Have you ever had an instinct? An instinct that begins as a gnawing...Then grows into a raging burn; a burning instinct that something is wrong...
Your baby continues to get sick from the very foods he is supposed to thrive on. I did. I am a mom of a little boy just diagnosed with FPIES.
And that burning feeling now? Extinguished. My instincts? Stronger than ever. Guiding me, with my faith, as we navigate through the murky waters of our new world created by something called FPIES.
"Faith is not about everything turning out OK; Faith is about being OK no matter how things turn out."
Sunday, July 31, 2011
I look back to notes from our last hospital stay. He was having issues with sleep at that time but otherwise doing ok. Once the PICC line was removed, he gradually started to sleep better. It took A LOT of work to retrain him for sleep. But also, the multivitamin (which had corn starch derived ingredients) fail set off his inflammatory reaction and I think we just kept seeing things cascade from that? His body doesn't seem to know how to shut off once it starts the inflammation. Is this an auto-immune component or an immature system?
Some kids take 5-7days to heal and ready for next food trials; while others- similar to little man- are taking 4-6 weeks before they are properly digesting their safe foods, gaining weight and reflux and such are not bothering them at night. So, for our little man, with the slowly healing inflammation, we just see compounding problems and then if he takes continued hits (accidental ingestion, accidental exposures, trials of banana, blueberry, elk, B vitamin, different probiotic strains....) it just continues to compound....
Will TPN reset us again? Is it necessary? What assurances would we have that we don't land here again? What would we do different after this TPN?
Saturday, July 30, 2011
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.
Friday, July 29, 2011
Our GI called to fill us in on the biospy results from his endoscopy procedure. The colonscopy results had already been back and were clear. We haven't even discussed further what the blood in his diapers is from. He had a diaper about a week prior with all blood- no stool, just blood. I consulted her about and she speculated it was a fissure she had visualized on a scope a few months back- but still puzzling why he is having so much trouble with fissures. Is it part of his FPIES? Part of just his body?
We already saw the pictures from the biopsy, so we knew the results would not be good...there is inflammation in his stomach and upper intestines. His esophagus looks great though (so what is the irritation in his stomach from?).
Well, now the final pathology report was in. The initial findings did confirm villous blunting (damage to his small intestine lining from the inflammation)
It is becoming more evident that Little Man is one that is prone to villous blunting, it is noted in the studies and research but we don't hear of it as being so problematic as it has for him. More than likely his restricted diet is a piece of that; another piece is the assault his intestines endured for months while we searched for a diagnosis. We feel his gut flora is really off set and that contributes a lot to the body not functioning properly. A vicious cycle...
There was some extra time taken to these sets of biopsy. First, because our GI had the path lab pull his old slides and contrast and compare, we got some answers with that. The next thing they were taking a close look at was aanalyzing the stomach inflammation. It appeared to be inflammation from an infectious source but all the tests were coming up negative for infections. They finally found something and it was a piece of wood fiber?!! Well, he does suck on wood a lot! But seriously?? Our GI then asked them to further analyze his intestinal biopsy's as well, deeper cuts into the layers- looking for auto-immune cells, auto-antibody cells, and other inflammatory markers, as well as eosinophils. The good news is that there is NONE of that! From this angle, he is healthy! Even the villi are strong underneath the surface! This is good news. It is more and more hopeful that we can get control of this beast of FPIES...narrowing down the mechanisms that it isn't from.
But it also tells us we need to look further into what may be causing it to be so complex in him. We just want a tiny menu- why can't we get any new foods, to the point that recently we started questioning if even his safe foods are ok??
We discuss next steps. TPN (parental nutrition- IV) again and/or steroids. Dr.J (research doctor in NJ) had recommended them as well but we weren't ready to try them with him- for fear of him reacting to, or having side effects, from them (the drug itself or the additives) and setting him back or potentially making him really, really sick. But, we're getting closer to just needing to try something like that. He isn't improving with all our other efforts. We need to start thinking about it. The GI discusses a few options: we could just try the steroids now and see if that heals him (without TPN being that his villi aren't as damaged as they were in Dec.), or we could do TPN for optimal nutrition, and then add the steroid with that (via IV). We are to think about it, knowing how hard this could be on him, how hard hospital stays are on us all; but how much he needs something to give.
