Setting up CHOP appointments, are we really doing this again?

Little Man's biggest issue right now is his anemia.  The hematologist here feels it is nothing but iron deficiency anemia and he will continue to have it with the diet he is on. As if I can just change that? We need a second opinion before he needs a 3rd blood transfusion.  Maybe this would be a benefit of going back to CHOP as well?  To at least have assurances that they acknowledge the FPIES mechanisms into considerations of why he continues to fight such threatening anemia.

No one, no one wants to feed my child more than I do. But how many times a day do you give your child something that will hurt them? Something is NOT right in his gut, and he will continue to build these reactions until we figure it out. And then he'll have no foods left to try because his gut will have pushed everything over his limit and attacked it. THEN we will be in more trouble than we are now.  We have a great GI, helping us at every step; but it is clear that we need more heads in the game, more opinions to get to some next steps.
So, it is decided.  With our GI's support, we will get appointments lined up at CHOP.  We called and immediately got a follow up appointment with Allergy for Aug.23. I have NO idea how we are getting there or how we are paying for yet another trip out east but I really feel we need to do this- for Allergy, GI and Hematology appointments, all while we are there.

Little Man still weighs 24#, he is 18% for height and 7th% for weight at today's appt. No weight gain in many weeks, months? We need to get labs done, check CBC so we can see how his hemoglobin is doing, and GI wants to run a full immune panel as well (something he had done in the very beginning) to have fresh labs done for CHOP. We also need to do IV iron again soon. We await to hear if/when our GI got his other appts scheduled for, so we can work on finalizing the plans....

Time for CHOP follow up?

We prepared all weekend for to discuss the plan further with our GI doctor, we were beginning to feel alone again.  The isolation that comes when the doctors simply do not know what to do next and your child is getting more sick by the day.  It is a scary beyond description isolation.  We've already been down that road, alone.  We don't want to be there again.  We discuss with the GI that they would like to see us push through some reaction symptoms, to possibly trial steroids, to just try something.  We aren't getting anywhere with the plan we've had; but we are all nervous about pushing ahead and making him more sick- especially as he's already sliding...although over the past week he seems to have been turning a corner again? 

We talk more about his biopsy results and that they show that he has inflammation which results in some villi damage but that he does not have atrophy, like he did in Dec.when we did the TPN last. We know he would benefit from the added calories/nutrients to help him heal faster but he IS holding- his weight is holding, his appetite is holding, he is holding. AND the past few days, he's been doing well. And really, he's done more well than not, since his transfusion in June - so maybe this inflammation is from before that and he is on the upscale of healing?

We've discussed getting him a follow up at CHOP- it's been a year and that was the plan....to return for a follow up.  And it is becoming more obvious we need not only a follow up with Allergy, but a consult with GI and with hematology as well.  We discuss this with our GI and she is in support of us going back and getting some further insights before we make next steps.

We have a good discussion about where we've been, where we are at now and where we are going....I break down, it's been a really stressful week, I'm over tired and I've begun to fear for my little boys life. Not how he is right now- he's stable, doing ok actually but how did we get back to severe anemia? Why does he have blood in his stools and how many more blood transfusions will he have before we figure this out? I can not just sit here and treat symptoms like this, I need to be proactive in his care again....we need to move ahead of where he is at.

We decide that we can hold off on TPN for now, there is just too many other things at play here and he seems to be stable- despite the ulcer-like inflammation we visualized on scope, his body is coping. He has started drinking more of his bottles and appears to be responding well to the new/change in probitoics; so we agree that we can wait. IF he doesn't turn around, starts to lose weight, or something we do decide to do, pushes him over- we can do TPN then. GI's main goal is to maintain Little Man's health while he grows and thrives. Good, exactly our goal as well.

This is a huge relief, and my head is spinning....what a long day...

Multi-layered treatment plan?

A lot to think about, alot to process.   Scope, Functional medicine and intestinal healing, new probiotics, thinking about steroids, declining little man, considering TPN again.   What do we do?  

I feel like Little Man's treatment really needs to be layered right now:

1). Healing his villi/restoring his nutrition, his immune system will be out of whack just because of his lack of micronutrients alone. Although I am grateful Hemp is a very nutritious milk and that his body tolerates it well, I am constantly worried about his missing micronutrients. It is the sole reason why we keep pushing things with him- but also the reasons why we aren't getting anywhere...

2). Healing his gut -the Functional Medicine approach is something I can appreciate and really feel strongly that we need to incorporate it into Little Man's big picture and long-term healing. We will work through some new probiotic suggestions- more customized for little man.  Realizing probiotics and restoring his intestinal flora will take a long time- but I need to do these steps to assure not only that we are doing everything we can do now to help him thrive through this illness but also to protect his gut from the things he is at risk for in the future. We've already seen that the addition of probiotics in March had been a major significance to his health when we added them, so I do feel this gut healing is essential for his course.
3). Allergy/Immunology consults- a return to CHOP?? Dr.J (research MD in NJ) mentioned the need for further research into FPIES kids who do not respond to the current treatment of elemental/elimination diet in healing them....that there is further immune system involvement with these kids.

It's time to make some big changes again, we are just too stuck with current plans and he is declining again.   

Reflections on a year....

On a year since our trip to CHOP, since receiving FPIES diagnosis, confirmation of what we suspected, since his 1st birthday.....

I recently found a post I did on babycenter for this very trip, taken a year ago....I titled it "Anxious for our consult at CHOP", and wrote....Ok, talk about flooding the message boards but when alot of new things start surfacing or tying into my son's issues, I get a little hungry (no pun intended) for knowledge! Learning from all if you is helping SO much, thank you for listening to me! ;)
We have our appt in a week, we will travel from MN to PA for this 2nd opinion, and as my son only continues to get sicker- I am anxious to go and see what their evaluation will bring.
I know so many of you are in the same boat, and it is good to see all the support...thank you for letting me learn from you all! No doubt I'll have a few more things to add in the next week as my mind remains ON this upcoming evaluation!
If anyone has any suggestions for what to ask or say while we are there, I'd appreciate it. I got us an appointment with a Dietitian that day as well, we gave a great Dietitian here but wanted to get insights from them to bring back here too. We are just so excited at the possibility of getting some answers and recognition for what our son goes through on a daily basis.

