FPIES makes simple things complicated...

I imagine this is how it is with any chronic illness but that doesn't make it any easier to cope with sometimes.  He has a chronic illness, an illness that has become complex- why would I expect things to be easy?  Or quick? Or simple? Or uncomplicated?  Maybe it is because when you're having good days, you get a false sense of security.   You celebrate those moments and you live in them for a bit- not even thinking about all the days of hardship behind you or the ones that lay ahead.   But that is a double edged sword too.  Living in the moment, enjoying it for what it is helps cope and appreciate the small things....as long as things continue to go well.   But when things start to compound, or spiral....having been given "normal" or hope, only to have it taken away just seems like a cruel thing.   You have to learn, and accept that nothing is simple in FPIES.

Today, I had a list of things to accomplish...or- try to accomplish.  I had high hopes to actually get a few things done, and even an order to get them done in.  But between the weather and Little Man, there were other plans in store for the day and now I feel myself wondering what did I accomplish today?   I made formula, but only one batch because even though little man's daddy was supposed to be home- he had to go run an errand, which would've been fine if Little Man was anywhere near in a good mood...but he was not happy today and we spent too much of the day trying to figure out why.   Was it the millet porridge last night?  I thought millet is safe but I always find myself questioning almost everything in his tiny menu when he starts to have "issues".  Is it the omeprezole?  Or is it something as "simple" as his teeth. Teething is not "just" teething- we find ourselves dissecting all the symptoms that would otherwise be normal for a baby to display for teething.  But fussiness, screaming, decreased appetite, disturbed sleep, runny diapers, mucous in diapers, etc all can be lead up to reactions symptoms too!  Nothing is simple with FPIES.....

So, I made 1 batch of the formula....will have to get to another batch tomorrow.  Then it was time to run errands.   Errands included a lab check for Little Man (more on that later).  Errands also included researching at our local organic food store for pure vitamins (ie- no corn,dairy,soy,yucca,rice) for Little Man.  I did find a company that is showing some promise but I also want to investigate further if the pharmacy can compound a multivitamin- just of the micronutrients he is not getting in his current hemp milk formula.   With a calorically adequate formula and a source for vitamins; we could take food trials at his pace - SLOWLY, and not stress so much about his nutrition during it all.  But the catch is, regular Multivitamins do not come corn free!  There is either corn in the inactive ingredients, or there is corn in the vitamins themselves!  This has led me to do hours of research, contacting company's for ingredient sources, scouring corn free forums for leads....and only getting disappointed over and over again.   Nothing is simple in FPIES....  

Now, I would much prefer my son get his nutrition from actual food -- vitamins, minerals, protein, fats, carbohydrates, and calories-- right from the source where it was intended to be.  But when you have an over-reactive immune system acting foods and an immature digestive system such as Little Man's where very little food consumed (and none in it's raw form) is able to be consumed without pain, and the potential for an FPIES reaction, you can't "just" give the foods you know should be included in the diet.  And to add to it, having a reaction means inflammation in the gut with weeks of healing, poor weight gain, and stomach pains, with bloody stools;  and now we are also contending with villous atrophy (that is in my head continuously as to where it came from, is it healing? what are we doing wrong? what should we do differently? Is millet safe? Was it the soy? Is it the sunflower oil in his hemp protein powder?  Could it be the peaches?  How could it be the peaches? Are we doing everything we can to help him?....).   Nothing is simple in FPIES....

Finding a treatment plan with an illness that is so little understood, and more often than not, not taken as seriously as it is....especially when you are doing so much at home to help your child to thrive....so they look so good on the outside but their insides are a mess.  Finding doctors to help and to trust your instincts as a mom, to know that you have done countless (ok millions) of hours of your own research on your child's condition so that you are aware of best treatment options.   Driving hours to specialists who are familiar with this little known diagnosis, teaching new doctors more about the illness you have become so familiar with, scheduling appointments, rescheduling appointments when they get sick, leaving messages for doctors- hoping they get the message...and understand it in the context in which you intend it to be received are all part of the everyday agenda of an FPIES parent.  A chronic illness protocol involves a care plan.  A simple run down of the diagnosis, doctors involved, and the treatment plan.   But is a simple run down enough with a diagnosis so many have never even heard of much less can wrap their head around the complexity of?  Nothing is simple in FPIES....

So, today my day involved making formula, running errands, getting formula ingredients specially ordered, looking in to where to get more peaches, researching vitamins, plus all the other "normal" things that keep a mom of four busy (laundry, dishes, groceries, homework, potty training, decorating the Christmas tree, making meals);  and of course worrying about next steps with Little Man...especially after his labs came back in today....

Weight gain!

Little man has been maintaining a baseline for 3 weeks now, with only slight deviations from it.  He continues to have sleep disruption for the past month, pain is accompanied when his stomach is empty and he can't seem to get enough to eat but since his stomach only holds so much at a time- he is awake until he gets enough....usually 1-2hrs in the middle of the night.   We have tried getting more in during the day, we have also tried "dream" feeds- which work some nights but other nights seems to make things worse.  Maybe it helps so that his stomach isn't as empty but it disrupts his normal sleep pattern so he ends up being awake anyway.   He isn't screaming or wide awake in pain, he is just not "settled" for a few hours almost every night.  Sleep, precious sleep.....someday, maybe....

Aside from sleep, he has been doing primarily well and then all of a sudden he started to slide on Friday and by Saturday was an-all-too-familiar-mess.  Wednesday & Thursday's changes were an addition of an extra tablespoon of safflower oil in his formula.  Friday night two things entered his system: 1)bath water that his brothers had put soap in and 2) his new Rx: compounded omeprezole.   Sunday we saw a return back to baseline (thank goodness!) but I was nervous about things getting worse if it was the omeprezole, and little man's daddy was at work Sunday so I skipped that dose; but gave him his dose on Sunday night.  He slept well and woke up singing!  Today was a very good day- lots of giggles, playing well, SINGING, and eating and napping well.  The only worrisome symptom?  Hiccups, twice this morning.   Yes, hiccups- hiccups he only has had when things are not going well in his digestion....but hiccups alone are no reason to stop a medication that could be helping his inflammation finally heal.  

We decided to do the omeprezole trial on the recommendations of the GI doctor, who feels that he is stuck in this cycle of not tolerating food/pain with eating because of unhealed inflammation.  Inflammation that is being washed over by stomach acid, causing irritation.   This makes sense in a few ways: 1) of course a sore can't heal with acid washing over it, and 2) he has had extra acid production in order for his iron supplement to be absorbed.  This is about the time frame we started seeing these back arching, uncomfortable-when-his-stomach-is-empty kind of symptoms, so it makes sense and hopefully is the right fit.  Hopefully his body doesn't have an adverse reaction to the medication before it can do at least some of it's job.   It is specifically compounded to be free of his allergens, so the intolerance to it would be his body not liking the Proton Pump Inhibitor (ie- stopping stomach acid production).  You need stomach acid for digestion, so I am leery of him being on this too long- fixing one problem will just create another.    We will let him guide us, as he has done....we will listen to his body and the symptoms and signals he sends....