I begin to think about it, no TPN? Really? Could it be this "easy"? But we do not know how he will tolerate the steroids, and he's already all inflamed and declining by the day? That is a big risk to take, when we know he would benefit from the IV nutrition and get back to a baseline of no intestinal inflammation- so that then the steroid can work on his immune system and not just healing the intestinal damage. But I also feel the probiotic will be crucial for his immune system as well. All 3 (TPN, steroid, probiotic), Dr.J has recommended to look at for Little man. Maybe this will be it? The time we get ahead of his dysregulated gut and immune system? We watch as he is going downhill and not getting better, we feel he would benefit from the TPN. We decide to give it some further thought, if we do TPN; we could also work through this new probiotic while also deciding on best time to trial the steroid. It's a lot to think about on my own.
Thursday, July 28, 2011
He recommends we try his version as his claims are that it is pure, powerful, and hypoallergenic. He wants us to start with his D-Lactate free line. He says Lactate poses an issue with protein intolerant kids whose bodies perceive things as toxins (need to research more on that as I'm intrigued). He instructed us to start with 1baby scoop (these scoops are TINY) and work up to 3-4 scoops/day. He stressed that only I will be the judge of how much he needs, I will need to monitor his symptoms and see when he needs to move up, or back down and at what level he will be therapeutic at. He said anytime you have an imbalance of flora, and you start playing with that flora- you will see side effects; he didn't call it die off but that is often what it is referred to.
I ordered the D.Lactate free, and he sent samples of L.Saliverus and L.acidophillus for him to trial once we work through the D.Lactate.
He had so much to say about how probiotics help the body function, he likened it to changing your environment from a cloudy day to sunny weather. He was helpful and pleasant and wanted me to keep in touch on Little mans progress. We'll see where this road takes us.
Wednesday, July 27, 2011
The family's story is something that hits close to home for many FPIES families...."I googled baby vomits rice cereal"- wow, did we all do that? Because who in the heck is allergic to rice? FPIES kids are, it's bizarre but very real, and scary. This mom's account of her daughter's reaction is an emotional account for any FPIES parent, as her daughter is one of the numbers of FPIES that went into full shock with the ingestion of rice. We are thankful, our little man has experienced shock symptoms but not to this degree. The statistics show that ~20% of FPIES children do (although I would dispute that number to be much higher based on what is shared in support groups). The interview is a window into an FPIES families world.
The news report includes an interview from the family physician who helps manage their daughters care, with emphasis on the need for ruling out other conditions, and the symptoms caused from an FPIES reaction.
Thank you to Ginny and her mom for a great awareness story on FPIES!!
My notes from the appt:
•He knows what FPIES is, and that it is a severe gut immune response.
•Would like to see Celiac and other auto-immune genetics testing done as this will help guide treatments (how susceptible are his genetics to these auto-immune and inflammatory conditions?)
•Possible for IgG testing (blood) in the future.
•Hyper-permeability of the intestines will result in reacting to proteins once previously tolerated- could be building up an intolerance to something in his regular diet.
Hyper-permeability can be from:
Nutrition (lack of nutrients)
•Would recommend a Nutral (?) test (urine and blood) – tests for nutritional Efficiency (vs.deficiency)- to see how well his body is utilizing the nutrients he is getting.
•Glad to see he is on a probiotic and tolerating it (acknowledges that you can react to a probiotic because of it being cultured on dairy if you are dairy intolerant), wants to look at other strains for him. He was intrigued that he is doing so well on sacchyromces strain. Would like to look at getting custom probiotics- strains made just for his profile as well as compounded just for his needs. Knows a PhD (the bug guy) that does custom probiotics to see if this can be done.
• Agrees with our GI that, right now, he absolutely needs TPN and that it presents with a unique and beneficial opportunity for working through the probiotic strains introduction. Was glad to hear he tolerates TPN well, from his history.
• IgG powder, feels he would benefit from this- we looked at the powder he carries and it looks “pure”, but he will get in contact with the guy who makes them as they are friends to be sure there are no corn (or dairy/soy) ingredients.