Wow?!  We have come SO far in one year!!  One year to diagnosis, another year to healing.     We went to CHOP for the diagnosis and with the consult of Dr.Pawlowski we were able to get a handle, and control of his allergy triggers....but not after the damage to his intestines had already been done.   Which brought us to our next doctor, a GI doctor who helped heal his inflammation (and confirm his soy intolerance)!  Our next stop was the PCRCD for a consult with Dr.Jyonouchi and her expertize on protein intolerances and the "root" of his allergy and beginning stages of healing his leaky gut.  We have learned, done, and accomplished a lot in a year.  

Recently, we had a benefit for our Little Man, to raise awareness and funds for research as well as funds for his medical and specialized food expenses.   His godmother, my sweet sister-in-law, made a slideshow to share his story.   I have turned that slideshow into a video to share here: FPIES: Our Little Man's journey.

Research

Having a child with FPIES leads to daily research...research we don't even realize we are doing.   Research and experiments to see what foods they can tolerate, research to find which probiotic to use, which medication they can tolerate, do they need an enzyme, why is his iron low, which doctors are the most FPIES knowledgeable, what that test means, what scope results tells us, which foods are safe, which symptoms mean trigger and which ones are intolerance's or related to a cold or a 'normal' toddler behavior....it is every.single.day.   If it isn't being played out, it is on my mind.   What is our next step, where do I need to focus my research. 

It first happened last fall when we saw a GI doctor on 2nd consult, he called Little Man "FPIES plus"...he has FPIES to some proteins but something more is going on....but what is that?   My research led me to Non-IgE food allergy.  It appears the foods he doesn't have FPIES to, he risks Non-IgE allergy to.  Non-IgE allergy is a protein intolerance, of which FPIES is the severe end of the spectrum (in my opinion).   But how do we avoid his Non-IgE protein intolerant responses from becoming FPIES trigger reactions? 

A visit to the PCRCD with Dr.J and her advice is to correct the gut flora- re-establish his gut homeostasis with probiotics, and enzymes if needed (Little man has not been advised to start enzymes as his doctors fear he will react to them at this time- it is something we will re-explore in the future).   Dr.J was even concerned he may react to a probiotic.  She even gave him an oral steroid as her experience was telling her, he may need this to even tolerate the probiotic to re-establish his gut flora to achieve gut homeostasis, and not be so reactive to all foods.   As my last post illustrates, he did not tolerate the probiotic.   Now what?  Oral steroids?   I am not sure.   Steroids can wreck havoc on gut flora too.  

We go to see an Immunologist tomorrow, maybe we'll find a few more answers there.....but I have felt the need to bump up my research once again- to search for next steps to explore our little man's "FPIES plus". 

In the midst of researching next steps, I am also helping with Little Man's Benefit/Fundraiser.   I have already received so much help- from my sister, and a fellow FPIES mom who made the fliers you see/will see displayed.   I am so very grateful for the community involvement for supporting our family through this difficult time.   The goal of the benefit is to raise awareness, and share with our community the story of FPIES in meeting our son.   The goal of the fundraiser to raise funds for CHOP research; so that moms like me do not have to research every step of the way, so that doctors are better educated and aware of the symptoms and complexity's of this allergy, so that research can begin to see what the parents see in the day to day of FPIES symptoms, reactions, intolerance's....our daily research.   I have hopes that the CHOP research will pick up where other studies have left off.   Past research has already brought us to FPIES being a T-cell response, a Non-IgE food allergy.  Research has begun to define the Tcells and cytokines involved in these FPIES responses.   More research is needed on the immunological responses, on genetics, on allergy, on the guts role in FPIES.   Although there are little current FPIES specific study's being conducted, there are studies being done on oral food challenges (at Mt.Sinai), and on T-cell mediated responses (across the country) as well as the Non-IgE food allergy cytokine breakdown (at PCRCD).   For more information on research being done on Non-IgE food allergy that applies to FPIES read here for information on the research currently being conducted at the PCRCD. 

CHOP: "Hope Lives Here".   Even if Little man will not benefit directly from the studies we are raising money for....FPIES will benefit from Little man.  The research will be important to defining FPIES, to defining better treatment protocols, so that even if a child with Little man's "FPIES plus" presents in a specialist office, if FPIES is well-defined and treated....maybe, just maybe....the symptoms that arise from it can be well addressed before they become complex.

"Come Eat for Sam" Benefit

Samuel is now 21mo.old, and was diagnosed with FPIES at 11mo.at CHOP; although he had chronic illness for months prior to the diagnosis. Samuel is our 4th child, 2 of our other boys had milk protein intolerance's so when Samuel started to show sensitivity to my breast milk, (gassy, "colicky", spit-up, loose stools, disturbed sleep) we assumed he had the same troubles with milk. A trial of formula confirmed this, with projectile vomiting and diarrhea and an increase of his intolerance's. Reflux crept in, so we tried to add rice cereal to his diet, thinking this would control the reflux activity of a breast-fed only baby. He tolerated this for awhile and then started to refuse it, had increased spit up, fussiness, disturbed sleep, increased reflux. This was the course of things for the next few months- every effort we tried to control his symptoms, resulted in him getting progressively worse.

We consulted our pediatrician at 6mo.; with a referral to gastroenterology shortly after as he began to fall significantly on the growth chart despite a no-milk/no-soy/no-wheat elimination diet. He underwent his first scopes, which ruled out Celiac disease and EosinophilicEsophagitis, but found non-specific inflammation and lymph nodular hyperplasia. We took him off of breast milk and all foods and put him on a strict elemental formula-only diet (Elecare followed by Neocate). For Samuel, this is when things went from bad to worse.

Samuel stayed exclusively on the elemental diet for 2 mos. We were advised that his intestinal inflammation would be healed and could re-start him on foods. We started with rice....because no one is allergic to rice, right? We quickly got our answer after a tiny handful of rice puffs brought on days of vomiting. Even after recovering from the rice reaction, Samuel continued to be sick on a Neocate only diet. He would vomit so violently when we tried to give him Neocate Nutra- the retching- stomach-emptying-vomiting that came with this elemental based-medical "food" was un-nerving.