The GREAT news of today is his weight check.  We are monitoring his weights closely- it has helped to see how long it takes for his body to re-coop from a reaction, and clues to what is going on with his digestion and absorption of calories taken in.    As I mentioned above, we added a tablespoon of oil (under guidance of our Dietitian) to his formula recipe (he already gets 3Tbsps in 80oz: a very high fat diet right now).  The side effect we should have seen was loose stools, but instead we are getting the opposite.  Side effect noted.  Moving forward.  He needs the extra calories, even if they are just fat calories, to regain some lost weight and begin to make some sort of catch up.  But even more, he needs to show us that his calories in = weight gained; which in turn show us that his atrophy and inflammation are healing and his body is re-cooping well enough to avoid alternative forms of nutrition (TPN=IV nutrition).    So, the scale today gave us good news....10kg!  He is now at his high of 22#!!   Not only that, he gained almost 3grams in 5days!!    We pray next steps allow him to keep this weight gain, while adding to it.   

Peaches continue to go well, and tonight he made big steps (celebrating I guess?!) and accepted some millet porridge fed to him by me, from a spoon.   This little boy has spoon aversion, too many times I fed him food from a spoon that later made him sick.  He prefers to feed himself, but he also has texture aversions (and will not feed himself from the spoon either), and he can not currently tolerate raw forms of food.  So, his peaches are in his mesh feeder or in "dots" (cooked, pureed, piped, frozen dots of peaches) that he can pick up with his fingers or his fork.   So, tonight I made him millet/peach "dots" and put them in the freezer for him to try over the next few days; and since I had fresh millet porridge- I put some aside in a bowl to see if he was willing to trust me and let me feed him.   He was leery at first but he warmed up and took a a few tablespoons worth from me!  I was very proud of him.  He also had a little mug that he asked me to put water in (as I was pouring water into my glass) and I did and he drank it right down and asked for more!  He did this 4 times, drank it down,and didn't spill a drop!  This is A LOT of BIG steps in ONE meal and I am very excited about it.  

Lots of good things to mark the day.  One month ago today, October 29th, he was bottomed out and awaiting a blood transfusion.   Now, November 29th: good weight gain, ate from a spoon, ate millet baby porridge, drank from a cup.   A day to mark on the calendar.  A good day. 

A Year of Thanks.

It was a year ago that our journey started to compound, that it took it's leap from "just" Milk protein intolerance to adding up to so much more....what that would be, we had no idea at this point last year. We had gone from not tolerating Good Start, to lots of throwing up and poor acceptance of baby foods, a hungry baby that wouldn't eat, a baby that was now fighting colic on a regular basis.  

Thanksgiving last year is when I started the supplemental soy bottles, we were going to be traveling for the Holiday weekend and since we knew regular cow's milk formula's were not going to work for our little man, I was ready to try some soy formula with him.  I'm not even sure if I anticipated there to be any problems as two of my other boys who have milk protein intolerance's have tolerated soy.   I remember the trip up north was good (6.5hr drive) and the weekend visit was great.  I remember he didn't eat very well, was refusing the spoon a lot by this point and the only thing he had been taking at home was banana, oatmeal and sweet potatoes and I had brought along the Gerber oatmeal/banana jar food for convenience, but he wasn't especially interested in that either.  He did eat graham crackers and cheerios, and cereal stars well.  I wasn't too alarmed in his poor appetite for his food since it is typical for my boys to get out of sorts and not eat well while we are traveling.  I try not to stress about it, they'll eat when they are hungry and when we are up north visiting- it is visiting the grandparents so I want their visits to be fun.   He took the soy bottles a few times over the weekend and we were trying to use them for on the way home because I did not have anything pumped and he was very,very fussy the whole drive home (had been so happy on the drive up).  I remember it being a very long day and lots of crying, and knowing he was just uncomfortable but not yet associating it with the soy formula.  And I do remember thinking it was from my diet - too many treats over the weekend, as I have IBS and have trouble with fruits,milk, and wheat; that I felt was carrying over into my milk supply and disturbing him.   Now, looking back- it was more likely all the soy.  Especially knowing what we know now about the villous atrophy that just came into our picture after our soy trial.   Villous atrophy causes the body to absorb zero nutrients (at the points where it is affecting the intestines).  I look at Little Man's growth chart and the time from when we started soy to 6weeks after that, he went from in the 40th% down the to in the 20th%.   He also had severely low Vit.D and low iron levels at this time.  This piece of the puzzle is coming more into focus now....villous atrophy from soy protein.   Does Little man have Milk/Soy protein enteropathy and Rice/Corn/Grains FPIES?  Is this why his illness is so complex? 

I take a look back at a year ago today, because we really have come so very far.   A year ago today we were starting our downhill slide with Little man, we were losing our grasp and it was after the holiday season that I knew I had to get more help for him.  It couldn't be "just" milk/soy protein intolerance, it wasn't "just" reflux, he wasn't gaining weight and was starting to lose, his pain was growing daily, his sleep patterns were erratic and his night sleeping was pain filled, he was hungry but wouldn't eat, and he cried and cried and cried. 

Since then, we have done elimination diets and trialed reflux meds, we have ruled out Eosinophilic Esophagitis, and Celiac Disease.   We have had gut rest and changes to amino acid formula.   We have food introductions, and fails that led us closer to the FPIES diagnosis.  We have received help and support from some great Dietitians.  We have traveled half way across the country for a consult with an expert Allergist to confirm the FPIES diagnosis.   We have found a very sensitive trigger (corn) for Little man and moved away from any and all sources of it -- to have the daily pain attack and sleep disruption and random vomiting all STOP.   We have formulated a formula, made with ingredients his body tolerates.  We have been able to add millet and peaches, very slowly, to his diet. He may be failure to grow and gain weight but we have kept him thriving, and growing at his pace.   He is a smart, active, interactive, inquistive, happy, kind, quiet and loving little boy. We have found a very supportive, and knowledgeable GI doctor, in our home hospital, to help us manage this complex, chronic illness and manage it at home.   At home, where we can maintain a quality of life and normalcy of life for Little Man's brothers- despite food trials, hospital stays and diagnostic procedures.

And we have slowly been adding pieces to solving the puzzle that is Little Man. 

We have come a long way, and today on a day of Thanks- we are thankful.

Corn is in Everything!

I have a few corn website resources since we discovered corn being a trigger for Little Man.  I wanted to do a post about it, as I have been finding new resources for corn allergies...and also finding something as reassuring as finding FPIES support groups....that there are other corn allergic people out there, facing the same challenges and obstacles to health. 

The first one I found still has been my biggest "go-to" as she put a lot of work into her webpage to help corn allergic people.  She provides a list of corn derived products, product names that could mean corn....it is at the very least a great list to know which ingredients I need to yield to, and check on....I always check everything for myself.    You can not simply trust a doctor or a pharmacist, or a company customer service representative (no matter how well meaning)....or the ingredient listing.  You need to know the ingredient source of all ingredients.  Corn has many names and many things you wouldn't suspect corn to be a part of, has corn derivites; and many people simply do not recognize this to know.  It is up to you, as the corn allergic person to check ingredients.  Her website is: Corn Allergens

The next one I came across is: Corn free foods blog
This is an active blog and has provided some eye opening topics, discussions, articles.  I follow this blog daily. 

Another good blog to follow is: Live Corn free.  Visit this site to sign a petition to get corn added in the top 8 Allergens.