•Pancreas elastase stool test showed “robust” pancreatic enzymes but reports that only 10% of enzymes are put out by the pancreas, the rest are from the brush border- which he acknowledges he has none right now (with inflammation/permeability).Would like to find an enzyme product that he could tolerate, as this will be beneficial for helping his body to breakdown foods.
•Agrees 100% that we need to do things systematically and slowly, one thing at a time and monitor each step made.
•Acknowledges that there is no one thing to do that would be the “click” but that we can look in all these “corners” and find things that help piece it together for him to thrive through it.
•Above all, needs a collaborative effort- not just from other MD’s on his team but mainly from us as his parents. He will suggest things and I will need to know if it is something we’d like to try or not.
•Unfortunatly, it does come down to the parents managing a lot in these kinds of cases; we know our children. It is a lot for parents. Supportive doctors can help.
•Discussed his soy and corn reactions- he confirms Dr.J theory on the cytokine storm and relates that it is localized and not full organs (as it reports in literature). Avoid soy. Corn needs to be avoided in all derivatives, acknowledges this to be very difficult to do and echo’s Dr.P (allergist) in a “tiny menu”.
•Interested in being put in touch with Dr.J (research MD in NJ) shared those study results with him and he acknowledges that the results are not concrete and really don’t tell all but can be used as a tool (along with many other testings-stool, blood, urine, genetics, etc) to help guide treatment/next steps direction; especially when you hit bumps in the road.
•Feels my instincts on that Little Man's severity is directly linked to his gut flora is right on and that the focus needs to be on correcting his dysbiosis; and this will help correct his over-reactive responses to every.little.thing right now.
FULL day, lots to absorb and process, lots of my own research confirmed. A new doctor to add to our resources for Little Man's team. A team to keep him thriving through such a confusing diagnosis.
Tuesday, July 26, 2011
Our GI doctor is perplexed and will discuss things further with us when we get biopsy results back. I e-mailed Dr.J for her insights to which she replies how, from their studies, she is beginning to find how there are two sets of FPIES kids- ones who respond to the elemental/elimination diet and ones who do not, whose immune systems are different. This makes a lot of sense for what we see among FPIES kids- those that are typical and classic and those that are atypical and then a spectrum in between- which equates to a lot of variables in reactions, there must be just as many variables in treatments.
I have said for months that we need to do individualized treatment, think outside of the box, follow Little man’s signs and symptoms to find his path to healing. With the biopsy results still pending, we head to see an MD trained in Functional medicine, to explore this path to healing. The consult could not have come soon enough as clearly we need a different path to heal Little man and help him thrive.
Monday, July 25, 2011
But the main concern is his severe and persistent anemia. Dr.J (PCRCD) has speculated that he may have a macrophage activation causing his body to chew through his iron. It sounds like a logical place to investigate but our current hematologist isn't interested. The feeling is that he simply does not get enough iron in his diet, combined with chronic inflammation and bleeding- he has iron deficiency anemia. Period. No further work up needed or necessary. This simply doesn't add up to me, and my instinct tells me there is more going on. We need a new consult, we need them to look further; so we decide to proceed with the scopes (upper endoscopy and colonscopy) to rule out the GI involvement for the low iron and persistent anemia.
Our thoughts are that we need this to help further investigate the anemia, if we find inflammation or villous atrophy than we need to evaluate that and we need to consider that is the source of his anemia. But, on the flip side, if he doesn't have any inflammation, we can be cleared to do a food trial! Eggs?? I found corn/soy feed free chickens!
Little man has gone multiple scopes and each one teaches something more about his course of illness. I agonize over putting him under yet again, for yet another scope; but his FPIES mechanisms are so atypical and we are losing direction on what to next- because of the recurring anemia. We can't keep giving him blood transfusions!
We proceeded with the scope....
