantity). Hemp milk, the first day he drank 50oz., without so much as a hiccup! Peaches, the 2nd day of trialing them, he grabbed the entire peach and ate half of it - whole and raw...not one burp or any symptoms. With potatoes, he ate a half of a bag of CheeCha puffs on day 1 with no symptoms whatsoever. Today, with the introduction of ProViMin, we have symptoms. Red, red, rashy cheeks, blue lines (signs of cyanosis) around his mouth (most concerning symptom), some decrease in appetite, and a sensitive mood....but nothing over the top or unmanageable. So, we press on and through, starting again tomorrow and working within the 6tsp. dose....until symptoms either disappear or become more clear.

Potatoes and Good Days!

Ok, NOW we get to the good days!!   Despite all the granulation tissue and tube issues this week...we are having a good week because Little man is tolerating potato!!!    

It started last Sunday when we had decided we would discuss adding the beet root compounded VIt.C (it would be a trial) to his Alimentum feeds- even if we could only get one Ali feed in per day, we could use that feed to get him all his Vit.C (the biggest missing nutrient from his hemp milk formula and the amount of peaches he eats isn't enough to fill in the gaps when he is low).   We went to an evening church Mass and after church, we have potluck and it was right at dinnertime and someone made some really yummy smelling dishes- everyone was hungry!  Including Little man!  He took his place in the buffet line and grabbed a plate and fork and asked for food.   

Many meals at home are hard for him too, the smell of the food (natural instincts), the gathering of the family,...he wants to be included too.  And we WANT him to be included too- and for it to remain a positive experience for him, not one where he feels left out or deprived.   So, we've found safe ways to include him, but somedays (even at home) we miss the mark and either nothing is distracting enough from everyone eating and him not, or I don't have anything to offer him.    Today, at this potluck, was a combination of both.   People were gathering, I was there alone with the boys (hubby working) so no one to take him out to distract him + yummy smelling food + a desire to be included (kids his size and age grabbing plates)+ dinner time= hungry....but I did not have anything for him!!   I, of course, brought his bottles so I offered him that to which he said "yes" he wanted his bottle.   He claimed his spot at the table, fork in hand, with his empty plate.   I got him his warmed bottle and he looked around and declared he did not want his bottle.   I ran to the van to see if I had some millet puffs stashed away- he hasn't had them in a long time but maybe he would be distracted by this safe snack.  But, no puffs.  Shoot!   I went back in and scanned the buffet line, a clean fresh garden salad- lettuce, cucumbers, peppers.  He hasn't reacted to any of these foods, they aren't UNsafe, so I put some on a plate and hand them to him- he is excited at first but then not as distracted as I had hoped.  I decided, on a-still-don't-know-what-made-me-do-it-whim to give him some mashed potatoes!  I knew they weren't boxed potatoes, but that they likely did have milk and butter in them.   But I am still encouraged that him not having any acute reactions to Alimentum (a broken down milk protein he had previously reacted to as an infant) means that he is outgrowing/outgrown of his milk allergy (and the only way to know is to have him try it).   We aren't in any rush to try dairy because of fear that it would sensitize him to the broken down dairy in his formula but he hasn't had an acute reaction to it....I made the quick decision to take the chance - on potato and on dairy, and let him have a tablespoon or two.   On the back burner in my mind is his growing POSITIVE relationship with food- to be included, to try new foods, to learn to eat and thrive on eating.  FPIES doesn't get easier as they get older, the challenges just change.

I put a tablespoon on a small plate and he devoured it!!  Well, nibble by nibble in the way he devours food!   I nervously sat and watched, immediate reactions of hives or anaphylaxis aren't our concern but this could be a long evening and a set back....was this a weak moment or a good decision??   He asks for MORE, he ate another 1tbsp or so.   I decide I better find out exactly how she made them, and what ingredients were included.    Real potatoes, milk and butter....but she usually also adds sour cream and cream cheese (they are REALLY good and popular and she makes them every potluck!), today she did not.   She even comments on THIS being the reason why....everything happens for a reason.   

We got home and he was overtired, and had a few tantrums and I was getting really nervous that this was the beginning of a reaction....mixed emotions were setting in- guilt, anger, fear....I finally got him to settle down and he fell into a deep sleep.   Huge sigh of relief as I watched the clock.   It was 5:15pm when he ate the potatoes, typically it takes 6-9hrs (sometimes longer although he is NOT the norm because it is usually 2-6hrs) when we see a reaction.  11:30pm came and went, 12:30, 2:30, dawn, he woke up happy and ready for the day!  What??!?!!  NO vomit??!   No middle of the night choking on green vomit fest??!   

Ok, dairy ingestion aside (cross that bridge another day) but this is a GREAT sign for potato!!   We have done potato a few times in the past few months and some days it went ok and other days he had symptomes.   We had started with baked potato.   One  day, an unplanned trial (kinda like the one above) began when I had gone to Wendy's for dinner for the boys - I decided to include Sam and get a baked potato (plain of course), dinner "on the go" just like his brothers (they get the baked potatoes too)- he was THRILLED and ate a few bites.   That night, NO reaction symptoms and the boys started to think of ways to add potato to his diet.    The timing wasn't good and we decided to not proceed with a full trial since we couldn't risk a reaction at that time.    We came back to potato during Dec/Jan trials we were doing.   Baked potato worked once or twice but he lost interest quickly, so I found some potato chips NOT made with corn or vegetable oil (the oil used was sunflower or safflower; he is safe on safflower but sunflower hasn't ever been fully trialed.  We got vomit - FPIES bile vomit- from these chips, changed brands (still same oils), also vomit.  Was it potato or the oils?   The oils won't always cause a reaction in FPIES kids, but little man has had reactions to oils (corn) so we can't be sure.    

Tolerating the mashed potatoes is REALLY encouraging!    Monday morning, his brother asked for a snack and so little man did too- this isn't new....he often asks for a snack when his brother does, everyday hopeful that he will one day get to eat what his brothers eat.   Monday, I decided it was time to pull down the bag of CheeCha Puff snacks!!  They are potato starch, potato flour and salt.   They are puffs shaped like wheels, a company from Canada and you can only buy them online (Amazon).  I got 3 sample bags from the MN Food Allergy Support Group Fair this past fall from The FPIES Foundation samples CheeCha puffs donated to give out (cases of full size bags!).   Since you can only order them online, and by the case, I took advantage of the samples to have enough to do a trial before we buy the case. 

