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."
Thursday, September 30, 2010
On Wednesday, we took Little Man to see a 2nd allergist. We love our first allergist but he is a few states away so we are looking for more local support and resources for our team here for Little Man's continued care of this chronic illness.
The drive was an hour and a half and we went as a family. Little man's brothers are getting used to doctor appointments being part of the normal.
This allergist was friendly and after reviewing his history, confirms that Little Man clearly has classic FPIES to some foods and not-so-typical reactions to others (tapioca). He speculated this is likely from the chronic inflammation and once this is healed, food introductions/tolerance should become more “FPIES normal”. He has not had patients that have no safe foods/no diet.
No foods are safe, all food can be a trigger- it is all about the trials. Avoid the common triggers for now, start with fruits and veggies and grains. He suggested wheat, quinoa, carrots, spinach. Meats may be more difficult to digest right now, agreed lamb may be good, also suggested pork. Emphasized these are only suggestions- it is really up to us what we would like to do. Agrees with Dr.P at CHOP that goal should be a tiny menu and hold for awhile with Sam being that he has multiple triggers.
Recommends small incremental doses (1oz/1tsp/day and building)- monitoring for signs of symptoms of intolerance. He does not see need for hospital trials for all food trials (we’d be in the hospital all the time) but emphasized/stressed to have a plan and head to the ER at the very first signs of reaction. Acknowledges that we clearly can recognize early signs and to not wait for the vomiting with Sam because he is too fragile with the anemia.
Reminded that he already has a diagnosis, and now a 2nd Allergist has confirmed it. Carry an ER letter and information on FPIES (provided 2 journal articles- that I already have, please can someone give me one I don't yet have?) and tell new doctors not familiar with Sam and FPIES that “This is his diagnosis, it is called FPIES. He has received this diagnosis from an Allergist; here is some information about it”. They don't need to diagnosis him, they need only to learn about his diagnosis to help him. He spelled out what I have been trying to do since his diagnosis.
He will send letters of FPIES explanation and ER letters to our "team" here doctors here and a copy to us to carry with at all times.
It was a beneficial visit and we will wait to see if it becomes fruitful. My hope is having a MD with familiarity with FPIES that our team here can/will work with will help everyone to learn....to help Little Man and to help the next little one's that come behind him with this illness.
All of this only reminds us that "Little Man isn't under the doctors care- he is under our care; doctors are there for advisement on his medical needs." (direct quote from Little man's daddy). We coordinate his care, we research his diagnosis, we monitor his baseline and labs and weights and compounding diagnosis', we decide what foods to trial and when. This allergist helped us to see that once again.
We went to Whole foods right after the appt. We picked up raspberries, blueberries and baby cucumbers. This morning, we tried raspberries - he would not even touch them, so I moved on to the blueberries. He played with them but wouldn't let one touch his lips. So, I got brave (thanks to the allergist) and cooked him some hemp milk/millet porridge- he LOVED it!! Lets hope it loves him....the only thing we can do is wait and see...so stay tuned (and pray)!!
Monday, September 27, 2010
Janice Joneja Vickerstaff, PhD, RD is an inspiration for me. I had the privilege of hearing her speak at a Nutritional Peds conference a few years ago, her discussion on allergy’s intrigued me…and actually inspired me to get my then 6yr.old tested for allergies since he had such bad eczema and asthma and this is when we found out he indeed had an uncontrolled low IgE egg allergy. I learned so much from the elimination diet and having to understand allergy at a 1st grade level so I could explain it to him- so he understood why he could not have eggs (because he also has equivicol (neither negative or positive IgE numbers to milk and wheat- hence the elimination diet). I bought one of her books on “Dealing with Food Allergies in Babies and Children” right away, and hungry for more information, I also bought another one of her Allergy books. I have also since bought Digestion, Diet, and Disease to learn more about gut health from her perspective (to learn more about FPIES and my own IBS). I find her books very easy to read and follow, and full of valid medical information; so have utilized it as a resource time and again.
I wanted to share a bit from her prelude first as she shares her story (as she did at the conference as well). She had her PhD in Immunology and was working in Immunology and in fact trained under the man who discovered the RAST blood testing for IgE allergies. And yet, when in the 1970’s she had a son, she became helpless when he started having many allergies and developed the side affects such as eczema, asthma, migraines, and even anaphalatic reactions that we not recognized at the time to be from food. She writes, “in those days, anaphylaxis was considered a medical emergency but the underlying cause- food allergy- was not part of the medical lexicon”. She goes on to say, Doctors were convinced his symptoms were from stress in the family and he needed a “parentectomy”. At 13yrs.old her son went on a vegan diet because he associated himself that meats and milk were making him “feel sick” (found out later he was indeed allergic to beef and pork). Still his doctors denied the existence of food allergy. She goes on to say she felt so powerless- being so knowledgeable and still unable to help her son, or to get the medical community to hear of her connections. It was in the 1990’s when she got her Registered Dietitian that she began managing food allergy in the clinical setting and has been doing so since with her own allergy clinic in Canada.