Another one I just found is appropriatly named: a Zillion Uses for Corn.  It is a quick-read-great eye opener for just how many things corn are in (wish I would have found it when I spent a few hours researching why Little man kept reacting to the sheetrock he was putting in his mouth, or the books he was eating!)...paper and gypsum board have corn in them!!

An article shared to me shed some light on why corn is in everything, it is: http://www.abmf.com/news/nabitor It is good to know why it is in everything because then you know when to suspect it might be in something. In short, it is a mold-inhibitor...so a preservative (for food and non-food).

There are many more, and the list grows.  I have started a corn resources section on the side of my blog for those following that also have to worry about corn for their little ones (and my place to keep it all organized!). 

Little Mans corn intolerance is quite sensitive.  I get worried when I wonder if he'll ever outgrow it...being so sensitive to it and us taking so long to discover it, his last corn ingestions were in August, but then he did have an accidental ingestion, of sheetrock (we really need to get our basement finished - we started the remodel of the walls before Little man was born but we haven't had the time or money to finish mud/tape/painting it!).  Since his anemia is better, he doesn't have the PICA he has had in the past so his sheet-rocking seeking obsession isn't as bad.   And since the last time he accidently ingested it, he hasn't tried again -- maybe he finally associated that ingestion with his reaction.

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....

Thankful for...

I am thankful for this little face.   Little man has been recovering from recent "fails" and returning back to a baseline.  His demeanor has been good, great.  His activity level has been good.  His pain has been minimal.  His appetite has been good.   Tomorrow we bring him in for a weight check to see if he is gaining (he should be - he's eating 1000 calories/day, so he is meeting his needs).  But weight loss has always followed his reactions.   Was soy a "reaction"?  It wasn't an FPIES trigger but we still are not sure what the symptoms we saw were- if they were attributed to soy or to food dye.   Whatever it was, it has done it's damage as Little Man's recent scope biopsy's have been coming back and are showing some villous atrophy, along with his inflammation that was visible on scope.  The colon biopsy isn't back yet and that may tell more.  More tests will need to be ran because we are not sure why there is this villous atrophy.   We do know what it means, villous atrophy means malabsorption.  Little man can not afford malabsorption of nutrients on his already restricted diet.   We meet with the GI doctor tomorrow to discuss next steps. 

More pieces to the puzzle...

FPIES builds character

I don't mean the humility, patience, endurance, perseverance, strength, forgiveness, empathy and advocacy it has taught me.  Although it has taught me all these things to a new level, and so much more.

FPIES is building a strong character in my Little Man.   At his baseline (which he is at now), he is a quiet, content, happy little boy.   A toddler who can sit quietly and color, build blocks, "cook" in his kitchen, put together a puzzle, play with cars and puppies.  A little boy happy with his surroundings, and exploring and learning from them one step at a time- taking it all in....appreciating it all.  As if he knows what he has missed out on from days of pain, days of only wanting to be held, days of tears (from both him and I).   Ask for a kiss and you'll get it, ask for a hug and his arms surround you, ask for a high five and he can't stop handing them out! 

We are holding our breath (and smelling the roses!) while he remains at a baseline.  How long can we keep him here?  Days like this that we feel more in control of our lives and his FPIES, that we feel we are winning...that we've been to he** and back and we can still see the light from Above.   We know the dark side isn't far away, and those days of pain can be back in a blink of an eye, or more like the swallow of a crumb....so we stay cautious as we always have; but it is fun to just be.

Baseline

Despite having visible inflammation (on scope yesterday), Little Man is at what we call his baseline.  He eats well, sleeps well, plays independently, learning, growing, typical toddler days.  

We anxiously await biopsy results on Monday to (hopefully) tell us more about his inflammation and intestines. 

There is a great temptation to stay here at this baseline, maybe even through the holidays.   Enjoy the baseline, enjoy Little Man, enjoy our family.  But Little Man needs a tiny menu, a tiny menu we haven't succeeded it building him yet- in finding what he can tolerate without pain or reactions- protein intolerance, FPIES, or enzyme deficiency.   He enjoys his millet puffs, and peaches continue to go well....what else can we add? What else would be safe?    The GI doctor wants a multivitamin, and now wants to consider a acid reducing medication.    I am not aware of a multivitamin or an acid medication that does not have corn.  I do not feel right doing things that would even potentially set him back when we can trial foods that have better chances of moving him forward....

For now, we enjoy the baseline- enjoy every minute, enjoy his kisses and hugs and cute gestures, learning new words and milestones. 

Lesser of Two Evils....

It seems with this FPIES, I am constantly having to make choices that are difficult to make.  Choices where there is no good answer, that it comes down to the lesser of two evils choices.  

It began somewhere around elimination diet and elemental diet...lesser of two evils?  Elemental formula because at the time, I thought for sure my production was declining due to my high stress, and elimination diet that wasn't eliminating enough (and since I already follow my own dietary restrictions).  We began to suspect a corn intolerance because the proteins in these formula's are amino acids (broken down protein chains).   

Then on to Elecare or Neocate....lesser of two evils?  Neocate because the vomiting wasnt' as acidic and it slowed down to every 4days instead of everyday.  Without daily vomiting, we began to reintroduce foods back into the diet.   This brought us to FPIES for the first times after reactions to rice and sweet potatoes, that are "supposed" to be so low on the allergen scale for food introductions.

Then on to travel half way across the country or stay here waiting for them to learn about FPIES enough to provide a diagnosis....lesser of two evils?   A trip, turned family vacation, halfway across the country to see a specialist for a diagnosis of FPIES.  

Then it was trial soy or corn first, to find an alternative formula since the one(s) he had been on were not helping him get closer to a baseline, or tolerate any foods....lesser of two evils?   We knew he had a soy intolerance and was already so sick, corn we suspected but were not sure....we didn't anticipate it making him as sick as he did, for so long.   We began to learn why he has has been so sick all his life....corn is in everything.

ER visits with a lethargic baby or wait it out till Monday morning to see his regular doctors....lesser of two evils?   Monday morning to see his doctors because we know the ER doctors (although always helpful) will not understand what they are seeing with little awareness of FPIES at our hospital.  We begin to learn how fast he falls when he starts to spiral. 

Reintroduce Neocate or have a baby on a low carbohydrate diet (unflavored Original Hemp milk has little carbs alone)....lesser of two evils?  We wanted to believe we could find a thresh hold of tolerance with the Neocate that could provide him complete nutrition of trace nutrients while meeting his macro nutrient needs.   We began to learn more about trace proteins and thresh holds of tolerance.

Transfusion or Oral iron...lesser of two evils?  Oral iron....which was going to be slow but he was tolerating and responding well to until we got a refill and it was compounded with red food dye.   We begin to learn more about his many intolerance's and end up with a transfusion anyway because of continued drop in hemoglobin to dangerous levels. 

Acid blocker medication or see if his inflammation heals on it's own....lesser of two evils?   This one, I don't know yet....