We knew something was horribly wrong...but specialists couldn't help because no one had heard of this thing called FPIES. I researched day and night, read every journal article I could find, brought everything to our pediatrician...and then decided we needed to see a specialist familiar with this rare illness, if we had any hope of a direction. We went to CHOP, we traveled from Minnesota to Pennsylvania in an RV loaned from friends (bless their hearts) with our 4 children and got confirmation of the diagnosis of FPIES. We returned, hopeful we could finally have a path to wellness for our little man; only to quickly find that even with a diagnosis- too little is known on how to treat this rare disease.

With Samuel getting increasingly sick from his formula, and a growing suspicion of corn allergy; we trialed corn. It is difficult to put into words how scary this time was for us as we watched his little body try to re-coop from such a violent reaction to such a small amount of food. We had no help, his doctors did not understood what was happening to him, or believe that it was indeed from the very things that were supposed to be nourishing him. He suffered from vomiting, diarrhea, lethargy, dehydration, high fevers, a febrile seizure...and still, the mechanisms of his disease were not understood by his doctors. Our Allergist at CHOP had advised he would continue to decline if we did not remove his triggers- in every form including the corn syrup of his formula, and medications. The mucous/bloody diapers, the continued vomiting, the refusal to eat, the spiraling from the inflammation in his intestines....would not stop until we removed his triggers from his diet.

We were scared, we held him all day, even in his sleep as we were afraid for him to sleep alone for fear we would lose him in his sleep. We needed to put him on another source of nourishment for his body to heal; unfortunately all formulas have either dairy or corn in them, this is when we started him on Hemp milk - he drank 50oz.the first day! His body perked up and he came out of the very scary stages he had been in for all those days....we knew then that we would need to move away from the elemental formula and build him one out of Hemp milk. He now thrives on Hemp milk formula but continues to not tolerate food introductions. His FPIES is chronic, and has become complex- it is "atypical"; his body cannot properly shut down its attacking mechanisms once it begins to perceive a food as a toxin. I continue to research daily into his mechanisms and complexities. More medical research is needed into this rare disease so that other infants do not need to experience such complexities from this diagnosis. So that recognition of it is a reality in doctor's offices, and treatment protocols are uniformly established and begun immediately. So that support is found in specialist offices. So that everybody suffers a little less and eats a little more.

Come Eat for Sam....Join us in our Fundraiser/Benefit Event!!

Also visit my Fundraising page: CHOP FPIES United Family Fund

Thanks for all the support!!



FPIES Experts

I've had this post sitting in "edit" since Dec.22.   I sat in the hospital room with our Little Man, recording his daily activities (intakes, outputs, symptoms, etc) in my logs as I always do, watching over the plan laid out by doctors, checking on ingredients of things to be put in his body or on his body.  I wanted to trust doctors, doctors who I know have my Little Man's best interest in mind but also doctors who have no experience or prior knowledge with FPIES- or really anything like it.  Instead, I have had to become knowledgable in FPIES.

I've been finishing this post in my head with how I want to desribe what an FPIES expert is.   Lets start with the definition of Expert from: merriam-webster.com: "having, involving, or displaying special skill or knowledge derived from training or experience".   Well, my training is in nutrition and my experience is a 4th time mom with first-hand, deep-in-the-trenches FPIES experience.

Parents are experts in their own children, they know their moods, their cries, their personalities, their likes and their dislikes.  To add to that, the parents of FPIES children quickly become experts in FPIES.  We have to, there is so little known.    Yes, there are studies, such as FPIES caused by Solid Food Proteins and FPIES: Case study presentations and management lessons but there is only so much everyday information that one can gather from these studies.   At this time, the most I have learned is from other moms, found in online communities for support.   We would all like to see that changed, so future children do not have to suffer as ours have - searching for a diagnosis, a direction, a baseline.

A few days ago, I did a post on what FPIES has taught me.   Of course, having a son with a chronic illness has taught me a lot about patience, advocacy, perseverance, strength, love, and all that life has to offer.   But I am talking about what FPIES, first hand, has taught me about Non-IgE food allergies, delayed-gut, food allergies.   About digestion and GI health.   About food, and how it is made,processed and produced in this country.   All these things, I have been taught/trained in- I have a degree in Nutrition....but none of these things I understood the way I understand them now, having learned things from experience.   What I haven't learned first-hand, I have learned from other moms- with first hand experiences.  Connecting all this together for real FPIES information, real help.  

This brings me to my daily question: if I'm an "FPIES expert"- then why is my son struggling so much to find a diet?  To tolerate foods?  God is giving me a very important, and strong life lesson.  He sent Little Man because He knew he was strong enough to endure this- to teach me, to teach his daddy, to teach his doctors, all to help other children.   He knew that with my background, and my nature to want to help others- He could use me as an instrument. 

If Little Man hadn't been as sick as he was, in pain daily we would not have made the trip half way across the country to take him to an expert Allergist,  if he had tolerated the Elemental formula, if we didn't have to fight with him to eat just half of his needed calories from that formula, to remain on it exclusively- keeping food from him for 5mo. with only worsening symptoms (and inflammation) - I would not have had the drive to make my own formula for him.  If Little Man didn't have continued intolerance's, making his illness complex - I wouldn't have pushed for 2nd opinions and a change in doctors on his Medical Team for his care.  If we hadn't done a soy trial- would we have known about the villous atrophy component of his FPIES?   God is putting all this in our path, all the atypical FPIES experiences in one Little boy so that his doctors will learn and help other children; and so we will learn and pass along what we have learned in hopes of helping other children/families. 

We, the moms, are FPIES experts, and the learning continues daily....

Meeting another FPIES child and mom!

Today R and N got to go home from their week long stay at this same hospital where Little Man stayed a month ago. I got to meet them....we've already met online and I knew they were coming and was excited to finally get to meet another FPIES kiddo and the strength that stands behind them.

And strength I met. R is a super mom, little N has been sick most of her life like Little Man. We share many commonalities in our children and our experiences. Little N has had a tough-tough road and, as a testimony to her parents sacrifices, she looks just like any other 10mo. old little girl. She is adorable. I look forward to having her and Little Man meet the next time they come to town.

Progress is made in small steps. The doctors here have now seen at least 2 FPIES cases within a months time, will they start to utilize this great opportunity to learn more so they can continue to help? Continue to be the world renowned hospital that provides hope to so many other families for so many other rare illnesses?

Only time will tell.