Tuesday, July 19, 2011
I began making a formula out of it this time last year, we had some bumps in the road, such as: we couldn't mix it with Neocate and find a threshold- we would need to move away from any and all traces of corn for true healing. We tried tapioca starch for added carbohydrate calories, and failed- miserably. This fail had FPIES symptoms but likely was also related to his dissacharide deficiency. That brought us to arrowroot starch, safflower oil added to the hemp milk -- which we saw great improvements on. But a blood draw revealed that it wasn't helping his pre-albumin levels (protein stores) improve (past the losses we saw with Neocate, likely from malabsorption due to inflammation brought on by the corn). We found Hemp protein powder, and began to add it in in August of last year and his protein stores improved nicely. Hemp provides a great nutritional profile for protein, nutrients and calories. It is a good source of iron, but a plant source- which is not as readily absorbed by the body as a meat source. With anemia in our cycle again (still), I have been looking again for more information on hemp nutrition; although I am not finding specific information to the bio-availability of iron, I am reminded of how nutritious, and functional, hemp is.
Taken from a company that markets Hemp, Nutiva:
"The hemp seed is the richest known source of polyunsaturated fats, containing a perfect 3:1 ratio of Omega-6 linolenic Acid and Omega-3 linolenic acid. In fact, the hemp seed balance of Omega 3 to 6 fats is the perfect proportions for human dietary needs, making hemp an ideal addition to a healthy diet and lifestyle.
The Omega fats, or “essential” fats in hemp seeds are crucial to human health and they are called essential because they can only be obtained through our diet. Without essential fats, the body is at an increased risk of developing deficiencies that can cause slow wound healing, hair loss, skin conditions, and a greater risk of infection from the common cold and flu. Imbalance of Omega fatty acids, including too many Omega-6′s, common in trans fats containing foods like fast food, animal products and processed junk food can lead to diseases including heart disease, hypertension, stroke, arthritis and diabetes.
The hemp seed is one of the best sources of nutritious plant protein. Hemp is also loaded with all 20 amino acids (including the 9 essential). Plant protein, such as hemp, is vital for vegans and vegetarians, and is also preferred by many world class athletes for its dense nutrition. The protein found in hemp hearts is very digestible, with no known allergies, which makes it an excellent choice for sensitive diets.
Hemp also contains phytonutrients, which may boost immunity, purify the skin, and strengthen connective tissue. Hemp seeds may help with the reduction of cholesterol and blood pressure, reduce inflammation, increase healing time, improve circulation, and boost overall immune function and performance"
And, the Nutrition facts label of the hemp protein powder Little Man consumes.
What a superfood!
Sunday, July 17, 2011
The title is deceiving but eye catching and eye catching is what we need. We need to continue to raise awareness, that from the outside looking in, despite how it looks from the outside- our children are sick and our families are struggling because of the lack of awareness and treatment plans.
Please take a moment to view the video, if you have not already watched it. Good Morning America did a good job in covering many of the facts that FPIES families share.
Friday, July 15, 2011
FPIES or 2? Again, my thoughts are put to pen by a fellow FPIES mom. These two little boys and our families share many commonalities and, as it seems, we have yet another. These words, her thoughts,in this blog post: FPIES or 2? ...."When do I worry and when do I let it go? When do I correct the behavior and when do I cuddle it? These are behaviors that are not normal for [him]. We saw a lot of these as a result of internal pain that he wasn’t able to express" hit so close to home.
It is hard for me to see little man acting out or being cranky without associating it with something from his FPIES. Something that does help me (other than being with him 24/7) is to keep logs/journal/notes- whatever you want to call it. I take note of things that I suspect may be out of the ordinary, even if it could just be 2yr.old tantrum. Then, I know if it starts to show in a pattern- I can look for that. It helps a little....when I have time to review the logs!
Little man has primarily a very mild natured, even tempered, take-a-lot-of-hits-before-he-loses his patience, kind of personality. A personality I knew was there as an infant...hidden underneath all the pain....I knew he was in there....this is the little boy that I waited to come back to us. And now, we have hit the temperament and independent, coupled with frustrations of two....and we walk a fine line of phases of 2 or stages of FPIES?