Little man had his 3rd upper endoscopy and sigmoidoscopy today to check for inflammation and ulcers, obtain biopsy's, find the source of his bloody stools, and (hopefully) put more pieces of the puzzle together.  Our (new) GI doctor is amazing and she has been very helpful, taking Little Man's health and his protein intolerance's and FPIES very seriously.   Treating FPIES is all new territory to her as well but she is very familiar with other GI disorders, and has a special interest in food intolerance's and their affects on the GI tract.  Her assessment of today's scope/sigmoidoscopy is that he has a fissure that could be the source of his lower GI bleeding, but also took biopsy's.  He also has inflammation in his duodenum (upper intestines, right past the stomach)- this may be from an allergic inflammatory response but she also feels it is being exacerbated from excess stomach acid.  This stomach acid could be from extra production due to his iron supplements- they are elemental iron and the body needs good amounts of stomach acid to absorb the iron.  So, his body is doing what it needs to do- giving him rich acid to help absorb and utilize his iron supplement but then this may be impeding this inflammation from healing very quickly.    He doesn't seem to be in too much pain from it the past week- occasional bouts of pain when we know his stomach is empty- but if we keep his formula flowing through at least every 3 hrs (day and night)- he doesn't experience the pain.   Also, his acetaminophen that is compounded with calcium carbonate (a natural acid blocker) helps with these bouts of pain.    The body needs stomach acid.  Without stomach acid, he will be missing an important aid in digestion of foods.  The breakdown of foods will be inhibited and could lead to increased allergenic potential.   In the past when he was taking these medications, he had almost immediate yeast infections- one so bad it burned the skin off his little bottom with just the couple second exposure to the bowel movement.  So an acid blocker/proton pump inhibitor could delay food introductions even longer, and give way to a host of it's own symptoms with pain.   What does he need more- increased nutrition or less acid.....lesser of two evils.....

Lessons....sharing....

The past two days consisted of two full appointments, with Pediatrician and GI doctors, appointments to move to next and new steps.  History was discussed, past reactions and steps taken to get to where we are at today and next steps discussed on how to move forward from today to continue to get Little man to a healthy baseline to thrive through these FPIES years.  The pediatrician's goals are simple: growing and developing on track.   GI's goals are more complex.   Learning about treating FPIES here, learning from Little Man, learning how to avoid his allergens while still helping him thrive with the nutrients and medications he needs.  Developing protocols for treating FPIES, for food challenges and trials.  But right now, today, Little man continues to have bloody stools and the GI doctor has moved this up to top priority to seeing if further testing can tell us why.   Little man will undergo his 3rd scope on Friday morning.  Our GI will peform that scope and run some new tests/biopsy's. 
The past two days have been busy, and my head is spinning from all the potential progress....and while Little Man maintains a beautiful baseline.  He is a happy, playing, cuddling, kissing, giggling little boy the past few days and we are enjoying every minute of it. 

Last night, a friend posted her Lessons FPIES has taught her.   FPIES mom's come from all over the country, all over the globe!  We connect on this level....every one of us has a picture in our head that goes with each of these lessons learned....I not only want to link to Carter's Maze but also share her text here.  Thank you Carter for teaching your mommy, who teaches me, who supports me, who has shared so many of the ups and downs over the last 6mo. with me.   FPIES has taught me these lessons as well.  

Carters Maze: Lessons

"Things FPIES Has Taught Me . . .



· Two year olds have more compassion than the average 30 year old.
· Walking around wearing a shirt with vomit stains really isn’t that embarrassing
· It is possible to go a whole day with poop on your shirt and not know it and not care
· Doctors are human too, which means they are fallible
· Doctors don’t keep up with the latest research in their field of expertise
· Crumbs are scary
· “A taste of this won’t hurt” is completely untrue
· The internet is amazing. You really can find anything!
· Mommy instinct should always be acknowledged
· Poop really is a four letter word
· The American culture is completely intertwined with food
· Support is essential to sanity
· Passing a food feels like a kid on Christmas morning
· Failing a food is heartbreaking
· Just reading food labels will not keep our kids safe
· Seeing your child’s face covered in food can bring tears of joy to your eyes
· A birthday cake doesn’t have to be made out of flour or even food
· Every sunrise is a chance to start over, full of possibility
· Strength is tangible
· Sleep is optional
· It’s amazing how creative you can be with foods
· No matter how many children you have, you are never prepared for this!"

And here are my additions:
-Corn and soy are in everything
-Projectile poop is something new, even to a 4th time mom...

-Acidic vomit stains carpets...
-FPIES is an ironic, ironic world
-Love and support of family and friends is truly amazing
-I used to think I knew what busy was...
-I used to think I knew what tired was...
-I never knew how strong a sick baby could make me
-Everything amazes me and nothing surprises me anymore.
-Instincts are amazing
-Faith sustains me
-Everything, every.little.thing, happens for a reason.

Quality of Life

This term first came to me while working as assistant food service director in a long term care facility.   The goals for chronic illness were always centered around maintaining a quality of life.   If symptoms interfered with the quality of life, a care plan needed to be developed to address this symptom and how it was to be treated and monitored, with goals set for continuous following.  

FPIES is a chronic illness.  Finding a quality of life with a chronic illness is a big part of managing the illness....for the patient, the care givers and the family.   A care plan for the medical team helping assist with this is a helpful tool.    What goals do they have as a medical team? What are our goals as caregivers/parents?  What are Little Man's needs?  What do we do to help maintain his baseline and prevent ill effects from his illness?   What is the plan when he suffers these ill effects?

I have had a goal to find a quality of life that we can maintain for Little man's illness but not only for little man- but just as much for our family, for his brothers.   We will be coping through FPIES and all of it's restrictions and sufferings to Little man for the next couple of years (maybe longer but Faith allows me from worrying about that far ahead), living with constant worry or anxiety or devoting all time and attention to his illness will not help him achieve a quality of life and will do nothing for his brothers' quality of life.   Our recent goals have been surrounding this.   Recognition that FPIES is a chronic illness, recognition that we can do everything in our knowledge and power to keep him healthy but his illness is still there, lingering around and he may suffer ill effects from trigger reactions and intolerance's to many foods, but it isn't because we aren't doing enough to help him or keep him from those reactions.  Realizing that his care will take more of our time than his brothers at times (many times mostly) but that doesn't mean we aren't doing everything everyday to help them continue to thrive as well, and that Little Man's illness doesn't run the household.  Realizing that we are a family and we're in this together, even if it means we all give up a few things to help Little man.  Realizing that we have so much support from so many family and friends,so many people just holding us up in prayer for continued strength to help Little man.  

We will always look for better ways to cope with this chronic illness that affects not only Little man but our whole family, we will always look to be better about accepting help that is offered because it is better for Little man and our other boys, we will always look for ways to accept the humility God sends us. We continue to strive for a better quality of life through our FPIES.

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.

"Atypical" FPIES

FPIES "plus" is another name a GI doctor we saw recently coined Little Man's case.   Atypical FPIES is what our (new, great and supportive) GI doctor has called it.  A little puzzle to solve.   I agree, it is a puzzle...we have long since called him our little puzzle as well.   One piece fits over here but then the next piece doesn't fit like it should next to it....and so on and so on.....