He ate half the bag the first day!!!!  He finished 2 bags by Thursday!!  He LOVES them, and has been eating them constantly!!  We SHOULD be able to call potato a pass, with such large amounts consumed with NO symptoms of concern....but I'm nervous to say "pass" just yet.   But, he's getting them as if he had....he couldn't be happier!!!   

So, there is our good days!   So many other things going on but he is tolerating a NEW food, a 6th food to his safe food list, something he can have in a convenient snack too! Something that he can eat in large quantities without causing issues with this carbohydrate intolerance (they are puffs of air), something thatmakes him feel included and BE included.  

Pressing through and ready for a break...

With the picture muddy, we decide to increase the dose to help clear things up.   The increased dosages start in that afternoon (Wed), and he begins to perk up even more, improved demeanor but he looks more pale and tired, and has some blue (cyanosis) around his lips.  I am very encouraged by his improved mood but still confused on the paleness and cyanosis episodes. 

We anticipated some symptoms, Little man’s body has not accepted new food in so long- and the plan was to press through….we will push through to tolerance or reaction (vomit/diarrhea/dehydration).  We need to know.   This isn’t a typical trial- it’s more of a challenge but not a challenge we can guess on, a challenge we need to be sure.  This formula could help sustain him for the next 2+yrs as his body outgrows the level of reactivity it has had, as his gut heals, as he grows.  We will press on.  We need to know.  

We also need to significantly increase his doses, a discussion with the dietitian reveals he will need ~50oz. to meet his Vit.C needs;  which he does not get in his hemp milk formula, the only thing providing Vit.C is his diet is peaches which he doesn’t eat daily.    The Alimentum will also provide some B vitamins that his vegetarian protein source (hemp) does not.  The B vitamins help his body turn the iron into oxygen rich hemoglobin and further help prevent his anemia.  We are quickly overwhelmed by this realization that the Alimentum will not be a supplement but pretty much needs to replace his hemp milk (in volume).

Wednesday night he continues to receive the feeds through the night, he has increased gassiness in his sleep and is tossing and turning a lot through the night….nothing that wakes him out of his sleep but definitely different than how soundly he has been sleeping for the past many weeks.  Thursday morning  brings a cranky and whiny boy in the morning but he eventually starts playing and plays well through the mid-morning.  During rounds with his pediatrician today, we discuss how we want to increase the Alimentum to get closer to what he is going to need and see what that does for his symptoms.   So, the first day (Tuesday to Wednesday) he got 8oz. of Alimentum (mixed with his hemp milk formula) via the NGT, the 2nd day (Wednesday into Thursday) he got 12oz. of Alimentum (mixed with his hemp milk formula) via the NGT and now, the 3rd day (Thursday afternoon to Friday afternoon) we want to increase to 24oz. Alimentum mixed with his hemp milk formula via the NGT (we divide this increase into 12hr.segments- building him up slowly). 

I also discuss that because of the symptoms he has been having, that are still not clear (don’t seem to be building but are still present as we press forward), we will need to try and rule out if what we are seeing is a chronic FPIES.  A recently published article outlines the chronic FPIES: “The CurrentUnderstanding of Immune Mechanisms of FPIES” (page 4).   Little man has more chronic FPIES reactions than acute FPIES.   Once we realized FPIES was what was ravaging his body, and removed his trigger proteins we saw dramatic improvements in his gut symptoms (chronic vomiting, diarrhea/constipation, etc)….reintroduction of these proteins results in the classic acute response (profound vomit followed by diarrhea and dehydration).  His worst trigger remains corn.  You can almost set your clock by a corn ingestion- even trace/contamination amounts.   So, to be sure he is tolerating the Alimentum, I propose we remove the potential trigger protein- going home for the weekend, get back to a baseline (be sure what we are seeing is stress or hospital related) and we reintroduce a dose on Monday – a challenge dose; followed by a 24hr.”feed” of the 50oz. he needs for his nutrition (to be sure he can digest this volume).    The pediatrician agrees to this plan and we press through Thursday, increasing the Alimentum by Thursday afternoon. 

That afternoon, he is very tired and cranky after his late nap, mostly just wants to be held, noticed cyanosis on his legs again, and also a “lacy pattern” red flare color to an area on his arm, and his skin is mottled looking.   He clearly isn’t feeling good but it is not yet clear why.  Then around 7pm, he finally has a bowel movement and there is clear streaks of blood in it.  We get it tested and it is positive for blood.    Blood in a the stool of a child with Enterocolitis is concerning but we press on; this will be evaluated later, especially if he continues to have them.  Our GI has been clear with us that she wants to assure he is fully tolerating the Alimentum before committing him to a Gtube – which is why we elected to the NGT, so we can do 2weeks full strength and then assess where he is at, with as sensitive of a system as he has- we know to proceed cautiously

Going into Friday, we’re getting ready for a break.   We are ready to go home.   We are still concerned about the blood in his diapers and other symptoms but hope that this break will help us see things through a clearer light. 

He is SO excited to be home!  He even asks for his peaches and eats about a 1/4cup of puree peaches!!  He is a little clingy in the evening and struggles to calm down to sleep but he slept well overnight, and awoke looking more rested than he had been in the hospital- maybe it’s the lighting?  Maybe it’s just the comfy bed at home vs. hospital atmosphere.  This little boy is glad to be home and plays well with his brothers.  His diapers do not show any signs of blood through the weekend.  By Sunday evening, we are seeing the effects of not drinking enough of his hemp milk formula.  He used to drink 64+oz/day before TPN, meeting his 1200-1400 caloric needs/day; but he’s not used to having to drink that much since TPN provided him 800calories/day, he dropped down to ~30oz.; so now he is only back up to ~45oz. and it is clearly not enough.  If he were to stay on hemp milk formula alone, I’d have to go back to setting my alarm and waking at night to feed him through the night to get him back up to his daily requirements (I did this for 2mo. after his initial TPN in Dec.2010, until we started probiotic and his appetite increased during the day….I would do it again if need be.    But we are heading back on Monday to rechallenge the Alimentum RTF.  