I called her clinic and inquired about FPIES research and if anything further was understood since the latest research article (she has a section on it in her book that pretty much sums up the articles- which she notes in her references and where many of my initial research stemmed from). She answered the phone herself and took the time to discuss with me. She explained that even with FPIES- it is all about food trials and doing it in small-small incremental doses would be the best/only way to know. She did advise that to develop oral tolerance- once you start seeing first signs of symptoms develop to go back to the dosage that was tolerated and stay there before trying to increase again. This is based on the T cell response of protection vs. allergy vs. oral tolerance and makes SO much sense. Although we do know that gets very tricky with FPIES when these kiddo’s react to even the smallest introduction of foods and even crumbs!
I’d like to explain more, from my understanding of her books. I will be using her words for much of it but not always directly quoting all of it- I merely want to provide an overview/synopsis/outline of the pertinent information to this “type” of allergy. This is just me, thinking out loud, so follow me if you are interested in my thought processes...
“Reasons that one child’s body responds to food by developing distressing symptoms and another uses the same food for comfort and nurture may be found in several factors”.
1. Child’s inherited genetic make up.
2. Circumstances within first food encounter
3. Microorganisms that live in child’s GI tract
4. Medications take by mouth, or exposed to (such as mothers milk)
5. Other factors research is only beginning to understand.
“Food sensitivity is unlike any other disease entity….any food is capable of triggering an allergic reaction to a child who has been sensitized to it, or lacks the systems required to process it adequately when it enters the body”.
What is the mechanism for food allergy? And how could this relate to FPIES?
When an allergen enters the body of a person at risk for allergy, an extremely complex series of events “set in motion that will finally result in the release of chemical (called inflammatory mediators) that act on body tissue to cause the symptoms of allergy. All immunological processes involve the various white blood cells (leukocytes), and different type of chemicals they produce”.
First step: the body recognizes the invading antigen. “An antigen is a protein within the cells of any living material that enters the body. When an antigen causes an immune response, it is an allergen. When the antigen enters the body, the white blood cells (lymphocytes) are activated. Lymphocytes are the first cells of the immune system that recognize and respond to anything foreign entering the body (sentinels of the immune system)”. There are two types of Lymphocytes in the blood: Tcells and Bcells. Tcells are the ultimate gatekeepers and controllers of the immune system. “Th (helper T cells) are responsible for identifying foreign materials that enter the body. Th cells initiate and direct the subsequent activities of the immune system if foreign material is deemed a threat…. T Cells exert control by means of messenger chemicals, cytokines”.
The response to cytokines is Th1 or Th2 and this response controls the way the body reacts. “When a pathogen (disease causing microorganism- virus, bacteria, other foreign material) enters, the immune system protects the body by a Th1 response (IgG). Cytokines are produced and stimulate the formation of antibodies of the IgG class which destroy the invading microorganisms by means of the complement cascade…symptoms such as fever, aching muscles, fatigue, malaise (much like the flu) are the result of the body’s response to cytokines and other inflammatory mediators produced during this battle between the immune system and foreign invader”. “The complement cascade is a group of over 20enzymatic proteins in the blood that act together in response to antigen and antibody to destroy foreign cells by splitting them apart (lysis); this process releases various chemical byproducts that act on opsonin’s, chemotoxins, and anaphlatoxins to help destroy a threat to the body and results in inflammation in various tissues.”
So, if FPIES is an IgG response, it is the Th1 response that is activated. It sees the food as a foreign substance – in the same way it would see and attack a virus or bacteria.
Th1 response protects the body from disease, and the IgG antibodies are responsible for the ultimate destruction of the invader. The Th2 response results in allergy, IgE antibody.
So, if Th1 is the first line of defense against IgE, FPIES is an overreactive Th1?
Some evidence that allergy is inherited… “the characteristic inherited is the potential to respond with a Th2 response when harmless foreign materials enter the body…. Some suggest Th2 is more primitive response than Th1 and as baby matures, the potential for immune system to respond with Th2 is gradually diminished. This decline, together with the maturation of the digestive tract (that provides a barrier between food molecules and immune cells) would explain why children outgrow their allergies”.