Thrown in for some extra added confusion in his "atypical FPIES" is the mix of Anemia...and we didn't catch it soon enough, will we next time? And why is he still struggling with such severe and persistent anemia? I have been polling other FPIES families among different support boards, and there does seem to be a commonality of anemia happening. Thankfully, most not as severe as little man's. There must be something connecting this commonality among FPIES kids as well. Dr.Jyonouchi, whom we consulted with at the Pediatric Center for Rare and Complex Diseases has a theory on it, and we're hoping to consult with a new hematologist about that soon. But, first, we need to rule out additional intestinal involvement- does he have villous atrophy or inflammation that would not allow his body to absorb the iron? Does he have active colitis, or something more involved in the colon that he is having acute blood loss? Or is the plant protein based iron in hemp not bio-available enough for absorption in his body? Many questions to answer and I feel like we're in a race to answer them before we land with needing transfusions again. We sit and try to assess current symptoms, are they related, are they developing into a pattern? Do they look the same as his previous patterns of inflammation/villous atrophy/severe and persistent anemia?
- His cheeks are still somewhat pink, he still has his tanline from vacation.
- His mood is all over the place, but he is two.
- His energy is up and down, mostly down. He wants to be held and carried more every day.
- His sleep is only a little disturbed, but when he does wake up he is MAD and it is difficult to console him back to sleep.
- His diapers have a "glossy" look to them but there is no blood detected (tested for 6days last week).
- He is gaining height but has not gained weight in over a month, or longer.
- His appetite is up and down, but we still manage to get in ~1100 calories/day, in a 24hr.period.
- All of this we could do the "wait and see" approach but that approach has never ended in good results in the past when things start adding up like this; but mostly we can not because of the severe and persistent anemia.
Wednesday, July 13, 2011
Monday, July 11, 2011
We have had to lead a fairly sheltered life due to Little Man's needs, although with 3 other active boys, it doesn't always feel like it! That is until we are in situations where we are not sheltered and and then I can't help but wonder what it looks like from the outside looking in....
Sunday, July 10, 2011
Little man had 2 IV iron infusions, but his hemoglobin barely moved and was still very low when we rechecked it. Iron stores remained low as well. Why is this happening? It is becoming more evident that he needs a full anemia work-up (re work-up) but not this week as we were planning on leaving town for a few days. A discussion with the GI and we know he needs more treatment, he is going to need another transfusion. His iron stores got too low again and his body simply can not make enough hemoglobin or make it fast enough with the stores he has. We decide it is best for him to get another blood transfusion. My heart sinks and I melt. I can't help it, I am crying....I ache for my little man. He has to endure so much. Is it something I missed? Something I did, something I allowed him to have that is making him decline again? I don't even know and can not think about it. No time for a full melt down, he needs my strength. And we need to get to the infusion center so they can begin the preparations for his transfusion....
I pack some bottles and some toys, blanket and off we go. We get to the Pediatric Infusion Therapy Center (PITC) at the hospital and Little man already is unhappy with where we are going. This has been too much- needle sticks, lab draws, IV infusions...too many this week already. They call for the IV team, and lab draw - "difficult stick" she tells the technician on the call. They arrive and get to work- assessing veins. Veins that are hiding and mad. Surprisingly to all, he is not as bruised as one would expect him to be, given his severe anemia (one of anemia tell-tale symptoms is bruising). The think they have one in his left arm...poke- nope, it jumped. Get out, try the right arm (little man is getting difficult to hold down), poke- nope, it deflated. The blood is thick and not coming. They need a lab draw before his transfusion to type and cross his blood, the line is not accessible. The lab RN is ready to "call it" (sometimes they will call the Peds trauma team to get a difficult stick). A short discussion on what I would like them to do. Is going home and pretending all of this isn't really happening an option? No, no it's not. I'm holding a tired, anemic, growing little boy who needs adequate hemoglobin to have ample oxygen being supplied to his brain. "What about his heels?" I ask, cringing at the thought. "Will you let us try a heel?" the RN asks kindly. "f you can use a heal for the transfusion, and can get a vein...then yes- please try", as I remove his socks and shoes. Immediately they found a viable vein. Poke, bingo. Now, a new problem is securing a line in a heel of a 2yr.old who wants this stuff "off?!". We manage to secure it and distract him, and then wait for a few hours while they type and cross and then prepare his blood. The transfusion takes 3hrs, it's ordered for 2hrs but even the technician that comes to run it acknowledges that might be fast for a little guy with his sensitivities. We did 4hrs. last fall and he tolerated it well and responded well to it so we decide on 3hrs....it's late in the afternoon already and it's been a long day. We're tired....and we're leaving the next day for vacation!