Muddy Picture

We knew we’d have things that would come up in the trial/challenge to muddy the picture, we did not anticipate Little Man completely refusing the formula and the decision made for us to starve him to get him to take it.  We did however anticipate the NGT (nasogastric tube); we had discussed it previously with the GI- how he may need it to do the trial, to be sure he consumes enough for us to call it “safe”, we also discussed a Gtube (a feeding tube surgically inserted into his stomach) if the Alimentum RTF was an FPIES pass but if he does not accept it…we know it will at least need to be a supplement in his diet. He has hemp milk that is providing the calories and protein and fats he needs, but it is lacking in some nutrients- the nutrients the Alimentum would be able to provide.   We haven’t done the math yet on how much he needs of the Ali. 

The Alimentum RTF trial continues and Little man continues to get round the clock “feeds” via the NGT of the 1oz. Ali+3oz. Hemp milk mix. He appears to be tolerating it well and in the first 24hrs. with 8oz. of Alimentum in.  This is a good amount, but because of the events of the day before , prior to the NGT, we are no longer at a baseline  and we’re not sure if it’s the Alimentum slipping him downhill or simply his body trying to recover from the previous days’ events.    

We are optimistic of his progress, Wednesday morning his cheeks are rosy, he slept well overnight, wakes up ready to play, and enjoys some playtime  when his brother visits.    So, we began to discuss the next 24hrs.  at rounds on Wednesday morning, we express and discuss how we feel we need to increase the Alimentum he is getting- because of the symptoms he is presenting with that are unclear if they are building FPIES or leftover from the stressful beginning days.  I am hopeful it is just the stress, and am encouraged that we haven’t seen any of his “classic” building symptoms of pain, agitation, decreased appetite and disturbed sleep.  But I am troubled that we jumped right into lethargy, pallor and cyanosis.  But, even though these are seen during an FPIES reaction (acute or chronic), they alone (without vomit or diarrhea) are not defining criteria; and especially not now for little man because of the stress his body has been under.   So, we press on as planned but decide that we should continue to increase his doses to see if that makes the muddy picture clearer.  We will increase to 2oz. Ali RTF + 2oz. hemp milk for the next 24hrs. 

He'll eventually get hungry enough....

Little Man is admitted for the formula trial, and the hopes to find him a supplement to his hemp milk formula.   We have decided on Alimentum RTF for that trial because although it contains casein (dairy) hydrolysate and tapioca starch as well as a tiny amount of soy oil, and also DHA/ARA that is becoming common place to have in formula’s….it doesn’t have corn.  And believe it or not, all of these risks are less than his risk to react to corn.  He has become more sensitive to corn instead of less over the past 2yrs., but the hope is that he is becoming less sensitive to some of his other original triggers.  The trial is a challenge and will be done in the hospital.   Little man is a chronic reactor (delayed reactions) so we also want to document any symptoms he has along the trial, even if they don’t lead to an acute FPIES reaction.  We get admitted on Monday and get all settled in, reviewing the plan with the doctors, and attempt to begin giving him the formula.   We decided a 1/2oz. mixed in 3oz. of formula may disguise it enough to test it out.   I was wrong, there was no disguising the taste of this formula and little man noticed it immediately and was not happy about it!   It is a long afternoon as he becomes more and more hungry and frustrated, and keeps asking for his bottle – to which we give him the 1/2oz. Ali RTF and 3oz. hemp milk mix.   His daddy arrives for the night and I am hopeful a new approach will help.  Daddy tries Ali hot, cold, over ice, in a special sippy, through a straw little man drinks about few swigs before he realizes and then refused.    We try to be optimistic that he even took the few sips but we are concerned over his refusal and hunger.  It really isn't something we are comfortable with, it isn't why we are here (in the hospital)

We request to speak to the Pediatrician.  We've seen little man starve himself before and we've watched him spiral down fast....of course this is after a reaction when he's already very sick; but we're still very worried because of his yet unexplained hypoglycemia (low blood sugar) issues he's had in the past.   If they have to give him dextrose to keep his blood sugar stable, he may not feel hungry and the dextrose (being from corn) could "muddy" the trial (he's had it in the past but with questionable symptoms).   So, we are advised that "he'll eventually get hungry enough and eat".   Well, I know that is an old saying for "typical" kids who are being picky; but it doesn't work that way for kids whose food causes pain.  We have had hospital admissions for this very reason, "he'll eventually eat when he gets hungry”, they feel he will eventually accept the Alimentum mix if it is his only source of food.   We do not agree with this recommendation because of what we know about Little man but we agree to give it overnight.  The main concern beyond him being hungry is that this isn't what we came for- to starve him, to dehydrate him, we came to challenge a formula- to find him food- not take it away.  

Thankfully, his daddy does get him to "dream feed" in the middle of the night- he takes some of the mix in his sleep.  THAT is a good sign and we are hopeful; but it wasn't enough.   He has one wet diaper in the morning and then not again all day, his lips are getting dry and his eyes are looking sunken.  And he is NOT happy.  He refused any attempts or mention of the bottle….and we fear he is gaining mistrust in something he has trusted for so long- his one stable food source, and a true comfort item.   By the time the doctors rounded (late morning) it now had been 22hrs and he's had 6oz. to drink (1.5oz. of Ali RTF).   

We discuss the nasogastric (NG) tube.  Our GI discussed this with us before we came to the hospital- how we may have to do the trial with the NG if he refuses to drink enough; we would then know if it was "safe" and see if he would drink it (while he maintained his NG); if he would not drink it; we would have to do a Gtube (surgically inserted into his stomach) to assure he gets the nutrition he needs.   This does seem drastic when he DOES eat by mouth, he takes his bottles VERY well and can drink up to 60-70oz./day without a problem, it is adequate in calories but lacking in some nutrients.  All he needs is supplemental nutrients- that he can DRINK.  But we have searched and not found these in 2yrs.....