During our Dietitian visit at CHOP, we commented how it was strange that this fine medical institute does not see this. To which our Dietitian replied..."oh, they see it- I have no doubts they see it, they just don't recognize what they are seeing". She went on to say that CHOP didn't either a few years back but with continued EE cases, they kept getting this subset of kids with the same common problems. Once they began to recognize it, they moved in strides to begin to help. CHOP is a leading FPIES medical facility. I do so wish that we lived in a place that understood, recognized and knew next steps for Little Man's rare illness but try to keep the positive perspective that by caring for Little Man (and Little N), they will only learn and be better equipped to help the next FPIES case that comes through the door, and then continue to build their knowledge from experiences gained. In my world, there is always a positive side to things....despite not getting the full support we need for our child, we hope and pray that others soon will.

I just don't understand why it has to be so hard

Caring for a sick child is hard enough, and I have many levels of support and coping for this.....this isn't even the hard part anymore (especially since I have built him a formula that makes his tummy happy and gives us a happy, active boy). Why do we have to fight just to get him a little help? Why does a child with a clinical diagnosis deserve so much less than a child with another chronic illness?

No, I wouldn't say our Peds GI was ready for us. They are not ready for FPIES diagnosis here. It makes me sad- sad for my little man and sad for all the other kids who could be helped by such a fine medical institute.

The GI doctor even said it herself, little man may have too complicated a case for them here....we may have to go back to CHOP. If his labs and weights, and blood in stools don't stop on this formula I have built him- they are not sure what to do. So, no- no answers....not much direction than I am already doing -- on my own. No more support than I had yesterday or the day before, or the day before from the medical community.

The support I want isn't hand-holding support. My group of FPIES mommy's help with that, my family and friends have always been there through the very beginning stages of this. The support I don't understand why is too much to ask for is support for caring for a sick child. That if he were to get sick- from a food fail or even a crumb or heaven forbid he got the flu....that I would have assurances that I have doctors that will help us help him.

Is that really too much to ask?

Does it really need to be this hard?

Crumbs are an issue

"Crumbs are absolutely an issue, and (looking around the room at our other boys)...I'd say you've got a management issue". Words from our Allergist at CHOP when we asked about how bad crumb ingestion came into play, we had quite the little crumb scavenger.

We took all food, except the elemental formula, away from Little Man at the end of Feb. Yes, that's right- he has not had food in 6mo. He was almost 9mo. old, he knew what he was missing from a "I put everything in my mouth" perspective.

We knew we had to get a tighter control of food in our house because we could not become a dairy/soy/rice/corn free home without it affecting our other boys. Their little brother has FPIES, but they do not. They have lived 9,7,and 3yrs without these restrictions. They are people too, they have needs too (and even their own dietary restrictions). They are also growing and need the nutrition that these foods provide.

We instituted a "no snacking outside the kitchen" rule, we locked the pantry, we got rid of all "snacky" foods, we began to teach his older brothers about the importance of "no crumbs", we tried to get them to eat sufficient enough calories in their "3 square" meals rather than needing snacks (although, personally-I believe 5-6small meals/day are healthier for our digestion than the Standard American Diet). We also made hemp popsicles, and hemp seed cereal, and ice chips - things little man can "play" with (even if he doesn't eat) in a high chair while his brothers eat.

We have tried countless techniques and are always looking for more/better ones to continue to manage a houseful of growing boys when one of them can not have food.

Maybe I stress about it too much but a crumb the size of your fingernail was Little Man's last, and one of his most repeated reactions (the vomiting didn't stop when he emptied his stomach, he ate and it started all over again!). A crumb from a cracker that escaped the soup bowl at lunch one day....a crumb.

Crumbs make him sick. Little Man can't have crumbs. Mudpies (aka big brothers) know this but our mudpie#3, who is 3 can not separate this from his own needs -- if he's hungry (from not eating enough at the meal is the biggest culprit of that), he wants food. It's a natural thing for a toddler.....disrupted by Little Man's FPIES.

The other day, when Little Man was having his tummy ache from millet grits- mudpie#3 was hungry for a snack (and maybe just wanted an escape from the screaming). So, upstairs he headed and unlocked the pantry (this I heard him doing). A few minutes later I went upstairs to check on him (I'm carrying Little Man around the house at this point- so know he is "safe")....I expected to find him in the kitchen, at the table (at the least). Nope, not there- but the pantry is wide open - so he's got a snack and eating it somewhere. I call out to him....and I hear, from in the living room, "yumm, yumm, I'm a cookie monster. I eat cookies. No, no, no crumbs for Sam, crumbs make Sam sick"....points for him as he was eating over the container.

Irony is- the cookies were bought in a Mommy guilt moment for them.

Millet Trial, day 3

No, you didn't miss a post. Day 2 was a day off (from millet). Little Man had too many loose stools from the millet grits, his system needed a break. This is an added component of Little Man's FPIES. He isn't the only one with it, our RD at CHOP is the one who alerted me to it. His scope confirms what we already knew: he has a carbohydrate intolerance. He can not fully digest the same amount of carbohydrates as other kids his age (FPIES or not), so if he gets over his "load" - his body will let it out in explosive diarrhea.

Mudpie #2 (aka 2nd son) has lactose intolerance that developed last year. I connected his issues down to milk (lactose=milk sugar). There isn't many non-invasive tests for a lactose intolerance diagnosis confirmation. So I relied on the tried and true elimination and reintroduction process. I pulled out all milk from his diet without him noticing for a week or so, and then I tested it with a nice big glass of milk one afternoon and sure enough, he was running to the bathroom. I asked him if he was having cramps- being six, he didn't know what cramps meant so he described to me..."it feels like firecrackers going off right here(pointing to his lower abdomen)". He has a way with words! And I couldn't think of a better way to describe it. Off to the store to buy lactaid pills and milk and problem solved. He doesn't have any of those bathroom trips if he takes his lactaid or drinks the lactaid treated milk. Lactose intolerance is the easy one of the carbohydrate intolerance's to fix. Although some people do have it so severe- they can't have any lactose in their diet, even with the help of lactaid...but I digress. Lactose intolerance is what most people are familiar with since it is the most common of the carbohydrate intolerances. It is the same result, from any of the different carbohydrates (milk sugar, starch sugar, fruit sugar....) for someone with a generalized carbohydrate intolerance like Little Man.

Firecrackers is what Little man was having- but he didn't even mind. He had some initital tummy upset from the Millet grits but never did have any issues after that; although the diarrhea continued all day and night and into yesterday with one loose diaper. Yesterday we didn't give him any millet.

