God Bless the Farmer

This season is a season to be thankful -- Thanksgiving, Advent, Christmas, and a New Year on the horizon.  I would love to say that Little Man had outgrown his FPIES or at least advanced his diet beyond his dozen foods but that is not God's will at this time.  However, God gives us the graces to get through our trials and He never breaks His promises to provide.  "Give us this Day or Daily Bread" is not just a line in a prayer, it is a promise from Above.  A promise that He will provide.  God has provided our family in so many countless ways, some I have recorded either here or shared in other places, some I hold simply in my heart but not a day goes by that His graces are not showered upon us through any struggles that are sent our way.   

One such powerful and life changing grace has been the pork Sam has had 'safe' for 2 years now.   A farmer referred to me from a friend as someone who cared for her animals and raised them naturally, not feeding them corn or soy.  A farmer I picked up the phone and explained Little Man's sensitive allergy to and who took the time to explain every step of the process from raising the hogs to interviewing the butcher in how the meat is processed.  A farmer who I have visited right at her farm and who Little Man has met.  The Farmer who makes sure before all her meat is sold, that there will be enough for Little Man.  A farmer who cares.  This pork, pureed into a liquid enough to go through his feeding tube, has been instrumental to his thriving, his not needing a hospital stay since his feeding tube was placed, his getting to be a boy discovering the world at the pace of any other 5 year old, not disrupted by frequent doctor visits, hospital stays, sick days and therapies.   He still has reactions to foods, symptoms from other foods, still needs to avoid more foods than he can safely eat; but we are so thankful for how far he has come and for each and every one of the few foods he does have to provide the nutrition he needs to grow and thrive.  One of those foods being the pork from this farmer. 

God Bless the Farmer. 

To see more about Little Man's story and Traeger Hills Ranch, watch this video I created last year as a tribute to this farmer: 

Sharing our story....

It was a decision to make our lives visible, our FPIES experiences known.  It was my first observation in this FPIES community, that there were more blogs and support groups than there was medical pages.  Sure, there were those handful of FPIES papers- mainly from Mt.Sinai where much of the FPIES research has come from over the years since Dr.Powell first coined this syndrome that had been noted by other doctors as a severe inflammation of the entire intestinal tract, causing vomiting, diarrhea, dehydration, often lethargy and sometimes bloody stools and shock symptoms.  There were a few moms, many of them also active in the support group on babycenter, that had started blogs - to share these everyday experiences, how they learned and their tools for thriving through this rare allergy.  In June, shortly after our formal diagnosis, I began this blog outlining our journey.  The good, the bad, the ugly. I quickly learned that as I was learning, this blog was becoming more about what I was learning- my inner working thoughts of how I was tying what I was reading in journal articles to my own son.   So, I started a 2nd blog- My FPIES File Cabinet, a resource page of sorts.  A place to keep those "research" posts filed, while continuing to chronicle the experiences we were having with our little man and his FPIES.  Since then, I have also joined with other moms and founded The FPIES Foundation.   I am passionate about sharing our story, sharing my personal insights seen through my clinical nutrition goggles; but also about sharing resources, tools, experiences, and insights - not only from experienced parents but experienced medical professionals.

From June of 2010 to today, August 29, 2012; I have logged 385 posts (and yet I'm still months behind) and have had over 35,000 page views (wish I would've figured out how to put that little page ticker on my footer at the beginning); it's been humbling to see how many have followed and shared in our story.  Sharing our story has become an important part of this ride, as others have done before and beside me- it helps.

"When you stand and share your story in an empowering way, your story will heal you and your story will heal somebody else"  Iyanla Vanzant.

With the busy-ness of 4 boys and summer, the complexities of FPIES, and my work with The FPIES Foundation, I have been a little more absent from updates, but do promise they are coming.  I've been keeping a journal as we go through this muddy mess we've been in with little man's FPIES as he approaches and passes 3yrs.old and still stuck, still adding new triggers and not outgrowing old ones...the updates are coming!   Overall, he is doing well, growing and gaining weight and meeting developmental milestones, and continuing to teach us about patience and appreciation for God's simple gifts in life.

In the meantime, I would be honored if you would check out the new website for The FPIES Foundation.   It is a 'labor of love', a resource packed with tools for new families and struggling families, a place to share stories and find support - a network of caring.  A place to come home.   We are not alone.

What is a Rare Disease?

From Rare Disease Day website:

"A disease or disorder is defined as rare in the USA when it affects fewer than 200,000 Americans at any given time.
• 80% of rare diseases have identified genetic origins whilst others are the result of infections (bacterial or viral), allergies and environmental causes, or are degenerative and proliferative.
• 50% of rare diseases touch children.


Characteristics of rare diseases
The 6000 to 8000 rare diseases are characterised by a broad diversity of disorders and symptoms that vary not only from disease to disease but also from patient to patient suffering from the same disease.
Relatively common symptoms can hide underlying rare diseases leading to misdiagnosis and delaying treatment. Quintessentially disabling, the patients quality of life is affected by the lack or loss of autonomy due to the chronic, progressive, degenerative, and frequently life-threatening aspects of the disease.
The fact that there are often no existing effective cures adds to the high level of pain and suffering endured by patients and their families.


Common problems faced
The lack of scientific knowledge and quality information on the disease often results in a delay in diagnosis. Also the need for appropriate quality health care engenders inequlities and difficulties in access to treatment and care. This often results in heavy social and financial burdens on patients.
As mentioned, due to the broad diversity of disorders and relatively common symptoms which can hide underlying rare diseases, initial misdiagnosis is common. In addition symptoms differ not only from disease to disease, but also from patient to patient suffering from the same disease.


How can things change?
Although rare disease patients and their families face many challenges, enormous progress is being made every day.
The ongoing implementation of a better comprehensive approach to rare diseases has led to the development of appropriate public health policies. Important gains continue to be made with the increase of international cooperation in the field of clinical and scientific research as well as the sharing of scientific knowledge about all rare diseases, not only the most “recurrent” ones. Both of these advances have led to the development of new diagnostic and therapeutic procedures.
However, the road ahead is long with much progress to be made.

Get involved!

The FPIES Foundation is participating in Rare Disease Day all week!

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?  

"Mommy, I need you"

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

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

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

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

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

Pressing forward & Pineapple Pass!!

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

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

What if?

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

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

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

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

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

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

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

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

We need a new plan

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

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

Am I doing enough?

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

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

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

FPIES Island

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

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

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

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

Surviving not thriving....

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


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

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

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

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

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

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


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

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

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

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

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

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

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

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

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

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

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

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

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

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

How is Zucchini going?

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


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

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

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….

Zucchini

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

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

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

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

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

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

Care conference and a plan

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

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

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

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

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

Admitted, what is the plan?

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

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

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

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

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

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


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


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

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

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

4. Pain relief during symptoms? Stopping a reaction?

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

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

Reaction? Vit.C deficiency? Anemia?

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

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

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

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