This hospital stay was already becoming more complex than we had anticipated (and we anticipated complexities!).   So, right now, we need to focus on today.   Today, he needs nourishment.  Today, we need to find out if the Alimentum RTF is "safe" for him. Today, we need to make the decision to have the NG tube inserted to give him Ali RTF through it and let him have plain hemp milk in his bottles for his comfort.  

We make that decision and we head to the procedure room with the nurses to insert his NG tube.   After it was placed, we had to get it checked on xray to be sure of the proper placement, during that time- we offer him a bottle (of plain hemp milk) and he, thankfully, accepts it and instantly starts to (finally) feel better and drift into sleep (for a nap)….HUGE sigh of relief.   He's been through SO much already and we've barely just begun.  

X-ray showed that the tube was not placed in his stomach, so back to the procedure room we went.   This time was successful and the first feeding of 1oz. Alimentum and 3oz. Hemp milk went in around 3:30 on Tuesday.   The plan now is to continue "feeds" of 1oz. of Ali + 3oz. of Hemp milk every 3hrs for the next 24hrs. and see what/if symptoms present.  

The other thing that the X-ray showed is that his PICC line is not sitting in a good place, it is decided that it is no longer safe to use for TPN.   We will keep it placed in case he needs it for hydration while we work through this trial/challenge but plans are made to have it removed soon.  

Buying time....

So much has happened in the last few weeks, we are still trying to process through everything (in a place where changes are still happening).    The last update I gave was that Little Man was scheduled to have an endoscopy and covert his PICC to a Port (for IV nutrition).    Little Man has been on IV nutrition since Sept., 6mo. now.  We gave him gut rest and a chance to catch up- on nutrients such as Vit.C and iron but mainly on growth- he gained 6# on TPN!  He had 3mo. gut rest and then we tried zucchini, which didn't go well - not a full reaction but didn't see the benefit to push it because it was making him sick, FPIES or not- we needed to set it aside if we wanted to trial something else.  Such is the way it has been with too many foods for him- they cause symptoms that could lead to FPIES, or not, but he is not tolerating them and the benefits do not outweigh the risk.   By not tolerating them, I am not referring to a reflux flare or a rash or even some mood changes- I am talking about a whole different child emerging, with a wide range of symptoms (depending on the food), with random throwing up and questionable stools and just a whole heap of a mess of a child that is surviving each day....and of course not to mention that he shuts down to eating when he starts feeling like this and associating the pain/discomfort/vomiting with the food. Despite that, we pushed through several foods and got no where, except increased vomiting, no weight gains, a dramatic drop in hemoglobin necessitating a blood transfusion, again...after almost 4 weeks and with that drop in hemoglobin- we waved the white flag....we needed a time out.    An evaluation on what was going on and where we were heading.

With the scheduling of his surgery for a port, we wanted to be sure we were making the right move.  We called the allergist we recently started seeing and asked for an appointment before the scheduled scope/surgery - she agreed and got us in.    We also got an appointment with our GI.   As our Allergist put it well, "there are too many cooks here and no one is following the recipe".  We needed to get everyone on the same page before we put him through surgery!   Well, as if in a twist of fate, little man developed fevers 2 nights before his scheduled surgery- and we found ourselves in the ER being evaluated for another line infection.   In the ER, he was found to have increased white cell counts and he vomited everywhere....now I was sure he must have a line infection (sepsis)....but after waiting for 48hrs to see if/what cultures would grow and finding nothing- we suspected he actually had an FPIES reaction - although we still do not know to what!  We spent 2days in the hospital and his surgery was cancelled but his scope procedure was kept.   The scope results initially looked good- and we were hopeful we were finally going to see healing but the next day when the biopsy results came back, we found we were wrong- he continues to have blunting of his villi.   Although they are not as bad as they have been previously so we are hopeful they are healing, but healing takes time....

With the scare of a line infection, surgery was cancelled and then further discussed- was this the road we really should take?  At this point, what are his risks of being on IV nutrition vs. his risk of trialing a formula?  Either a modular non-corn elemental (we've searched and struggled for 2yrs to find that!) or Alimentum Ready to Feed (RTF). The question is- the risk of surgery and continued TPN or the risk of reaction to Ali RTF?  Which one is a greater risk?    With catch up growth established, nutritional status stable, line infection negative, and - most importantly- 2years since any trace of dairy protein was ingested and reaction from, we decided to trial Alimentum RTF.

Alimentum RTF has a casein hyrdolysate (a hydrolyzed "pre-digested" milk protein), but even more importantly for our corn allergic little man is that it is the one of few formula's NOT in a corn syrup base.  It has tapioca starch as the base.   Little man has previously reacted to tapioca starch but it has been 18mo. since that and we have never been sure if that was from his disaccharide deficiency or if it was an FPIES reaction- it was severe and he was hospitalized because of metabolic acidosis from the dehydration and dumping diarrhea.   So, this trial is a challenge - a challenge of his FPIES, a challenge of these ingredients.  It doesn't come without risks but we weigh the risks of keeping the IV nutrition and this challenge with his GI doctor and we decide it is worth the risk.  It is time, we have bought time by moving away from all dairy derivatives at 6mo. of age when we realized he had more than "just" an intolerance to it as he continued to get more sick as he got older and introduced more foods.  We have bought time by having him on TPN (IV nutrition) for the past 6mo. to heal this gut, give him catch up growth, and make him stronger.   His body has shown us with recent trials that he still has high re-activity to new foods but we remain hopeful that this could be something his body would accept.  

For us, FPIES has been about doing our best to keep him thriving while buying time....

Sunflower Suspicions

Sunflower derived Vit.E was in the Living Harvest hemp protein powder that we used to use (until they discontinued it earlier this year).  I always had a reservation of how he was doing with that derivative but until I found the Manitoba Harvest brand powder he is on now, we couldn't move away from it –and the symptoms were never obvious or severe enough to do that.    Recently, I tried the potato chips, with caution because the oil was sunflower….still not sure about it.    Following the potato chip vomit, the suspected pineapple, and then coconut, we decided it was enough.  We were done trialing anymore foods- he needed to rest and his gut needed to recuperate; and we needed to regroup.  We aren't getting anywhere but a sick little boy.