I have theories as to why Little Man's FPIES has become so complex....most of them stem around the fact that we simply do not know anything about it for so many months....no clue why he was losing weight, why the formula's weren't working and the solids were a mess, why my elimination diet wasn't helping enough, why he had colic at 3mo. old and why he developed reflux after we started solids.   He was a mystery to his doctors as well, no awareness of FPIES in the hospital where we have his care.   We hope we are changing that, but in the meantime...we need them to catch up - quickly.    We are very hopeful, and remain optimistic (who would guess I am actually a very optimistic person), that we have finally put together a team of medical professionals (Allergist, GI, Pediatrician, and Dietitian) that can recognize his FPIES, learn about it, and help us put the pieces together....one by one....

Right now, the puzzle is dumped out all over, and may even have pieces from someplace else mixed in....we need to learn if those pieces belong to this puzzle.  We are back in the hospital hoping to sort through that a little more.

Little man's biggest triggers are dairy and corn....we know what corn has done to him, we are not sure what dairy would do and have zero interest in finding out because of how poorly he did on broken down dairy proteins and dairy in my diet.   As I've mentioned before, corn is in everything -- so it is a tricky trigger to move away from.  Most especially when it is a high sensitivity and oils and syrups also trigger the allergic response.    We were hopeful that an elemental diet would get us to a baseline but after 5mo. on it and no baseline to be found; we knew we had to trial corn to see if this was the culprit....that piece to the puzzle was found and it is a big one.   If getting his diagnosis was the "corners" to the puzzle, then narrowing down the corn trigger, we finally had all the "edges" to our puzzle....but now we are still left with the "middle" - the rest of the puzzle. 

Right now the pieces that we are working on, and can't seem to find the way they fit - is the drop in hemoglobin causing severe anemia, the random bloody stools, the pain in his stomach, and his soy (is it or is it not a trigger for him?). 

Moving away from corn in all forms meant stopping the elemental formula he was on, that he had been on for 5mo exclusively that continued to damage his GI tract.   The plan was to put him on hemp milk to stop this inflammation and begin to heal his, now ravaged, gut.  We knew the hemp milk would need fortifiers and a tiny menu to round out all the micronutrients a toddler needs for good brain growth and physical development.   We were stuck in the puzzle for so many months trying to find his diagnosis, then we got stuck trying to find his baseline, and now we are stuck trying to find him adequate nutrition in this corn based society where corn is in everything (no offense to corn farmers intended here).

He has been so chronically ill from this affecting his gut for so long that he now continues to suffer the after effects of that.   We keep searching for that right puzzle piece.....will we find it now?   

Tag Team

Little Man was in the hospital for a week, with the efforts of his daddy and I - we were able to maintain some sort of order on the home front because we have such a system down by now.   All part of learning to cope with a child with a chronic illness -having a plan in place when he needs to be in the hospital to maintain order in the other children's lives.  His brothers are very well aware that he is in the hospital and that either me or daddy are there with him (while the other one is home with them) but they have needs too and this can't be forgotten.   Maintaining routines and order for them helps them cope better through it.   It also helps when family is able to come and help out, so his daddy and I can both be at the hospital or have an extra set of hands, and entertainment to 3 energetic boys at home.   (Thanks again Aunty K!)

We had a system, we coped through it; but we were glad to all be home under the same roof again.   Friday night was uneventful, little man even slept fairly well back in his own bed.  We continued to imagine how nice it will be to have soy as a "non-trigger".   I made a fresh batch of formula, 1/2 Hemp milk and 1/2 soy milk (with the fortifiers of safflower oil, hemp protein powder and arrowroot starch) for Saturday morning and the morning went well.  I also restarted his iron supplement he has been taking since mid-September.  The pediatrician at the hospital explained he would still need his daily iron, even though he had just gotten "loaded up" with red blood cells because otherwise his body would not make more red blood cells because it would think it could take a much deserved vacation!  So, I wanted to get that restarted right away.   He played all Saturday morning and went down for a nap at his normal time.   He awoke from his nap screaming and clearly upset.  I picked him up and immediately could tell he had a fever.  I took his temperature and sure enough, a fever.  Clearly he was uncomfortable so I went to get his ibuprofen (this is compounded for him to be corn free).  We had taken it with us to the hospital and it wasn't unpacked yet, however I had a new bottle of a prescription I had filled a few weeks ago (to have on hand instead of waiting until we ran out because compounding can take a few days).   So, I went to open the capsule (it comes in a powder, sealed in a capsule that we break open and mix with water, using a syringe to give it to him) and the powder came out orange.   Now, his iron that we got from Walgreens at the same time (second week of Oct.or so) also has been orange but I assumed that was because it was elemental iron and this suppliers was orange....but now, why would this ibuprofen be orange if it is just ibuprofen and calcium carbonate?  The other one wasn't orange.   He was screaming, so I gave it to him and then immediately thought- "why did you do that, you don't know what the orange is"!  I immediately called Walgreens and was relieved when the kind pharmacist that worked so well with us in the beginning with his compounding answered.   She looked up the information on this particular prescription and sure enough, they had added aluminum/red dye #40 to it!!  This is used as a "lake" and it helps keep the medication compounded.  They had wanted to put it in his original prescription compounds -- I remember because it was one of the many times they called to double check to see if they could add this ingredient or that.  I have been wanting to steer clear of food dyes.  Little Man's daddy was allergic to food dye as a young kid and his older brother has some issues with them as well, and I had observed in the past that he may also have these same issues.  I don't know if they would be FPIES related or not- I think just more of an intolerance/typical allergy.   But the pharmacist that did this newer refill did not know that, did not think to check because I had not spoken directly to him/her to discuss his food allergies and we do not have food dye's listed on his chart for allergies.  So, we have been giving him red food dye for 2-3 weeks in his iron.   Could this be the reason his hemoglobin feel from 7 to 5 without us noticing?  Could this be the reason he was having problems sleeping (hyperactivity is a symptom of a food dye intolerance) despite being so over tired?

It might be, but it could also be a build reaction to soy.    Saturday we stopped any medications with food dye but continued soy.   Saturday night was disturbed sleep, Sunday brought more fevers, more crying, more needing to be held all day, and a loose-soak-into-and-thru-blow-out-mucous diaper.    There is fiber in his soy milk, so maybe it is because of this increase in fiber in his diet.   Maybe he is teething, and sometimes stool can be a little more runny from that.  Maybe it was the red food dye reaction.  I was starting to get nervous about the soy....