Today? Today he had a COMPLETELY NORMAL BABY POO diaper!! If you're an FPIES mommy reading this- you're giggling...I know you are! If you're not an FPIES mommy, you're wondering why the extra excitement over poo! FPIES ravages their entire digestion, normal poo's are not their normal....unless things are right in their world! I have 4 boys - Little Man is #4. And I have changed many poo diapers in my life even before them - I have done daycare, I have babysat, I started changing diapers when I was 9yrs old (since I am #2 of 7 children it was par for the course)....I have never, never seen such a range of weird poo as I have from one child- from my Little Man.

So, today- millet puffs arrived (thanks M!!!) and we continue with millet trial. I "cooked" them with some hemp milk into a cereal and he ate some just puff plain. He LOVES them!! We will do this same amount for the next few days and then look to increase it as we go. I am still holding out hope that this will be the wrench in this viscous cycle of a ferris wheel ride ON this FPIES roller coaster ride!

Mom Driven Diagnosis

When I first was researching FPIES, I began researching through the medical journal articles- just as I had with other countless hours of research I was doing to try and connect something that would make sense to what was going on with my little man. I read through almost all of the medical journal articles but there really are only a dozen or so of those specific to FPIES. So, I started going into support groups (Facebook, Kids with Food Allergys, Babycenter are the main/active ones). This was the next obvious step --listen to what other mom's were saying....I kept hearing things like "mom-driven diagnosis" and not to mention all the things that made it sound like all the moms were standing in my living room!

Mom driven diagnosis is where I began to realize that yes, the mom's that are "in the trenches" ARE what is driving the recognition and continued research on this FPIES beast. And that I really did know what was best for my child...and that what I was learning from the other moms was more specific than the doctors that didn't know about FPIES. Diagnosis and treatment has come such a long way in just 5yrs, and I feel SOOOO fortunate for the moms that have been before me that have shared their story's, thus helping our situation....my hope would be the same for the mom's that follow us. Because even though I shudder that other little one's have to go through this- it is happening and so to have a place to go, a place of support and guidance is SO INVALUABLE!

The big hospitals that know FPIES well (Mt.Sinai and Children's Hospital of Philadelphia) have teams of knowledgable doctors. But not all of us live in or near NY or PA. There are plenty of other Allergists throughout the country that have become familiar with FPIES as well. Allergists are at the fore-front of diagnosis and treatment but a GI doctor, a Dietitian and a Pediatrician are all part of the team. These "big" hospitals that treat FPIES utilize this team approach. It has been my goal to duplicate that for my son, here at our hospital.

I value the assistance from the doctors, specialists and dietitians to help me keep my little man healthy. The doctors that have experience in FPIES have a wealth of information and assistance; and the ones that don't can catch up very quickly- they already have their knowledge in kids, allergys, GI disorders.

The two things that a doctor has to do to help a child with FPIES: 1. acknowledge the diagnosis and 2. listen to the parents.

Our Allergist at CHOP is incredibly knowledgable - because he has helped hundrends of kids with FPIES and thousands of other allergic disorders. I will never regret making the trip half way across the country to go to CHOP to meet with the expert allergists for the FPIES diagnosis. After much review of all the literature we could get a hold of, our pediatrician agreed that little man has FPIES, but we wanted to know from a specialist that has diagnosed and treated it before -- for nothing else but little man's health. We needed to know what the best treatment plan is. We were given a good treatment plan, and our Allergist remains a strong support for us as we continue to navigate through these murkey waters.

The other step that has been invaluable was my request for a consult to a dietitian (here and at CHOP -- it was not suggested, I requested it). FPIES is all about food, dietitian's are a great support and advocate for the little one's

I truly believe that the parents are a huge and vitally important member of the team, also the lead member in treating these little kiddo's. Our Allergist has given us good advice on how he best is able to treat such a clinical diagnosis in a medical community that operates on objectives...."listen to the parents"..."if the parents say there is something wrong..."..."there are no labs or tests to test for FPIES, if I feel a lab is needed, I know I need to talk to the parents again". We respect this because we know our son. We have had to follow his development, his tolerances and intolerances, his "normal", his sick days for every second of his life.

We. Know. Our. Son.


Navigating the specialists, and playing Dr.Mom in search of a diagnosis makes one want to put all the medical field behind us and just do it alone. It is a very frustrating diagnosis in this way. For me, I could go it alone -- back when things were rough with the medical community trying to convince them of what was happening with our son, my husband urged with me for us to go it alone -- because he completly trusts my ability to do so. There is a drive to do so -- he is our son afterall, I should be able to care for him- I DO know what is best for him...only, with FPIES, it is like being a first time mom with a newborn baby and you look to check on that thing they are doing is normal only to find out that one around you knows anything about newborn babies. So, even if I could possibly understand all of FPIES (which I never will), and even if the doctors aren't familiar with treating FPIES, there are co-factoring/co-compounding issues that arise and I feel it is helpful for the doctors to have a good baseline of where my son's normal is and what steps we have taken. But we are absolutly leaders on his medical team.

Bottom line- mommy knows best. Trust your instincts.

Are you reading this because you suspect your little one might have FPIES? Like I said, the support groups of mom of FPIES kids has been great. FPIES is a little known diagnosis. The moms are raising awareness but there are still way too many doctors that are not familiar with it. So, if you're looking for a place to start with journal articles to read and bring to your pediatrician. I have started a list. I am linking it here- please note it is a work in progress.

http://fpiesmudpies.blogspot.com/2010/06/do-you-suspect-fpies.html

"Hi!"

Little man can't stop saying it! To us, to his brothers, to friends, to strangers, to doctors, to nurses. The mask has been lifted, even if just briefly, again. Little man has lived so many of his days in pain, that he doesn't waste any time getting down to playing and learning new milestones, and words when he is pain free. He can say "hi","hi-5", "daddy", "momma", "woof", "quack", "neigh", "off", "on", "all done". He can give hi-5, wave bye-bye, clap, ask for his bottle, and he's learning puzzles. He also can walk and run of course! :) It is simply amazing to us that he catches up so fast to these milestones when he has less time to practice (because he has spent so much time in our arms, in pain).