Then he had a vomit session after sucking on some bathwater, that has happened before but not since he was on formula; and with formula he was chronically reacting to the trace proteins of corn which was making him extra sensitive to his environment.  So, was it from the recent fails?  His body was on high alert?  I suspected so, but was incredibly discouraged by it because it seemed to really make him sick…one reaction to much for his tired body.   I am saddened by seeing him like this, saddened he had to get to this sick.  And now to see him react to bath water….from a bath he shouldn’t have had but he wanted so badly….The PICC line is in his chest, it is difficult to wrap in a way that a 2yr.old would not get it wet and getting it wet would increase the risk for infection; so it has not been worth it.   We do sponge baths and it is sufficient to keep him clean but a little boy just wants to play in the tub! 

Well, 2 nights after the bath water vomit session, he had another random vomit session, and then 2 nights later- again.  What is going on?  As I’m cleaning him up, and washing sheets and just sad that he is this sick again- this sick that he hasn’t been since he was 7-11mo.old….when he was having chronic reactions to his formula; but he hasn’t been eating anything but his hemp milk.  His hemp milk….I took one out of the cupboard and read the ingredients…wait, how long has THAT been there?   Sunflower Lecithin?!  Is this what the random vomits are from?  Is he sensitized to sunflower enough to have him having these vomit sessions from it in this form?   A lecithin is the fat from the oil, “technically” it shouldn’t have proteins and shouldn’t cause an allergic reaction; but FPIES doesn’t follow those rules and avoiding the food in any form, trace and contamination of is advised - especially when you are seeing random vomit sessions.  Now, could it be from a sensitive system from all the repeated fails of the past weeks?   Quite possibly, and hopefully but I dig back to the back of my cupboard and find 7 cartons of the some other hemp milk from before I bought this new case; sure enough it does not contain sunflower lecithin so this is a new addition.   I contact the company to get more information about it and confirm it has recently been added- it will help the product not separate is what they tell me.   The switch back to the old version resolves the vomiting....which is both good and bad, good for little man; but not a good sign for the sunflower lecithin being the culprit to his problems.  C.R.A.P, maybe it’s time to panic now?  

Now he was getting more sick by the day, his lab draw that week confirms it is because his anemia is bad again- and his body is weak.   He can’t tolerate the trial of the sunflower lecithin and some friends suggest buying out what is on shelves in stores now, and shipping it to us.   I posted this on my facebook profile and the response has been amazing!!  Friends and family are checking shelves all over, finding a carton here and a carton there, a few dozen cartons are found and so far we have an almost 3mo. supply!!  We have the chance to get him well again before we need to address challenging the sunflower lecithin; or switching him over to a homemade hemp milk (can be made in a blender and then strained –we’ve tried it but the seeds are still not small enough for his bottle nipple.  He’s 2 ½ yrs old so really could transition off the bottle but it is how he gets his hemp milk, his nutrition, I had no intentions of even trying to change him over but maybe we will need to.   Thankfully though, we can do it when he is at a better baseline, which he is not right now.  Thanks to some amazing family and friends….

What did we learn?

Hopefully we learned more pieces to his puzzle placement and that what feels like 10steps backwards, might be a few steps forward?  

We had the recommendations from the GI that she wanted to try and push him, to push the food trials and stimulate his gut.  Has his body just forgotten what to do?  We took food away to go to elemental nutrition (and gut healing) at 7.5mo.old.  We were able to introduce 5foods within a few months, and then everything stopped.  Why?  She wanted to get a better picture of how his gut handled food before we commit him to longer term TPN (IV nutrition).   So little is known about FPIES mechanisms and what is known is more on the acute responses to 1-2 triggers.  The chronic reactions to multiple foods is complex.  Our GI hasn’t always known what to do to prevent his FPIES reactions while finding him a diet; but she has gotten him through some rough times, and she has advocated for what he needs.  The TPN he is on has literally saved his life.  Do I wish things were different?  That someone knew how to help him more?   Do I wish we knew what to do next to avoid having to make him sick first?  YES!  But little man's FPIES is complex, so even when we get one thing under control- something else sticks out of the box….and the harder we try to shove it all in a box- the more it sticks out!  There aren't any protocols to follow, we must simply follow him.

In doing so, we have learned with the TPN, that his body knows how to utilize most of his calories- there are a few nutrients that remain an open ended mystery still but have become more clear with the TPN supplying them – knowing that he is not malabsorbing them (or being malnourished as some may suggest). 

On TPN he has steadily gained weight (from Sept-Dec) and is now 30# (up from struggling to maintain 24# when we started).  He now gets consistently 1200-1400calories (800 calories TPN, 400-600 hemp milk formula).  It goes to show how much he was actually malabsorbing because his hemp milk formula intake was always above 1400 calories a day and he wasn’t gaining weight-  he would gain weight between reactions, only to lose it and lose ground again, during/after a reaction.  He always had these setbacks.  But the food break, with the IV nutrition has given him a chance to catch up- in so many area’s but the most obvious is his weight.  He also sleeps (mostly) through the night, he talks more, he gets to just be a kid more, less ups and downs from his chronic state.  With 6# gained and catch up growth, we knew he was ready for the food trials.   If his reactions have been because of his nutrition, we would now see if that was part of what was complicating things….and it really could be- I know this.  Micronutrients are important and he has been missing or malabsorbing many for so long- maybe a missing “link” in the chain of a cycle has affected the way his body can accept food?  Maybe it has complicated things? I would loved if it had turned out to be that "simple".  But, what we learned in the past month is that his malabsorption clearly made it harder for his body to recuperate but it hasn’t changed the way his body reacts (over reacts) to food.  The reactions, if anything are quicker now- which may be related to the TPN or his age; his body may be maturing?

TPN has given him IV hydration following a vomit session.  IV hydration is the treatment of an FPIES reaction at this time- sometimes a steroid can be given but that isn’t often used, first line is the IV hydration.   I have always wondered what IV hydration would do to help Sam’s reactions- he doesn’t go to full shock and he usually eats within 2hrs. of vomit, so we typically have not wanted to bring an otherwise healthy looking kid to the ER; however we always seem to regret that as the days that follow and he clearly is struggling to recuperate.   With the past month of trials, and subsequent fails- we have seen clearly how well the IV hydration helps him.  And yet, he still has struggled with the repeated reactions; so it doesn’t take away that his body still needs rest in between reactions to fully recuperate.   The IV hydration has made a huge difference and I am so grateful for it.   Without it, without the TPN- I would not have wanted to push him like this...we'd have been in the hospital by now for sure if we had!