Sunday night brought on less sleep (for everyone).  Monday morning, he was still running fevers so I put a call into the pediatrician to let them know how he had been doing and get advisement on if he should be seen and evaluated.   We weren't as worried about the fever itself but what it was doing to his body and how far it would go before he "snapped" out of it.   With his history of such prolonged and rough "falls" after a reaction, we began to worry.    I gave him his "safe" iron dose and within 30min he was screaming, screaming, screaming.  Inconsolable crying, agitation, mottling, drooling, hunched over-legs drawn up stomach pain, screaming.  I left a message for the GI doctor as he was now crossing over into concerning symptoms that she wanted to know about if he started to have.   I waited for return phone calls while trying to analyze what was going on and what next steps I need to do.   My mind automatically goes to next steps...what can I do?  What can I do to stop his pain, or at least ease it some?  What can I do to make sure this doesn't happen again?  What plan can I make for the other kids if I need to go to the ER if he should start vomiting?  Who can help me decipher through these symptoms?  So, yes- my anxiety was building.   I do my best to not let it show to Little man, he picks up on it so easily.  So, I hold him and hug him and kiss him and dance in the kitchen, and watch Baby Einstein, and try to get him to eat, and see if he wants to play...nothing is distracting him from his pain and the more I try, the more upset he is getting -- almost as if he was mad at me (maybe he is, maybe he is mad that I gave him the things that are making him now hurt).  I called his daddy at work to let him know the assessment of the morning.  He knew by my tone that things were not good, that things were all-too-familiar from previous reactions.   He came home immediately, his assessment- the same as mine....clearly reacting to something.  So, either red dye from Saturday (when his fevers and agitation started) or a build up to soy.    The good news is, Little man calmed down some for daddy....as long as he didn't try and put him down.   So, he cared for him while I dumped out all the soy formula and made a new batch of "safe" hemp milk formula.   Little man had already been starting to refuse to eat - is it the soy?  He will typically refuse something that he correlates with giving him a tummy ache.  Now he is mistrusting his hemp milk formula (something I've been very afraid of as I look for fortifiers for it).   He has a large blow out diaper and we decide he is going to need to be seen, even if our pediatrician hasn't called us back yet.  So, we see someone completely new.   All we wanted to know is if his fevers were related to a viral illness or not so we could continue to assess the situation and what we needed to do next.   She checked his ears, nose and throat and everything was clear.  There were no signs of a viral illness- although that doesn't mean there isn't one.   She wanted to help more, so she orders a hemmocult screen for his next stool per his daddy's request/insistence--which he kindly gave us a sample for when he got home from the appointment. 

Tuesday morning and the GI doctor called with results from the stool sample....positive.  With the symptoms since Saturday and a positive stool sample- she wanted him readmitted right away.  I agreed that I was worried about him but since he had not stooled yet that day, and seemed to be "holding" - we were trying to be optimistic that he would remain holding.....because Tuesday was his biggest brother's birthday (again, we try to maintain a quality of life not only for little man but for the whole family, through his illness).   GI doctor said to make sure to have little brother hug big brother-birthday boy because he was saving him from a hospital admission that day.   We were to keep her posted, if he continued to do ok- we could continue working on getting him back to a baseline at home before proceeding to next steps of a vitamin trial and follow up with her at our appt later in the week.   Our optimism didn't keep little man from going downhill that afternoon....

We turned in another stool sample that the GI doctor wanted in follow up to the positive blood one; and we did some blood work.   He had another rough night and clearly wasn't getting better,only taking in half the amount he is supposed to for formula for 3days now, and making less wet diapers and his lips were dry....our concern for him was growing.   So, this morning we called the GI doctor again and asked her to call us back right away.  She got the message and called us back, we reviewed the symptoms and all agreed he would need to be readmitted for monitoring to try and re-establish his baseline before proceeding with a vitamin trial and a re-trial of soy.

I am home now, tag team in effect again.   I needed to come home to make formula so we agreed that daddy would stay at the hospital with little man tonight....although he did ask today if this formula is something he can learn to make.  He can....but it's kinda like making candy - it is all in the feel of the stir....it is a delicate process and I have made a few batches that went down the drain. So, we'll save that for another day and for now- I am home to make the formula and be with the other boys.  Little man is at the hospital, with Daddy, and being observed....maybe today was the "peak" and he will do better overnight and tomorrow but if not- he is where he needs to be and I can rest my mind, even if it is just for tonight.  

Home

...from the hospital after a 1 week stay.   I am still processing through all that we accomplished, how much we were supported and cared for during this week at the hospital.   We are so very hopeful, and have been praying for, a better path of health for Little Man.   We hope conquering his anemia and this soy trial are getting us steps to that- giant leaps maybe even?

The symptoms we were concerned with with the introduction of soy did not build or worsen.  In fact, they seemed to even out a bit.  We may be dealing with some refluxing as his agitated states are when he gets up in the morning and when he wakes up from his nap, so that could explain that.   He doesn't reflux unless his body is not properly digesting a food. So, that is noted.    His flat affect was very concerning, his "poker face" as his daddy calls it- an expression-less deameanor with no interactive play.   But he didn't have that much today, and the refluxing pain was less as well.   We felt everything looked stable enough to at least continue this at home- sleeping in his own bed and playing with his brothers, getting back into our routine may help filter out his fussy spells and disturbed sleep -- or it may make it more clear.   

Whatever happens, we have a plan.   If things stay the same- we continue soy and have our follow up with GI at the end of next week.  If symptoms build, and are not vomiting/diarrhea/dehydration/lethargy- we call GI and go in for direct admission back to be monitored; if vomiting/diarrhea or concerns of dehydration and lethargy - we go right to the ER.   We have a plan, a "safety net".  We have a new level of stress relief, a heavy-heavy weight lifted off our shoulders. 

Little man giggled when we walked out to the van after being dismissed - as soon as he saw the van, he giggled.   He got excited to get into his carseat and sang on the way home, calling for "daddy" (his new favorite word). I've spent most of the time with him at the hospital, so he has missed his favorite playmate- daddy....and of course his brothers (we've been working on him learning how to say their names).  

We continue with soy, mixed into his formula - increasing every day...unless we see new symptoms that deserve attention.   I'm still nervous to call it a "pass".   Soy is so closely related to dairy and his dairy intolerance is so sensitive, we've never even "trialed" a whole dairy protein because of his reactions to dairy in my breastmilk and from crumbs and from hydrolyzed formula's.    What if he builds up an intolerance to soy?   What if we're pushing his body?   On the other hand, soy is a common FPIES trigger and if he could truly pass this, and thrive on it -- would really give us hope that we can do bigger food trials with more confidence.....although there is no rhyme or reason to FPIES triggers in kids but to have so many fails only under our belt- it will feel good to have a pass, especially such a big one.....

Why Soy?

We came to the hospital because of Little Man's severe anemia.   We went ahead with the transfusion and he responded well to it.   The next steps are twofold- to figure out what is going on with his digestion and absorption....why/how did his anemia get so low?  And food challenge turned into why are we struggling with new foods so much?  FPIES kids aren't usually so intolerant to everything.  We did one immediate test  (the Meckels scan) to rule out that as a source of the bleeding/low hemoglobin.   It was negative.   It was then decided that since we were here, and the GI on Little Man's team is on hospital service this week, what better time to do a food trial.     But what to trial?

Little man needs iron, protein sources are the richest sources of iron - meat being the highest.   We just do not think he is ready for digesting a meat yet when he has had so much trouble with everything else we've tried.   We had started discussing soy at the allergist 2 weeks ago, this allergist is somewhat familiar with FPIES, some of the things he said made me nervous (like pressing on with food trials if there was a little vomiting) but some things were ok, and he seemed to want to learn, and he is an allergist who was listening to my concerns.   So, we discussed his soy intolerance.  Do we remember vomiting with soy?  No, it was a muddy time when we were losing ground but do not specifically remember vomiting- spitting up, yes but vomiting no.   Colic and sleep disruption, and a nasty-nasty cough, yes.  Soy formula has corn syrup in it, we were doing rice around that time as well (or had recently stopped it).   My other boys were milk intolerant but did good on soy; and tolerated milk after 12-18mo.  Further discussions like along these thought processes with the new GI and we all feel it is worth the trial.  Maybe, just maybe he had a soy intolerance or sensitivity that he has outgrown- and was not FPIES for little man.   We hope. 