We are very grateful we have pulled him out of his failure to thrive state he was heading down. Many FPIES kiddo's have this somewhere in their days, sometimes before diagnosis when little is understood about what is going on, and sometimes after when the right diet is being identified with food trials and eliminations. We have been running close to the wire for so many months, and we do our best to stay on top of his illness so we can learn more about how it is affecting him.

Little man had a follow up with his pediatrician yesterday (he now weighs in at 20#12oz!!!). The major Children's Hospitals (Mt.Sinai, CHOP, Jewish National) utilize a team approach for following these kiddo's and I am trying to duplicate that here- it makes perfect sense. Pediatrician's are at the center of their care, as they help manage the care of the "team" when so many things come into play- Allergist recommendations, GI work ups, Dietitian follow-ups. It all has to be monitored closely to be sure he stays thriving. I know this is where it would help to be at a place like CHOP (where our Allergist is) but we don't live in or near PA, so we are trying to make the best of what we have here. And what we have here, practically in our backyard, is one of the top leading medical facilities in the country- in the world! The doctors that want to learn about FPIES have been great, and they are really trying to help. Our pediatrician is great, and has been through all of this....but I still feel a disconnect between understanding FPIES and helping Little Man. I wish I could figure out how to get this better connected. The many days, months, and now going on a year of Little man being ill and monitoring and connecting his symptoms, and finding a diagnosis to explain it all, and managing his care, on top of him continuing to be sick, and on top of the other everyday things in life- our other children and their needs, our jobs, our home, etc....is all starting to get overwhelming when I also still feel I need to teach everyone about FPIES while trying to connect how it affects Little Man...most especially when he is as ill as he was before being hospitalized. We are trying so hard to find his baseline so we can build his tiny menu. It will be more difficult to do food trials when he is still having blood in stools and waking up screaming at night.

Dietitians

I believe I've said this before....but it always warrents repeating! Thank goodness for the Dietitian's in Sam's care!!! It was an RD who helped us get to the diagnosis, and today big thanks to our RD at CHOP and our inpatient hospital RD at Mayo.

We struggled finding a baseline for Little Man, we visited with our Allergist at CHOP who suggested his intolerances could suggest a corn intolerance and trace corn proteins in the Neocate formula could be preventing the inflammatory process in his body from calming down enough to find him a baseline to build from. We trialed corn and sure enough, after just 1tsp, failed it, we pushed it another 1/2tsp to be absolutly sure what we were seeing was a true FPIES response and we got our answer. The true beast of this syndrome is that you have to feed the child the food that he could react so violently to to know if he is intolerant or not....it is a terrible thing to watch, I can only imagine what a terrible thing to experience. Little man let us know what a terrible thing by restricting his intakes of the formula, to less than half his needs for over a week...he got so weak, so fragile. We then started him on Hemp milk, putting it half/half in his Neocate bottles and he began to rapidly improve. We started to move further away from Neocate and it's trace corn proteins to more and more hemp milk - he was doing beautifully. We were planning on building up the hemp milk to equal the nutrition in his forumla; while we moved away from the Neocate- adding the nutrients we felt he needed in addition to the Neocate, starting with the starch. The addition of the starch coincided with the first blood in stools we have seen in 13mo. We stopped starch and gave his body a 3day rest, and then restarted; only to have everything come sweeping back- with full force of the worst watery explosive stools I have ever seen! This drained little man so much, so we tried other starches- with no better effects. Now little man isn't trusting his milk again and limits his Hemp milk to less than 1/2 of what he has been taking- going down to the same mere 400 calories he did with the Neocate after corn fail, only with the hemp milk (that is low on carbohydrate) - not getting adequate calories meant he wouldn't get enough glucose for his body to feed his brain....which is the state he entered in to on the weekend before his hospitalization. We knew he was sick, we just didn't know how to get the right help for him. We also knew we had an appt with his pediatrician right away that Monday morning; and knew she could help.

That was one week ago. Where did the week go. I find myself wondering if the doctors here knew about FPIES and what they were really seeing in Little Man upon admission was not malnutrition but failure to thrive from the foods we give him.....would we be ahead of where we are at today?

The focus is on his nutrition. A growing baby needs nutrition first and foremost for brain growth. Little Man's body knew this, and knew what to do to give it to him....burn his own stores to make glucose for his brain- it is a state called acidosis. Little man's body was starting to go into acidosis because his stores were so low....from being so sick since corn fail; and not from a diet of hemp milk.

Which brings us to today, where everyone can hopefully start to catch up. Dr.P at CHOP has given us some great advice...."the parents know the child". If the parents are worried about the child, then there is something to be worried about...."listen to the parents". Dr.P has been calling us daily to check in because we are worried about our little man, so he knows to be worried too.

Today brings us new doctors, new perspective, new approach....the Mayo way- a team of doctors to look at things from every angle; not only to give best care but to make sure nothing is being missed or overlooked.

Our good news today? Our dietitian from CHOP was able to give our dietitian here the resources to show that Hemp milk is a nutritious milk alternative (not to be confused with a formula alternative); but a good base on which to build a good diet for an FPIES child with intolerances to dairy, soy, rice, and corn. Confirmation that I was in fact not malnourishing my child, his FPIES was.....

So quiet....

at home. Hubby takes the night shift with little man. After many months of having a sick child, while still caring for our other 3 boys; we have become a well-oiled (although admittedly sometimes squeaky) machine. And right now, I take the day shift with little man at the hospital so I can be there to talk with the doctors; and he takes the night shift so he can be there for Little Man. This allows all our boys to get time with each of us as well. Cuddle time, bedtime stories and bedtime prayers with mom right before bed are critical for the 3 big brothers. Playtime and cuddle time with daddy before bed are essential for little man. But we miss Little Man at home. He is anxious to get home too...hopefully tomorrow afternoon...

Little man has not had good weight gain yet. I already could have told you he wasn't going to. He drinks in adequate calories but his body isn't absorbing them all because of his gut inflammation, so this will continue to be a cyclic affect until we can rid his body of the inflammation. He started to have a rash on his back today- just another sign of the inflammatory triggers in his body. He also looks more pale today.

Our allergist at CHOP has been calling to check on him, this makes us feel very supported and is helping with a lot of the questions we/the doctors here have about FPIES. We have learned so much on this journey so far, I continue to look to learn more- not only to help our son (who still needs so much help right now) but to help learn and raise awareness of this roller coaster ride.

Threshold of tolerance...