What is Oral Tolerance? “Oral tolerance is a term to indicate that although the immune system of the digestive tract can recognize that all the material we consume as food is completely foreign to our bodies, a tolerizing event has occurred that has taught the T cells that the foreign material is harmless and can safely enter the body”.
Something is askew in the FPIES child that the T cells perceive this as a harmful substance; they are taught to remember that it was originally perceived as a harmful substance; each subsequent ingestion has more T cell memory (army) to fight this perceived invader.
All food is foreign to the body (upon first ingestions)….what is it that allows food to apparently evade the barriers of the immune system in a normal child vs. an FPIES child that food is perceived as invaders to the immune system? Is it an overactive Immune system? This would be an auto-immune disorder?
Immune System and Digestive Tract
The Immune system of the GI is different than other parts of the body, it is called the GALT- Gut Associated Lymphoid Tissue. “Processing of food through this system allows the uptake of nutrients through the digestive tract walls without triggering the protective response that would otherwise form a barrier to the foreign materials in food. At the same time- any virus or bacteria taken in through the digestive tract are effectively excluded by the GALT. We have a system that can- at the same time and in the same place- recognize and differentiate foreign materials that is safe (food) and foreign material that is a threat (microorganism, toxins….).”
So, is FPIES a breakdown in the GALT? A deficiency mechanism, or an over reactive one? My little man is rarely ever ill, he is only ill when reacting to a food- does that mean his defenses are down because they are attacking the food so it lets the virus in?
“But that isn’t the whole story, the GALT must also distinguish between invading microorganisms and other permanent residents of the large intestine- gut microflora”. And many moms have suspected/known their little one’s microflora is “off”. “Microorganisms in the large intestine defend the bowel from invasion by harmful microorganisms by competing with them for space and nutrients; also keeping surrounding tissue healthy by stimulating the GALT in a positive manner”.
Process of Tolerance:
“The process of oral tolerance involves the T cells and their “messenger chemicals” cytokines. T cells that first encounter the foreign food when it enters the very first time (breast milk or solid foods) are of the Th1 type (Th3 immunological protection). Food molecules are picked up by special cells in the infants digestive tract. The antigenic parts of the food (proteins) are then “presented” to the T cells where they couple of special receptor molecules on the T cell surface”.
“T cells with attached food molecules are then transported on the lymphatic system to the thymus gland. There, the regulatory T cells (Treg) stop any further action on the part of the Th1 cells when it is discovered that the “foreign molecules” pose no threat to the body. This process of inhibiting T cell action is carried out by cytokines especially TGFβ(Transforming Growth Factor-Beta) and possibly IL-10 (Interlukein 10).”
Is there a deficiency in the thymus gland production or in the cytokines (transforming growth factor, or IL-10) that inhibits this regulation to happen in the FPIES child?
“The ‘educated’ T cells are then transported in the blood circulatory system and back into the digestive tract GALT as “memory cells”. Is this where the reactions become full body? Eczema, itching, thrombocytosis, angioedema, etc. leading up to a trigger reaction, as the T cells make their way through the body and back to the GALT and now have memory cells to attack with future ingestion's (the delayed-“build” response some of us see)?
FPIES is a Th1 response- the body sees food proteins the same as disease causing microorganisms ; there is a skew in FPIES kids bodies T cell response- Th1 is OVER REACTIVE!
IgE allergy = B cell lymphocytes= Th2 response =produce IgE antibodies
Non IgE = T cell= over reactive and does not proceed to Th2 response (thus preventing IgE food allergy- actually protecting the body- as it does for viruses/bacteria).
FPIES is a skew/”mis-fire” of the Tcell action….the food proteins should be going through the Th1 as “foreign but safe” and to the Th2 responses and either producing antibodies (IgE) or continue on as safe (already passing Th1 gate keepers). But with FPIES, the food proteins go through the Th1 response and get picked up there as foreign and perceived threats; the immune response is not initiated because it is begun in the non-immune Th1 response- it is attacked before it gets to the Th2!
(I had a graph here but can't figure out how to insert it in)
-White blood cells aid the immune system in recognizing foreign proteins.
-T helper cells produce characteristic cytokines.
-Specific cytokines determine response – Th1 protection vs. Th2 Allergy vs. Th3 Oral tolerance
Food = white blood cells recognition =Lymphocyte = T cells= Th cells= cytokines= protection vs. allergy. FPIES is a disruption in the Th1/Th2 response.
