Which way are we going?

We have no new foods, and we have put ProViMin aside.  Something was causing full allergic colitis.   We started by checking labs and stools and correlating the ProViMin (increased and decreased) intakes to the results.  The first weeks it was evident something was going on but, just as we were left guessing with his symptoms and ups and downs, the labs/stools left us guessing a bit too....was it leftover from Alimentum RTF?  Alimentum caused full enteropathy: reflux, villi damage, decreased ability to digest sugars, large stools, dumping proteins, malabsorbing calories and blood in stools.  It did not cause the vomit issues we have seen with his reactions, so it was either something trace or broken down and it didn't seem to be flaring his enterocolitis.  But enteropathy is just as bad- just not as sudden.  It took us months to determine that yes, he was in fact reacting to the Alimentum.  I didn't want that to happen again so we were taking the ProViMin cautiously but so many good signs were there too, initially.   ProViMin has less broken down dairy (from hydrolyzed dairy proteins to a piece of a full dairy protein - sodium caseinate) and yet his enteropathy was flaring down, his body was beginning to absorb nutrients again and his hemoglobin that was declining had picked back up....but his iron stores tanked again and his protein stores were not picking back up.   Did we just need more time?    Did we just need to push the ProViMin more?  

So, we came back from camping and I pushed the ProViMin- anytime he was sitting down or started acting up, I would ask if he wanted a bottle; I would sit with him if I needed to get him to sit still and take a bottle.   We did dream feeds (where we feed him in his sleep) and he would wake up 1-3x/night to eat himself.  He was getting ProViMin around the clock.  Things should be improving if he was merely recovering from Alimentum.   

My theory was that while recovering from Alimentum enteropathy, his body wasn't fully accepting the ProViMin either.  My instincts were right.  

His body has been having allergic colitis from the ProViMin.   I'm not sure when that kicked in, or if it's been there the whole time because he did seem to be doing ok on it at first, but then he slowly began to get more fatigued and have poor color, he gets this subtle cyanosis around his lips when his intestinal inflammation is "triggered" - it comes and goes if he is getting the protein throughout the day (such as in his bottle); or it will flare up for a few hours if we are trialing a food.  It's beginning to be a good red flag for us....but I can't stop the food on just that alone, I (unfortunately) need symptoms to add up.  

When we got back from camping, we were turning in samples and going to the lab- one Friday's lab results revealed a "spike" in his normal- he had Eosinophils in his blood and his hemoglobin was dropping, Ferritin was quite low as well.  His stools from a few days (week) prior had a significant jump in the markers for inflammation.  Something isn't right.    The GI called and she was alarmed at the results and felt that it confirmed he was reacting to the ProViMin.  Her thoughts were that we needed to stop the ProViMin and get him scheduled for an admission to re-start TPN (total parental nutrition via an IV).   She has no other idea's on why his body can not accept food right now.  It is the answer that she knows will keep his body alive and his brain fed the nutrients it needs.  I know she is just looking out for his needs- both in the now (stop the ProViMin because it's hurting him) and in the future (provide nutrients via TPN so his brain doesn't suffer later); but I just can't help but ask myself if we are doing the right things...do we look further? Do we go somewhere else for 2nd/3rd opinions?  Do we put him through new tests and hospital stays and who-knows-what-else?  Or, do we just do what we know to do and stay home.    I don't yet know that answer, I've been praying and praying about it.  I just can't see through this muddy dark tunnel.   