The GI here has not done FPIES food challenges so she wanted my input, she would create a flowsheet from our records of symptoms from little man's intolerance's.   Everything from reflux, diarrhea, agitation, sleep disturbance, mottling of his skin, hives, eczema flares, diaper rash, hyperactivity to the end all of vomiting and diarrhea.   There is no FPIES protocol here at this hospital, she is creating one to use for Little man.  She is hearing my concerns, addressing them, validating them and supporting me through this.   I simply can't ask for more at this point.  We will learn together.   Little man is a puzzle and we have a specialist finally interested in helping us solve it, one step at a time.   One step in the right direction is a good step.   Day by day is all we know to take this beast of a diagnosis.    So, we proceed.  

I obtained Organic Unsweetened Original Soy milk (WestSoy) from the grocery store and brought it in.  This is what we would use since it is the simplest form, although the hope is that we would possibly be able to add a soy formula- Ross Carbohydrate Free (free of corn) to his hemp formula to add the micro nutrients, and iron he needs while on no food.  But we start with the pure form of soy milk.

Yesterday, Little man was to receive 1oz. every 1hr for 4hrs and monitor for symptoms all day.   Well, Little man (who- remember, did not read the literature on his diagnosis!) decided he didn't want to take the 3rd serving of Soy milk.  We do not want to force feed him, so we decide to change the rules a bit (hey, these rules are being written for little man, I guess he wanted some input in them).   We waited until he was hungry and then gave him 2oz.  and by then he was ready for his nap so after a few additional ouncdes of his hemp formula and he fell asleep.  He slept 2hrs and awakened not quite rested.   He acted hungry, so we got more soy milk and hemp formula.  He ate it well and stayed a little fussy.  I got him more milk, was he still hungry?  He didn't want more milk and soon didn't really want anything- put him down, pick him up, arch his back, pull up his legs, scream,....this cycle went on for about 30-45 minutes.  I tried all the distractions that we have used during the hospital stay when he gets fussy or bored, nothing was working.  He screamed and screamed, his skin was mottled, there were small red spots on his chest that popped up, his stomach was tight and nothing was helping.   Finally he started to calm down and a new distraction got us ahead of the game.  He remained clingy and fussy until a little over an hour later when he had a bowel movement.  This diaper had mucous in it.   What was all this about?   Is it just digestion? Is it going to get worse? Is his body adjusting to the protein? Or was he just simply hungry or tired?  The rest of the evening was pretty uneventful.  Actually, it went better than uneventful  - he was happy, giggly, interactive, flirting with nurses, singing, playing little boy....maybe a bit too active, he was a bit restless in his sleep until 11pm when he finally settled into a deep sleep.  He slept well until 4am and then woke to eat.  Went back to sleep and was up to face the day at 5am.   He was restless and couldn't settle back into sleep; he did cuddle with me for about an hour and then we were up for the day at 6am but nothing was making him happy.  He wasn't agitated but he was fussy.  Something wasn't right in his world.  He had more to eat at 7am but his mood did not improve....neither did his color.   He was looking so washed out, pallor, bags under his eyes....where did that color go that we just had?  Pink cheeks? GONE!  Everyone noticed his new look, as I try to explain - and see if I can be heard this time around -- that this is one of the reasons why new foods become so difficult for us.  This goes on for days at home while we trial a food ounce by ounce....not clearly going towards FPIES fail but clearly not tolerating and thriving from the food either.   Stuck.

We pressed on.  6 more ounces of soy milk in 2 oz. increments when he was hungry enough to drink it.   He stayed "blah" all morning, took a nap mid-morning and was worse when he woke up!   Color off, flat affect- no expression, no playing, no interacting...had what his daddy calls his "poker face" on- just "blah".   How can this be ok?  This isnt' thriving anymore than not having the food is.   Then, as quickly as this all set in- it was gone.   Mid afternoon he finally moved away from me and started playing on his own, within minutes he was dancing and singing in the playroom!  He stayed happy demeanor all afternoon and evening.   We got a stool sample sent and some blood work.   Now we wait for those to come back to see if it offers any clues. 

We are all stumped.   A soy pass would open up windows.  Press on and he fails, he becomes so, so sick again.  We don't like these symptoms but do not feel they are enough to "fail" soy yet, although they are not enough to "pass" it yet either.   So, we will continue on tomorrow.....stay tuned

Next steps...

As promised, I am going to attempt to write about our new GI doctor and experience.    You see, I am still processing through how so very awesome she is, and the perfect fit for us and, we hope, someone to help us continue to solve our little FPIES puzzle.  As many know, I have struggled not only finding Little man's diagnosis but the team of physicians that would help me care for his needs enveloping this chronic illness.    This chronic illness that has become complex, so my drive to find the specialists he needs has been strong.   I can't do this on my own, no matter how strong I am, no matter my convictions, my instincts....I did not go to medical school and I need help making sure I am keeping him healthy through this.  

I may finally have this, help....and a team that is not only recognizing and acknowledging his diagnosis for what it is but is anxious to help with the ill effects he has suffered from it.   Amazing, truly amazing.  This journey has been long and we are no where near at the end of the road yet but to have a TEAM will help assure Little Man stays healthier despite his FPIES and will help support my caring for him through it.

With the transfusion successfully behind us on Monday morning, we were able to have a great discussion with the new GI we arranged to have Little Man's care switched over to.   Right away she was saying things that echoed my thoughts....much like we already have with our pediatrician -- someone who "gets" us, someone who hears and understands our concerns and uses that to help guide treatment within their knowledge and experience.  I have had a drive to only include physician's that I can trust....this is a clinical diagnosis, trust is big.  But clinical diagnosis aside, I have always felt this way about doctors.   You should always feel a trust in the medical professional that you depending on to assist you with any illness....this trust comes into play deeply when you/your child is very ill and your focus goes to only their immediate needs and not their medical record.   I need to be able to trust that the physicians caring for my children trust me, and I trust them.  

The GI doctor and I talked about past reactions, she wanted to know details of which foods they were, time frames, anything I could tell her.   Well, what couldn't I tell her?  I have kept a log from April on, and I had pages of notes from before that even.   I referenced back to this and I created a chart for her -- although it was 20pages long, I wanted her to know and see the patterns to his reactions....the "build" one's, the immediate ones, the questionable ones, and how we got to this point we are at now.....She loved it!  It was exactly what she wanted, and the format was perfect for her own self-declared OCD!   She wanted this list to help her understand MY perspective, not only what the many notes from the chart have to say but MY perspective that may not be included as well in the chart notes.  She wanted these records so we could leave the past in the past and only continue to move forward but not forgetting where we came from so taking what we need to learn from the past and utilizing it but always moving forward.  Little Man's health is top priority. My thoughts exactly.

Finding the source of his bleeding, or malabsorbtion, or what-is-making-his-iron-go-so-low "puzzle" is a priority for her.  So, she orders a Meckels Scan to check to be sure he does not have a Meckels diveritculum that could be causing pain and blood in stools.   After a whole day being NPO, the scan was finally done at 4pm and the results were read later - negative.  He does not have a Meckels diverticulum.    This was one of those times when that is both good news and bad news at the same time.   Good news that he doesn't have it and require surgery to remove it; but bad news because it would explain something and it is something that they could fix right away!   So, moving on....