Trace proteins in breastmilk were an issue from birth for little man, after introduction of formula, it became a lower threshold that I could eat before I saw symptoms in him (through my breastmilk). After introductions of solids, he began reacting to more through my breastmilk (looking back).

Little man does not tolerate trace proteins of dairy (without any doubts). He has also reacted to soy and rice. But the threshold appears to be higher for these as it was a month of supplemental soy bottles before we decided he had a soy intolerance. We still aren't sure about FPIES to soy but clearly was intolerant to it and at this point is too sick to trial it. Corn looks to be very similiar to dairy- reacting to trace proteins. Neocate has trace corn proteins, however it does appear to be the most broken down of the elementals(best strained if you will) corn proteins from the corn syrup solids.

We trialed corn. After corn fail, we saw less tolerance of his formula...and it was as if he can tell his body can't tolerate it because he started to self limit his intakes to less than 20oz.day. We moved to Hemp milk- doing a 50/50 mix and as we gradually moved further away from Neocate and more hemp- we saw these symptoms decline (only to come back full force if we move back up).

We asked our allergist at CHOP what he thought and he agrees it makes perfect sense. Finding that threshold can be tricky. We are going to attempt to find it. We have added back in 20oz of Neocate to his hemp milk. Upon adding it back in, he has had mouth itching, mottling of the skin, abd.;pain, reflux and pain from that, fussiness, burpy, and much disturbed sleep, and also mucous in his almost-too- formed-for-liquid-diet-diaper These have all been signs in the past of intolerance and all signs that decreased or went away competly as we lessened the Neocate. The doctors here at the hospital (who most are not currently recognizing his FPIES) want us to go to 100% Neocate and then see if he has intolerances. We are not here to make him sick and will not be doing this.

We had a long care conference yesterday to try and get our outpatient "team" and the inpatient team on the same page. It was intense and I really don't know if it made any difference. Our allergist at CHOP has been calling Sam's doctors and us daily to check in (despite being on vacation last week)-he has been a GREAT help and advocate for Sam. We will just have to see what the next days bring here, and then continue on with our regular "team" with finding Sam's baseline.

Mudslides

Our rollar coaster ride has hit a mudslide....the blood in his stool, then not eating well, then starting to refuse to eat, then a viral illness, everything started to look fuzzy through the haze of mud.....

Little man continues to run unexplained fevers and generally just feeling lousy- lots of crying like he hasn't done in a long time....it looks to be "just" some virus that he is fighting...day 5 of fevers today. So, we just have to keep a close eye on him.

He drank 70+oz of hemp milk today!!!!! But he was hungry all day....unfortunatly the hemp milk does not have enough carbohydrate and he does ok for a day or two but then it just caves on him and he needs the carb. I tried a few times adding the tapioca and cane sugar mix but he was refusing it, and gagging on it- and I can not have him associating this new formula with anything negative.

So tonight,after a very busy day, I started really trying to pinpoint where this mudslide started (other than the blood in stool from the neocate)- but that is where everything started to spiral and unfortunatly making it look like it was all connected. After much thought on it and careful review of my logs; I think it is the cane sugar he is not liking. He's reacted to things with sugar before, so maybe he has an aversion to sugar (or something more). Still not sure but for this instance, it seems that when I started experimenting with small amounts of adding cane sugar, things started to slide down this mudslide; that combined with his body fatigue from Neocate exposure after corn fail, and this virus- all just got to be too much for his tired little body.

I have added the plain tapioca starch back in this evening and he was almost immediatly a happier boy; so hopefully this works and I will stay away from the cane sugar for now -- will just have to figure out another way to get more carbs in him. His allergist at CHOP keeps suggesting a barley trial, but I am SOOO leary of grains right now!- I'm SO leary of ANYTHING that will have a chance of making him sick!!

But we do have to come up with some foods we think he will "pass" and be "safe" foods to expand his diet. I'm thinking potato is the better option for next trial- it is not a grain but it is a starch and would give him the carbs he needs, and so much more! Also, looking at Arrowroot starch. Need to get him healthier first though....

I keep searching for that little barometer that tells us that he is ready for a food trial, that his illness is or isn't related to food intolerance, or when a food is going towards an FPIES reaction.....still can't find it!!

Happy Father's Day!!

Ok, so I am running a little late on my Father's Day wishes! My husband is a great father and provider and teacher to our little guys, we will always be grateful for the extra time he gets to spend with them...being a Mr.Mom on his off-shift days! That is how he got to spend his Father's Day since I worked this weekend.

Samuel has been declining again, I'm assuming it is because of his reaction to corn 3days ago. But it reminds me of right before the hosptial stay- he is self limiting his formula and is now down to less than half of what he needs in a day to grow and gain weight. He simply does not even ask to eat and refuses when we try. He doesn't seem to be too bothered by it, or hungry....until tonight- he was particularly fussy most of the evening. So, I decided to give him some motrin to see if this would help. 4hours later, he emptied the contents of his stomach. Motrin, I had been starting to question his tolerance of -- clearly helps him with the pain but have been getting that gnawing feeling that there may be something in it that is still objecting to his system...now, I'm thinking I was right -- there must be corn in it. Dr.P at CHOP did say there is somewhere that compunds medications corn-free...we need to find out more about that because little man needs something to help him through the pain he experiences. If we adults had to experience that same pain- we would be asking for a morphine drip, I have no doubts.

This FPIES is a monster rollar coaster ride. One day you feel like you're prepared and ready for the challenges and the next it knocks you right over.

Faith, faith is a good thing to have...I have a strong faith and I do know without any doubts that God has given us our little man for a reason; and he has taught us so much already, and not just about FPIES but about instincts, patience, perserverance, love from brothers, devotioin of husbands (ie daddy), love and support of family and friends all around us, and about Faith.

Well, now he's up crying again....tickets please...

Our "Sam Syndrome": from birth to diagnosis

Our "Sam Syndrome": from birth to diagnosis

I am a mom of 4 boys. I’m not new to this mommy gig.
Maybe the early signs should’ve been red flags: bringing him home from the hospital and having blistering diaper rash, eczema that started in the first week of life, gassiness, and explosive diapers. But I’d been through all of that before, our first born had milk protein intolerance with allergic colitis at 8weeks old, colic from 3-16weeks old and reflux with throwing up until he was 19 monthes. We had had our share of crying, gassy days filled with projectile vomiting. So I wasn’t alarmed when I began to see all this again. I did see all of this in our 3rd son, but he was only milk protein sensitive. So, I thought I was going down sensitivity road again.