For the immediate needs, hubby and I talked about it and we decided a few things.   One is that he wasn't as sick as he has been in the past and we still aren't sure it is the ProViMin.   He seems to be getting more and more corn sensitive, so maybe he got "corned" the day of the stool tests- his diapers that he has always been ok with are now causing rashes (diapers typically have corn starch in the liner, he has always seemed to tolerate Pampers baby dry) so we switched diapers and wipes (7th Generation diapers- tolerates MUCH better) and Waterwipes wipes...which are on the fence of tolerance but a significant improvement over the wipes I've been using with all the corn-y ingredients.   Also, a few days before the lab draw that showed the significant changes, he had had butter and a lick of yogurt (planned introductions to be "push" dairy to see if that is what he was reacting to in the ProViMin and both I had hoped from my research, would be corn free but wasn't positive), he also ate a good portion of pork (same night as the yogurt).   A few days prior to that, he had LICKED a paint brush while painting!!  No idea why he did that, took us completely off guard because he hasn't done that since he was a baby.   Any one, or all, of these things could throw these test results.   The GI was calling me on a Friday afternoon, before she was to leave town for a week with this news.   So, we decided it would be best to wait out the week- continue the ProViMin but do nothing else (no paints!) and see what the next weeks test results would be.  It was a rough week, giving him something we weren't sure if it was safe and that looked to be more and more unsafe but we also talked about doing an endoscopy before taking him off the ProViMin if the blood showed eosinophils again.    Eosinophils in the blood doesn't automatically mean EGID (eosinophilic gastrointestinal disorder) but it could and I don't want to miss this "snapshot" of his body telling us something.   We waited until the next Monday (when the GI doctor was back) to do the labs.  That morning, middle of the night, I gave him a bottle with an increased dose of ProViMin- timed so that it would be about 6hrs. before the lab draw....to see if the snapshot could tell us something, anything.    

The increased ProViMIn did not spike his eosinophils again, those returned to normal levels in his blood.   So, the spike of labs in that direction seemed more likely from the butter, yogurt, pork or paints- not from the ProViMin, so that was a relief.    But, this blood showed a drop in Neutrophils (white blood cells that during an acute reaction elevate but during a chronic reaction for him, they decline- like his body is fighting something and depleted (chronically).  He's even been on neutropenic precautions at the hospital before when he's chronically reacting (because these levels look like a little boy susceptible to every illness- a body fighting hard to keep those illnesses out that it depletes it's stores, chronically).  His hemoglobin did pick back up a bit but his iron stores continue to drop and his protein stores are not picking back up.   The blood in his stool samples continues to worsen but the inflammatory markers are improved.   The GI and I agree that this is allergic colitis; that although it is easier to manage than enteropathy or enterocolitis (in comparison to the two), it is not something we can continue to subject him too- it will only continue to get worse; and any "hit" to his system will flare it so significantly, he will end up needing a blood transfusion.  We need to find a new path, a different path that he can thrive on....but where does that path lie?    

TPN is the answer from the GI.   New food challenges and time is ours.  Which one will be the solution, we are not sure.   

TPN would meet his nutrient needs, but TPN is not free of his allergens (corn and soy).  We could potentially leave the soy out (that is in the lipids-the fats), since he takes a diet orally- we can give him fats orally (which he gets in hemp milk and safflower oil); but we can not leave the glucose/dextrose out- it is the "rider" for delivery of the nutrients.  What does a corn allergic person do?  I'm not sure.  Maybe we will need to explore that, but first- we owe him to try a few other things....

But his allergy is a GI allergy and shouldn't be affected by the TPN ingredients, right?   Well, the whole time he was on TPN, he had villi damage....so his gut was getting "hit".   Also, he regressed, significantly.   I saw it happening while he was on TPN but I noticed it even more dramatically once he came off.  He has underlying sensory issues that come out (significantly) when he is reacting a food.   Well, on TPN they stayed around all day long and his body couldn't cope with it.  He began to be very specific in his needs, we had to keep the house dark, he didn't want anyone touching him, his language had halted and was starting to go backwards....everything was in a heightened state interfering with his quality of life, and ours.  It's not the ideal situation.  However, he did gain weight, his hair grew, he expanded his developments (even though his sensory issues kept him from practicing them, they were there).  I know that if his body can not absorb nutrients any other way, we will have to do it - we just need to know that is the right option.

Rotation Diet, Protein Intolerance and FPIES?

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

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

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

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

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

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

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

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

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

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

Little man and his Mudpies

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

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

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

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

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

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

More Pieces to this puzzle?