The next test will be an iron absorption challenge.    This will test to see if he is absorbing his iron in the small intestine the way he should be.  We didn't go on to discuss a future scope but that would likely be next steps if this tests is also "negative".   The great GI we saw last week (for a 2nd opinion/resource/support) agreed that he would recommend a scope as well as checks on his other vitamin blood levels for clues into his absorbption.   I hope we solve that soon because last night, he had a diaper with blood in it.   Barely noticeable and it actually was "negative" on the hemocult test (blood in stool) but it may have been a sample from outside of the area in the diaper where the blood was.   The GI doctor and nurse last night both saw the diaper themselves.   The GI doctor is calling him her little "puzzle" (again, echoing my thoughts!)....he has been a puzzle.  

The key difference with her calling Little Man's FPIES a puzzle is that she isn't utilizing that to dismiss it but want to solve it more.  She said she would call it "Atypical FPIES" in that his presentation and complexity is not the typical course (he didn't read the research literature).   Maybe this is because his FPIES trigger is corn?  Corn is in so many things, his body has been "attacked" off and on for so long.  How much can one little body take? 

Corn is where his FPIES was born, dairy is scary, rice was not nice.  But soy - soy has always been questionable.  Clearly his body was not tolerating it at the time and we did not continue it after a few weeks of symptoms that were adding up.   But reviewing notes now, it could have been the corn syrup in the formula we were using, it could have been the rice he was getting at the time, or another food yet unidentified.   Two of our other boys were dairy intolerant, but did fine on soy; they also both outgrew their dairy intolerance's.   Discussions with Allergists and the new GI and we feel we have all of these things going for him; and the need for improved nutrition, and we're in the hospital in a routine by now....we decided to challenge soy!  

A soy pass would open up a lot of windows.   It is still a plant based protein, so the iron wouldn't be that much different than the iron he is getting now.   But soy is in as many things as corn- so to not have to worry about soy would be huge.    Preservatives and additives in foods, "processed on same equipment" foods, vitamins, legumes, and of course SOY!   And that only includes food- what about all the non-food?  Bath soap, toothpaste, crayons,....Ok, I'm getting ahead of myself because we are only 4hrs. in to his soy challenge but he has had 4oz. and is doing GREAT.  

It has been a whirlwind couple days and I will have more to update on later but I hope things puts a little window to how things have been for Little man over the past few days....

Pink cheeks

Little man has been spiraling and we were hoping this wasn't really happening again.  Hoping we could get a handle on things, that maybe it was just because of his general anemia, maybe we've been too busy, maybe he is just getting used to be carried.   By Thursday evening, I started to put more of his symptoms of the past week together, I had completed some logs for the week.... 

Let me pause here to talk about my logs.  I have kept track of little man's every ounce, reaction, symptom, and diaper since April.  I simply created a template in a word document, and we keep it in the kitchen.  It has helped on days when I am not home, it has helped to see patterns, it helps to look over things for the 2nd time when I am entering them in the computer (now at 80pages worth!).  

I used to try and do it every other day or so, but after finding his baseline- there is less to fill in except the ounces of his feedings and his diapers and then a few quick observations about the day or his sleeping patterns.   A few weeks ago, he got a crumb....this may have been the beginning of that slide.  Or is it from plums?  Or is something else going on?  Is he even absorbing his oral iron?  What is going on with Little man??  What do we do?  

Well, by Friday morning - my instincts were telling me that I would need to get a lab check on him along with the weight check I was planning on doing....that if we waited through the weekend we would likely end up in the same places we have before on Sunday afternoons with a very, scary sick little boy.  So, a quick call to the pediatrician's office and of course she orders his labs to be done after his weight check.   Weight check reveals a slight drop in his weights, which wasn't surprising as he has not been eating....like I last posted, a lot of coaxing just to get him to take a minimum of calories....

I was barely home from getting the lab draw done, little man was so very tired- asleep in the van, when the nurse called with the results....his hemoglobin had fallen to 5!!   My anxiety took over, and I simply cried, I ache for my little man.   My little man has been sick right under our noses....my instincts were right but not soon enough...how long had he been low like this?  What would we need to do now?  We have been so scared of a blood transfusion.  Of course, our pediatrician called us and decided to admit him for either IV iron transfusion or blood transfusion.

Because of the complexity of his FPIES and the need for his care to involve a care team, Little man has a new pediatrician.   This pediatrician is a consultant at the hospital, and was on this weekend.   We met with him after getting checked in.   We had a good discussion about where we were going with Little man's care, and how to best help him now.   His opinion was to do a blood transfusion, that he needed the red blood cells now- not an IV iron where he could potentially react and his body would still have to build up his red blood stores from the iron being put in.   We worried about reactions to both- IV iron and blood.   We decided that since he was being monitored, we could wait until morning to start anything so that we could all have some time to think about it....and pray.  

We knew what we had to do, it was time.   We just wanted to be assured it would be done in the safest way for little man's specific needs.   Not only was I worried about a transfusion reaction, or a blood product reaction, those worries were minimal compared to my worry about his low IgA and what that meant for him to receive blood, as well as just a reaction where his body would recognize foreign proteins on the blood and reject it.   I expressed all these concerns to the consultant pediatrician and he patiently addressed every one.  We were to think about it overnight and let him know in the morning.  We would proceed, we would put this in the doctors hands, by the power of God. 

The pediatrician not only listened but heard my concerns and he went one step further, to assure little man's safety.   They treated little man as if he was IgA deficient (which he is only low- not completely deficient).  With an IgA deficient recipient, the blood is either from an IgA deficient donor or it is washed- and washing it washes away proteins and particles that an IgA deficient person would 'attack'.    They were taking every precaution.    We had to trust that God was putting Little man on this path for a reason....

The transfusion started late in the day, around 3pm. It was a little uncomfortable for him at first.  Thankfully, little man's aunty K was able to come to be with his brothers so both his daddy and I could be with him.  

He tolerated this first bag well, and began to "perk up" at the end.

He had some sleep disturbance that night- seems like he is having some reflux/stomach pains and we are not sure what from but we haven't had reflux in so many months but it all comes back with a terrible familiarity. 

The next morning we found out his body responded nicely to the 50ml of packed red blood cells,going from 5.1 to 6.9 Hemoglobin!!    We proceeded with 100ml of packed red blood cells the next afternoon and tolerated this well too.    That evening, all the nurses were so excited to see his pink lips again- noting that his lips had become flush with his pale/pasty face.....I was noticing his pink cheeks....

This morning, his labs came back and his hemoglobin jumped to 10.5!!!  Such great news!! SOMETHING we finally have conquered!!  He tolerated it well and doesn't seem to be suffering any adverse affects from it.   Well, there is that reflux that reared it's ugly head again in the middle of the night again.   We are worried about what caused this decline....is he losing blood somewhere? Does he have a bleeding ulcer?  A GI bleed? Is he just not absorbing it?   Will it continue?   

Our goal of getting his hemoglobin restored has been accomplished.  Now we move on to GI.  We now have a new GI.  We are hopeful she will be the key to unlocking this little puzzle.....

I am off to bed now as Little man has a big day tomorrow with some tests and further consultation's with GI....so, to be continued.....