In general, these trials and reactions have reminded me a lot of when he was still breastfed.  He would recuperate after a vomit or diarrhea session by comfort nursing- to the point that he was doing it all the time as we kept trying to introduce new and different foods; and that he was getting more and more behind- which is how we finally started to see he was really sick….because otherwise he was maintaining- he was getting the hydration and nutrition he needed, from the breast milk- just enough to keep him going.  

TPN has helped him to maintain his hemoglobin levels- he struggles with anemia for most of his life.  The break from foods (Sept.-Dec) he was able to maintain his hemoglobin with little variations.   The week of zucchini trial, he fell an entire point on day 2 of the zucchini trial.  He was able to come back up on that point as we proceeded slowly through zucchini but by the end of the trial, he was down that point again (further showing how he needs slow trials.   We (I) watched that and noted that during the zucchini trial- that his body was keeping up with recuperating really well; but the more we pushed the less it was able to fight.  We pushed through other foods trials and he kept gradually falling.   ON TPN- he is barely able to maintain his hemoglobin levels – getting all the nutrition he needs, that does not go through his gut.  Wow! Enlightening and very scary all at once.  Throw trials into that and he struggles, ON TPN!  Why is this happening?  We still do not know other than severe malabsorption.    We just had his labs checked again, 2 weeks later his hemoglobin is now down to 7.1 and he is in need of IV iron or a blood transfusion- it did not come back up on it’s own like it did with zucchini, like we had hoped.  

Where did the month "push" trials get us?

Not far, backwards?

No, I've learned that all steps forward are just that- always moving forward.  Even if we can't see the progress in the moment or when it feels like a dozen steps backwards....always moving forward.  Faith that even when we can't see or understand something, there is always a purpose.

Since the last post of pressing through pineapple and on to potato, as we feared (but hoped not), he started reacting- but to what, we aren’t positive.   We were using potato chips for a potato trial, trying to get him interested in eating. He’s been surprisingly good about chewing food- even if it is in small tiny nibbles, for a kiddo who has had so little practice with foods. But he just isn't interested in trying new foods, it doesn't take much to make him leary of new foods, or even leary of his safe foods! 

I was mixing the zucchini into his muffins and his cookies; and then the egg as well.  He had vomit reactions to those and isn’t interested in his muffins now.  His cookies he has asked for but is rarely eating.  So, I thought for our potato trial, we’d use chips- they come in a bag and look like something his brothers would eat, ok- do eat.   He had them for a few days and then had a vomit session that we assumed was from the potato chips.   It was a rough vomit session and he wasn’t up for eating anything but his bottle for a few days…not even his pineapple juice.   Then, I made him his muffins- in an attempt to get him to accept them again and start trusting his food again.  These are from his safe ingredients- a recipe I adapted from a fellow mom's Merry Muffins and use as the base for pretty much everything- “biscuits”, “cookies”, waffles, and muffins.  Combining his limited safe ingredients, they make a cute little muffin.   He LOVED it and actually ate almost a whole one (a mini muffin in tiny nibbles that took him about 2hrs to eat), he also drank his pineapple juice.   Six hours later, he vomited.    The next day, he of course did not want his muffins but he did accept a few sips of pineapple juice.  Six hours later, vomit.  Ok, wait?  Is this a reaction to pineapple now?  Is this pineapple too rough on his stomach after potato fail?  Was potato actually a fail?   So.many.questions.  

He needed a few days to recuperate and our GI agreed.    The rule with an FPIES reaction- you wait until you are back to baseline; but we have a few factors in this "new" plan that we are supposed to be “pushing” him, he gets the TPN that helps his body cope following the reaction (the IV fluids he needs), so he is bouncing back much quicker and our GI wanted us to do this method for the next month- push things.  So, we started again 5days later, because he had been asking for his ‘chips’ almost every day.  Why is he asking for something that he reacted to?  He never does that.  Maybe it wasn’t the chips and it was the pineapple?   Only one way to know….so I tried a new brand of chips- Kettle.  The ingredients are: potato, and the oil is Safflower and/or sunflower (I wish it was just safflower like it says on the website), and sea salt.   He had them one day and did great.   The next day, I wanted to test out my new Vitamix and make him a juice, which I did – and combined a few fruits and veggies into one juice.   Ideally, this would get us the nutrients he needs- a pound of spinach condenses down to a little juice and he could get a lot of nutrients by juicing.  It also gives us the opportunity to see if “pushing” his system will “stimulate” his gut and give us more clear answers to his multiple symptoms and reactions.  He took 1 sip, pushed it away and asked for his chips.  Yesterday the chips went ok, so I let him nibble on them again that day (the juice was avocado, spinach, melon, carrot, radish and peach- very small amounts of everything except peach).   He has had carrots a few times over the summer but has not had any of the other fruits/veggies.   That night- vomit.  I automatically think the potatoes are the culprit but it wasn’t following the potato chip timeline from the previous week…maybe it was the juice (carrot)?    How much further could we push him?  I want to stop, but I feel like it would be giving up….that we still have so many questions and did we fully follow through with the GI’s plan with these foods/trials?  In there we had also tried golden beets in his millet muffins, also Enjoy Life chocolate chips (which I have since found out have caused reactions in some corn allergic people so those will need to be taken out- which is fine because he wasn’t even interested in them! So, over the past month or longer we have tried a pretty large variety of food but we are getting nowhere, Is this enough?   