I was exclusively nursing, as I had with all my other boys. I knew what I had to do so I started changing my diet to find the right amount of dairy my little guy could tolerate, this seemed to help some.

He was only a few weeks old and the middle of the night wide awake times began…this one was new but again I wasn’t too alarmed- just considered myself lucky that I had had 3 good sleepers before him. Also started to notice, although we enjoyed the cuddle time, that the little guy liked to be held upright for naps- he would wake up out of a seemingly deep sleep if we laid him flat. So, we created a “nest” for him in his bassinet and crib – with a wedge and a broken down bouncy seat for soothing vibrations (that he seemed to respond well to) and this helped us get some sleep…some nights.

It was approaching time for me to return to work and so trialed him on some formula, for supplements as needed (unsure how my production would hold up). This brought on projectile vomiting from the first ounce. We chalked it up to weird coincidence and tried another ounce in a week or so, only to have more vomiting. Based on our past experiences, we recognized that he wasn’t going to do well with regular cow’s milk formula at this young age. My supply was holding up so we decided to just hold off on supplementing with formula at this time.

But now, at 3mo. old we started to see a colic behavior creeping in- each evening seemed to get worse. We were very busy so we thought he was just picking up on the stress in the house of me returning to work, school starting, etc. My husband works 24hr. shifts, so our 9yr.old steps up to be the "man" of the house and help mommy out when he is on shift. He would tried so hard to help out, it broke my heart and made me so proud all in one breath. It took his words a month or two later, saying “Mommy, what happened to him?” for me to start to really realize I wasn’t the only one noticing that his fussiness was getting worse.

I had started to change my diet around more and noticed immediately if I had more than ½ cup of dairy in a day, I would have a wide-awake, gas filled little baby at 2am. So, I limited my dairy- not having any straight dairy but reluctant to pull all sources out at this point- still not knowing if it was sensitivity or full intolerance and since we hadn't seen any bloody stools, I was convinced he had a sensitivity like son #3. I did notice keeping control of the dairy was helping some but some days it was back to the same, wondered if he was affected by other things (?wheat) in my diet- speculating that with my IBS, he was getting incompletely digested proteins through my breast milk.

We were approaching 4mo,so we decided to trial some rice cereal to see if the thickener of it would help with what we speculated may be some refluxing issues. This seemed to work for awhile, but that would be the course of things to come for the next few months...we were slowly losing the grasp on what was really affecting him and what was really working. We turned back to the suspicion that my production was low, so we decided to trial another formula- this time trying soy. Soy brought on gassiness, stomach cramps, reflux, burping, and a chest congestion that sounded so badly after 5weeks, I feared he had croup or pneumonia. After almost 2mo of sporadic 2oz.supplemental soy bottles, we decided he must be building up sensitivity to soy. We stopped soy. He also was not accepting new foods well, and beginning to refuse many meals.

Our little guy was going downhill fast, we needed to find something that would help take away all his pain as well as nourish his body. We moved on to trial Nutramagin, followed a 100% no soy/no dairy diet (for me and him) while I continued to nurse him, then Nutramagin AA, then a reflux medication. A week on the new medication and his stomach aches doubled and his appetite decreased. His body was not responding to any of our many efforts and he was getting so sick and his pain cries were so intense.

That instinct? Now burning flame in the pit of my stomach. Our little guy was experiencing pain day and night. It was getting more heart wrenching to see....stand by and not be able to do anything, or enough of anything to help him escape this now growing constant pain.

We received a GI consult with recommendations for a new reflux medication and an elemental formula (and to stop nursing). We tried the reflux medication right away but it made him so sick within the first doses and he started to refuse to eat, and we then ended up in the hospital where we got him eating the new elemental formula- Elecare, but this only brought on daily throwing up. We waited for his stomach to adjust to the new formula. We proceeded to an upper GI and then to a Endoscopy and sigmoidoscopy work up hoping to find out what was going on inside our little guy that was keeping him from tolerating the foods designed to nourish his body. The tests confirmed that he did not have Celiac Disease or Eosinophilic Esophagitis but something was still not right with our little boy. We switched formula’s from Elecare to Neocate (another elemental formula) and the daily throwing up slowed down. We tried Neocate Nutra and the violent vomiting began. We would watch as he emptied the contents of his stomach, he was so sick. Then we connected it to the Nutra, we stopped this and the violent vomiting stopped…unless he got a crumb from the floor dropped by his brothers having a snack. He remains so super sensitive to any ingestions of foods.

We knew something was still not right…months on an elemental diet we thought he was ready for re-introduction of foods. And where do you start reintroduction of food? Rice? This brought on days of vomiting and agitation. With this, we begin to make some connections in the literature: could it be FPIES? We went to the next food, something he enjoyed before we stopped food- sweet potatoes. This brought on many days of colic and runny stools and everything the same as rice, except the vomiting- all from just a few teaspoons of pureed sweet potatoes on one day. This was getting to be too much again. We had consulted with a RD (Registered Dietitian) and she brings up a possible connection with FPIES. Here I am coming across this FPIES again. We continued reading the literature and connecting the commonalities. Have we finally found something that could finally explain what has been ravaging our son's digestive tract?

Where would we get an expert opinion? I dove into the world of online support groups and found and connected with other mom’s who echo my son’s experiences, echo the food sensitivities and reactions to foods, echo the anxiety of having an ill child with a syndrome of unknowns….many have gone to this place called CHOP- what is CHOP? I soon find out….Children’s Hospital of Philadelphia, and they have a program for Eosinophilic Esophagitis and in this program they have become one of the leading experts on FPIES. FPIES is essentially Eosinophilic Esophagitis ruled out. And that is what Samuel had- something that, to his doctors here, looked and acted like Eosinophilic Esophagitis but scope had ruled this out.

We call and get their earliest appointment, and go to PA with much hope and anticipation for some direction on how to proceed. The experts at CHOP recognize the signs and symptoms almost immediately. And our son is diagnosed with FPIES, a mere 4days before his 1st birthday. Happy Birthday Little Man!! Now, we have an action plan, we have an Allergist, and we are pulling together our resources to help us care for our son as we journey down this new path.