Antibodies in food allergy
IgE= recognized typical food allergy response, but there are other antibodies in food sensitivity. IgG is being recognized in Celiac gluten sensitive individuals. IgG is also thought to be what FPIES response is related to, although isolating the specific levels with other factors has not been achieved for FPIES. IgG is not yet a reliable test for food allergy.
“IgG antibody can be found circulating in the blood of people who have no signs or history of adverse reaction to foods. In fact, some think that an increase in anti-food IgG in some cases may be indicative of successful resolution of IgE mediated allergy. IgG mediated allergy remains complicated because of the nature of the antibody and the immunological reactions associated with it”.
Present knowledge (IgG):
-4 classes: IgG1, IgG2, IgG3, IgG4
-IgG4 subclass high affinity for food antigens
-IgG4 may be associated with milk allergy, in particular milk protein B-lactoglobin in atopic dermatitis (eczema) in children.
-Some evidence that total (all 4 classes measured together) might represent some protection from IgE
(again showing that a good IgG response is actually protective for IgE allergy)
-Food allergy in infants frequently associated with increased gut permeability “leaky gut”
"Antigenic food molecules passing into circulation trigger production of anti-food IgG. Thus, in cases of IgE-mediated food allergy that results in inflammatory reactions within the GI tract causing non-intact digestive epithelium, it is logical to expect to find higher than normal levels of anti-food IgG. Some think that these anti-food IgG antibodies represent a protective mechanism, rather than a source of allergic pathway”.
Wow! So FPIES is actually the body protecting itself from developing IgE allergy- is this because of the leaky gut already present or is it causing the leaky gut?
Conditions that predispose to allergy:
1. Immaturity of Infants Immune System – Immune system elements are in place at birth but do not function at levels to provide adequate protection against all infection. Antibodies are a fraction of an adults and secretory IgA (sIgA) the first line of defense of mucous membranes is absent at birth. Babies can have impaired or delayed maturing of various functions including cytokine production.
2. Permeability of digestive tract “leaky gut” – Infants intestines are highly permeable particularly 6-12mo.(become mature by 3yrs) and can absorb large molecules of foods that trigger the Th2 response.
3. Breast feeding – “breast milk provides the ideal nutritional, immunological, and physiologic nourishment for all newborns. Components of human milk enhance the baby’s natural defense and promote maturation of the immune system”. There is data that indicates the breast milk of atopic (allergic)mothers differs immunologically from that of non-allergic mothers. Atopic mothers tend to have higher levels of the cytokines and chemokines associated with allergy in their breastmilk. In addition, they tend to have a lower level of cytokines known as transforming growth factor (TGF-B) that promotes tolerances of food components in the intestinal immune response.
This is the end of my "digestion" of Dealing with Food Allergies in Babies and Children" by Janice Vickerstaff Joneja, PhD, RD. I have gone back to her books time and again- pre-FPIES diagnosis for Little Man, pre-little man for my own knowledge of allergy nutrition and pre-little man when his older brother developed an IgE allergy. I always learn something new. I wanted to re-read the sections explaining the immune system and body response to food allergy/antigen - now with "FPIES eyes". I have been meaning to do this since Little Man's diagnosis. I now want to re-read sections of her other book "Digestion, Diet, Disease" to continue to learn about gut health and how it may correlate to Little Man's FPIES.
I know I can't "cure" him but I need to try and heal him. Little man is wanting food again, begging for food- his gut is healing from his recent reaction 2 weeks ago and his body is sending him the signals to find food to eat. He wants food, but what do I give him? What could be safe for him to eat, snack, chew, consume without causing pain? I want to give him something SO badly when he gets like this....it goes against every grain of my being to not give him something, I cry inside and smile on the outside....and try to stay out of the kitchen....
Sunday, September 26, 2010
Thanks to another FPIES mom, I got the idea to roast Little Man's hemp seeds. First, I made him hemp butter the other day- from her recipe creation! It is very nutty tasting and a bit strong by itself but someday he will have an arrowroot biscuit to put it on. Until then, I roasted the seeds for him to snack on - I think he knew they were his! He was so excited to try them, they had barely come out of the oven and he was asking to sample them! He loves this kind of textures- I am really hoping this means we can still avoid real oral aversions but we need to get going on food he can actually eat (and not just primarily play with). But at least, with this kind of snack- he can sit at the table and be "normal". "Eat" he says....
Wednesday, September 22, 2010
Little Man needs Vit.C, his hemp milk does not have any- nothing in his diet supplies him with the Vit.C he needs...scurvy anyone? Well, his daddy was a sailor but lets not go there.