I was at the grocery store and picked up a few other things, some coconut milk in little juice box cartons – he thought that was pretty cool.   Of course, again with the processed food….but he really is losing interest in trying foods.   At first, he was excited when I was putting new foods on the table for him- the look on his face, as if he was saying “is my FPIES over? I can eat?”….because he didn’t want the foods I was offering, he was BEGGING for crackers, that his brother eats a lot with his lunches, he begged for fruit cups, cheese and yogurt- the snacks his brother eats….So, I got some SoDelicious coconut milk.  And again, he acted like it was Christmas morning- so excited over his own juice box!  “mine” and over and over again he said it.  The first night went ok, but enough questionable symptoms- I didn’t want to push him too much further after all he's been through and because coconut has been on the fence for almost a year since we’ve tried it a few times and don’t get through the trial for symptoms that show up- but always different and possibly not FPIES.   The next day, he asked for it again and I let him have some….6hrs. later, covered in vomit.   As with other nights, he falls asleep soon after the vomit and the TPN (IV hydration and nutrition) clearly helps his struggling body.   But even still, the next morning, he is really looking pale and off color.  I’m done, we’re done.   This is it- we’ve gone far enough, too far.    What have we learned through this?  

TPN

Little man has been on TPN for 5monthes now.   TPN is total parenteral nutrition and it is nutrition through a PICC line (Peripherally inserted central catheter), which is inserted into a vein.   So, he receives his nutrition through a vein.   The disclaimer here is that although this is part of our Little Man's FPIES (and what this blog is for), this is not typical for FPIES.   Although, it isn't uncommon for some kids with several FPIES allergies to have G-tubes, which is a feeding tube inserted into the stomach to allow for formula to be fed through the stomach; this is typically due to several severe reactions causing the little one to refuse to take enough nutrition through the mouth, or have complications from severe reflux necessitating the nutrition via a tube inserted into the stomach.    We have previously discussed a G-tube for little man but it wouldn’t change that his body does not accept any commercial formula’s; so the benefit vs.risk of a Gtube has not been enough for us to pursue it.   Little man’s FPIES results in severe inflammation, inflammation that doesn’t seem to turn off (is this an auto-immune component or simply his FPIES, we are not sure).   The inflammatory mechanisms have caused persistent small intestine villi damage; again we do not know why.   

Little man received temporary TPN in December last year for a month after soy challenge revealed villous atrophy, weight loss, malabsorption.  We knew his body benefited from the nutrition via IV.  That his body knew how to utilize these calories and bypassing the gut meant no malabsorption.  

This summer when little man had severe Vit.C deficiency resulting in Scurvy, he needed immediate Vit.C replacement to turn the Scurvy around.   Vit.C in this country typically comes from corn starch, added to foods, enriched in products, in over the counter Vit.C supplements, and even in pharmaceutical grade Vit.C supplements- tablet form, liquid form, any form.   There are a few products available that come from tapioca starch; but Little Man had a severe dumping diarrhea and metabolic acidosis reaction from that last year.  We did not have a supplement that was known to be safe.   He also was declining again from the persistent malabsorption following multiple trials.   It was decided that another PICC would be placed and TPN would be initiated.  The PICC was placed and IV Vit.C started in August, TPN was initiated in September.  It’s now January, it’s been 5mo. with a PICC line and overnight TPN feeds, 12hrs to provide ~800 calories; his homemade formula that he continues to drink during the day provides the another ~400-600 calories. 

TPN has given Little Man a break, gut rest while getting a break.  Complete nutrition that doesn’t cause an inflammatory response in his intestines.  No food trials for a few months, while getting nutrients his body has been missing for too long.  The homemade formula is missing or deficient in ~12 vitamins/minerals.   B vitamins are the big ones, being a plant based protein source, Vit.C (as mentioned) is another one. Some other ones we don’t often think about but are important for their own functions in our cycles of nutrition.    I know his nutrition needs, but I cannot provide for them.  This is an extremely frustrating component of this allergy for me, the knowledge of incomplete nutrition but not being able to provide for it, all in the same breath.

TPN has given Little Man significant weight gain, catch up growth.  He has gained ~1/2#/week while on the TPN and is now over 30#!  He went from 2T clothes being big on him to now fitting in some 4T clothes!  It has been dramatic and so great to see.    His hair also has grown in really well.    As mentioned above, he was missing some nutrients but in addition to that, he was also malabsorbing many.   His homemade formula provided the same amount of calories- or more! And yet he could barely maintain his weight much less gain weight.   He had times when he would gain (probiotics is one, taking breaks from trials is another) but then we would do a trial and he malabsorb for a few weeks again before evening out and then have to spend time catching up on the weight loss.  A vicious cycle.  TPN threw a spoke in that cycle and allowed him to gain weight and catch up.  

TPN has given Little Man the IV hydration his body needs after a reaction now that we have started food trials once again.   IV nutrition is the treatment following an FPIES reaction, the hydration he gets overnight via IV is exactly what his body needs to “flush out” the toxins the body turns his food into; we can see that clearly for him…and we are so grateful to not have him suffer for days on end.

So, what is the worry?  He’s thriving on TPN, can’t he just stay on TPN?  A PICC line (the IV access line he needs for the TPN) is high risk for infection that can quickly lead to sepsis.   Little man has had this once, I don’t want to see that ever again.     It was reassuring to see his body fight so hard, and know what to do, under such dire circumstances.   But I never want to see him have to endure that again.   But each day with the PICC line is a risk that it could.  TPN is also very hard on organs, liver and kidney’s that have to filter these substances directly- with no filter from the intestines to assist in this process it goes straight into his blood – directly feeding his cells.  These risks are high.  TPN is not designed to be forever, for now the benefit has outweighed the risk for Little man as it is what keeps him thriving, giving his gut rest while we wait for his immune system to mature – to not attack food as toxins.

So, how much longer will he have TPN?  It was supposed to be a more temporary situation but the gut rest alone hasn’t been enough, the nutrition it provides hasn’t been enough.  It has all helped significantly but it has not erased his body having FPIES.  It does not erase that we still have to trial all foods to find him a menu, it has not erased that he has recently had reactions to strawberry, egg, potato, and he refuses to eat coconut, pork, beef, cauliflower; and now even pineapple is questionable.  TPN gives us the safety he needs to push through food trials….but the clock is ticking….

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. 

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.....

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?

Zucchini trial....clear as mud

Well, there is a reason why I named my blog this: Mudpies.  Well, a few reasons actually.  I'm surrounded by boys and now I'm surrounded by FPIES.    FPIES, FPIES, FPIES.   Everything seems to revolve around FPIES.  I wish it didn't but with Little Man's illness being so complex and chronic, it just is.    We are really hoping and quite literally praying we can figure out a tiny menu, with the help of our team here guiding us through his reactions and symptoms of intolerances.    