My last post I talked about how the pieces to the puzzle were dumped out all over the floor, and that there may be pieces all mixed in from another puzzle.   Well, maybe (just maybe- not confirmed yet) that Soy is putting more of those pieces in their places.   Soy trial from 11/3 - 11/5, he consumed 6oz. the fist day, 6oz. the second day and double that the 3rd day.  We saw a few "weird" symptoms but they weren't hanging around and they weren't building; so we dismissed from the hospital on Friday night and stayed optimistic to call soy a pass in a few days.   Saturday afternoon brought on a whole new story.   After getting up from his nap, he was running a fever and was so miserable.  Sunday was the same story.  By Monday we wanted him seen and evaluated for a source of the fever.   Tuesday we were hoping he was turning a corner but by evening it was clear he wasn't winning whatever his body was fighting.  I had stopped soy on Monday morning and returned to his safe hemp milk formula that he otherwise thrives on.   Wednesday, after a positive-for-blood stool sample and discussion with GI doctor- we decided best to have him admitted for continued monitoring back to baseline and then look at either re-trial of soy or trial of a vitamin the GI doctor has found that we think may be safe.  By Wednesday evening, he looked like he was turning a corner and Thursday was a good afternoon.   But it was a false sense of security because overnight Thursday he began to run fevers again and developed a very "seal" like cough and runny nose and was just downright achy miserable.  His formula intakes were very poor through all of this, only taking in half or less of his needed caloric intakes.   We waited it out on Friday- trying to decide next steps, waiting to hear from the GI doctor on what her thoughts were for next steps (soy re-trial or MVI?).  By late afternoon, it was decided that we should proceed with a multivitamin trial rather than risk a fail with soy.   The main goal and priority now is to get the micronutrients he needs that his hemp milk formula doesn't provide....although labs were all done on his body's levels of these micronutrients and he is holding with all normal levels, so that has been encouraging.   Although the thought process is to treat it now before he loses these body stores and dips low (like with his anemia); and to have all the nutrition he needs in his formula and a vitamin as we press forward- taking breaks when needed, through food trials. 

The vitamin presented as possibly safe has some concerning ingredients that I will need to check on before trialing it.  I am in desperate need of assuring he is getting the nutrients he needs but also very worried about making him sick -- from a vitamin, synthetic nutrients!  I would SO much rather be doing a food trial....my thoughts go to Egg.   Egg has so many beneficial nutrients - maybe if he could pass egg, he can fill in the blanks to assure optimal nutrition from FOOD.   I am all for whole food vs. supplements but I do realize when the need outweighs itself.   So, I have some (more) researching to do this week; along with more research/reading on Protein Intolerance.  

FPIES is protein intolerance, it is viewed as the severe end of the spectrum of protein intolerance.  Much like anaphylaxis is the severe end of the spectrum of IgE allergy's.  Not everyone with IgE allergy's reacts with anaphylaxis, in fact it is rare.   Not everyone with Protein intolerance reacts with violent vomiting to bile/lethargy/diarrhea and shock- FPIES reaction.  It is also rare.

Soy brought on what looks more of intolerance symptoms (disturbed sleep, runny stools, bloody stools, mucous in stools, runny nose and a barky cough).  Not symptoms you would want to see at a baseline!  But not FPIES trigger either?  Not clear and not tolerating either way.    We will need to re-trial soy again soon to know for sure. 

Protein Intolerance is where I began this journey.   But his symptoms didn't fit nicely in that box.  Reflux was suggested, but his symptoms didn't fit there, EE was ruled out, Celiac was ruled out by biopsy.   Elimination and elemental diets brought us closer but still not in a box.   FPIES discovery, diagnosis and treatment brought us a baseline.   And yet we still were struggling to maintain that baseline.  More to come on protein intolerance vs/and FPIES as I learn more of this puzzle.

We did come home from the hospital (again) on Friday night.   We felt since Little man was FAR from a baseline (bloody stools everyday last week, with this congestion/cough/fevers) we couldn't introduce something and see a clear picture of how it was affecting his body.   We are so glad we came home!  We have enjoyed the past few days just being a normal family.