We've chosen to start that with zucchini.   Well, I've chosen.  I chose zucchini because we have never tried it before, he is not sensitized to it, and it's been over 18mo. since he's even had anything in the squash family.  It is a good source of Vit.C which he needs more of in his diet.  It is easy to digest and easy to bake in to foods (since his favorite food right now is his "cookie").   It is low in sugar and lower in fiber (skinned and seeded), it shouldn't cause any symptoms much less an FPIES reaction.   But if it should, it's only zucchini and not available all year (although I do have a freezer stash).   It WAS in season when we were going to orginally start these food trials so it was perfect (food trials were delayed a month due to a PICC line infection and treatment). 

So, here we are at zucchini.   Today is Day 6 and today went well; but we're holding our breath because we've been cycling through some concerning symptoms.  My hope and prayer right now is that these symptoms are merely his body demonstrating that it still struggles to find Oral Tolerance and to caution us to continue to be careful in the foods we chose next and how we prepare those foods, and how we help his body accept those foods. 


So, how has the trial been going?  What symptoms are concerning?

11-16 Zucchini Trial at 11:15am= 1 small slice and ½ a “muffin” with zucchini in it.  Fussy before nap, itchy ears, red cheeks, smelly gas, refusing bottle before nap. Woke up from nap pale but pinked up, and played well all afternoon/evening; very tired looking, drinking sips of his peach‘juice’. Slept ok through the night, some tossing and turning. BM diaper is "normal”- has been having them every other day, thicker.

11-17: Zucchini at 9:30am- boiled/purred- 1tsp. 12:30pm had bites of a cookie made with pureed zucchini. Got D10 for low blood sugar at 10:30am.  Refusing bottle a lot today, looks more pale this morning but happy throughout early morning, had some “blah” and fussy times. Found low blood sugar results from early AM lab draw,after getting unhooked from TPN; took 4oz.at 10:30, then had lab draw right after finished bottle. 1:30pm- irrational tantrum, drooling, choking, screaming (not crying-screaming),noted mottling (but was playing with cold water). Settled down and had a short nap, woke up crabby; better after bottle but still “blah”, evening tantrum noted to have mottling and feet turned purple when being held (cleared when put down), rash on right cheek, looks so tired, smelly gas. Decreased oral intakes, and decreased wet diapers (didn’t wet through the diaper last night as typically does). No BM diaper today.
Concerned that boiled zucchini caused the “episode” but could even have been from the D10 he got for low blood sugar. Slept well overnight.
11-18:  Zucchini at 5:30pm baked in cookies-ate 3, got approx. 1tsp zucchini.   Looks pale, purple around lips, lips look paler; but otherwise in good mood this morning. Started asking for his bottle again, and increasing intake mid-morning, took a good nap- woke up crabby but calmed down easily, played nicely all day. Willingly ate his “cookies” at 5:30pm- enjoyed them. 6:30pm noted rash on cheek flared up again, and smelly gas otherwise good mood. BM diaper had mucus surrounding thicker stool (playdough consistency).
Slept well overnight, but had to be near daddy. Woke up twice but didn’t eat.

11-19: Nibble of zucchini cookies at dinner 6pm. Fasting for AM labs to check blood sugar after TPN cycling, tantrums while waiting for 10am (fasting) lab draw! Ate well after that and played well- dismissed from hospital and happy to be home, ate “cookies” for dinner. Rash on cheek flared up after, otherwise no other symptoms- good mood and played well. BM at 9pm- “poop soup” (took pic)- watery/soaked in surrounded by thick pieces. Slept well through night. Eating better at home.

11-20: 9am bites of cookie with baked zucchini (same batch from last night). Asking for bottles, eating better today. Increasingly whiney all morning but playing. Rash on cheek flared again, itchy ears.

11-21:  9am BM diaper, blow out/liquid.   10:30am 1tsp of skinned/seeded/boiled/pureed zucchini- doesn't like it.   Fussy off and on today as yesterday, but slept well and increasingly better mood throughout the day, increased energy.   

I'm optimistic....

FPIES Funk

You know that feeling when things are happening faster than you can process? Or when everything around you is all about food (holidays, party’s,…) and your little one can have none? Or when your child’s intolerances continue to stump physicians and you simply just want to feed him and forget this world created by FPIES?

I’ve been in an FPIES funk the past few days. It isn’t the reason I’ve been unable to keep up with my blog, that started with just enjoying our baseline (shhh.don’t tell FPIES), which was kept busy with the work on The FPIES Foundation, but then led to anxiety about upcoming food trials and landed us square in getting an upper endoscopy last week and proceeding to the zucchini trial we are in now.

The upper endoscopy showed mild reddened area’s that are familiar to some past scopes. The plan was for a food trial to begin- zucchini; but the biopsy results back the day we started the food trial showed villi blunting, again. Do we proceed with the food trial? What is the villi blunting from? Is this his normal? Is it related to FPIES or something else? Could he have small bowel bacterial overgrowth, and is this why he did so well on the antibiotics when he had the line infection a few weeks ago? Is every reaction causing villi blunting? Was this from the probiotic? And why? Those ingredients were “safe” for so many months – why would it now cause this? Or was it from the Sorry board game ingestion? We certainly saw symptoms from that. Or, is it from something else in his diet? In his world? A cross contamination from the soap I use to wash his bottles? The exposed sheetrock downstairs (gypsum is made from corn)? The potential cross contamination in the Bob’s Red Mill products I use (they process corn meal on the same lines, anything is possible)? An intolerance to the arrowroot starch in his daily bottles, feeding his small bowel bacterial overgrowth? If this is happening this frequently- without glaring outward signs, then no wonder we’re struggling with passing a food, and no wonder he has persistent and severe anemia. If the reactions aren’t causing just inflammation but causing villi blunting- it would be more severe, and take longer to heal…..and no wonder we are always cycling. It certainly makes sense for why.

Still so much to process, still so much as we continue through a food trial- zucchini….