We have compounded iron- FeSo4 with Calcium carbonate in a capsule we break open and put with a teeny-tiny amount of water, mix, and syringe it in! He does NOT like it!! But he needs it. He gets it twice a day, so that his body can begin to rebuild his lost supply. His body has adjusted and we are not seeing tummy aches from it now. So, moving on. Iron is better utilized and absorbed in the body if it has Vit.C to go hand in hand with it. So, having a vegetarian diet with just barely enough non-heme iron and no Vit.C to bind it for absorption + bloody stools = anemia. Vit.C made from tapioca = reaction. The pharmacist said Vit.C is made from 1 of 3 things: Corn (um-no, major trigger here and we are FINALLY moved away from ANYTHING corn), Yucca root (ie- tapioca, we now have tapioca as an allergy because of the way he has reacted to it the 3 times we have tried to introduce it- each time getting more and more sensitive to less and less amounts; and finally it can be made from beet sugar. Ah, the smell of sugar beets processing in the plants brings back thoughts of childhood. No, we have not trialed beet root so we are unsure if he could tolerate this either. Why not trial an actual fruit instead? Orange comes to mind, tangerine looked more tempting.
This morning, little man got his first tastes of tangerine. Yea!!!!! He loved it- well, the pieces he would eat anyhow. I am getting more worried about his oral aversion stuff....he was excited at first and he let me put a segment in his mouth (the size of a pea) and chewed it and swallowed it well! He would not however let me give him juice from a cup, or a syringe or a spoon- so segments it was. He played with them for awhile, "pretending" to eat them but not really eating, but he did manage to put another 2 pea size pieces in his mouth along with letting me put in 1 more. It took a lot of "cheer leading" and coaxing though!!
We had some weird behavior for the afternoon but hopeful it was from sucking on a piece of chalk (yes, I am having trouble throwing out all arts and crafts supplies that his brothers still enjoy- keep fooling myself that I can "control" the situation when they come out). He didn't' get much but it may have been what caused his afternoon fussiness. Also, we are battling a cold bug that is making ME icky-achy so I imagine he is feeling similar. It lasted about 5hrs., so yet another day where I get nothing done except holding a 15mo. old little boy who isn't so light anymore! A little bit before his brothers came home from school, he started playing nicely and he has been one.content.little.boy- all evening!!
Wish us luck as we press on to give little man some Vit.C and hopefully aid his body in absorbing his iron so he can kick this anemia!!
Sunday, September 19, 2010
So, we're getting our ducky's in a row....he will have his iron and hemoglobin/hematocrit checked again tomorrow. And our pediatrician asked them to add another, more specific monitoring test to the lab work (reticulocyte test?). He seems to be improving daily so we're hoping the numbers match. His daddy and I have talked about slowing down the blood draws too though, as that can't be good for him either (to lose a vial of blood every week!)...I feel as long as he remains asymptomatic - we can let him be the guide. He always has been in the past, when we see a change in his demeanor, doctors order blood tests and then begin to "believe" us that we are already saying he is sick. Dr.P, our Allergist at CHOP doesn't think lab tests are necessarily necessary - "listen to the parents"..."the symptoms tell the story". He has an appt with the GI doctor on Tuesday so we'll discuss that with her then as well.
He has been doing well, feeling better since I added the Hemp protein powder back in to his formula. I was so nervous about the possibility of a build reaction that I had taken it out last Friday, I look back now and think that may have impacted his slide downhill so fast with the reaction. He likes his formula the way it tastes now, it is just shy of 20calories/oz so he still needs to drink 50oz./day (and he does).
He had a tiny slip on Friday/Saturday. He was playing in the playroom and I looked over at him sucking on a wooden play screw from his toolbox set- except it looked like it had something on it. In his other hand was a bottle of sunscreen. Stupid free sunscreen attached to the package of little swimmers we bought earlier this summer- how did that end up in the playroom? How did it not end up in the garbage? I rarely even use sunscreen! Ugh!! I grabbed it from him but not before it was too late- he had already sucked some down. A quick look at the ingredients...of course, soy (tocophyrols) and also mango and coconut extract. Sure enough, within the hour he was itching his mouth and agitated. He had a few rough spots throughout the afternoon of tummy aches and Saturday morning had mucous in his diaper. Ugh- I hope it didn't do too much "damage" and cause him to malabsorb too much. He seems better now today, so hopefully it is already passed.
He is getting 15mg of elemental iron 2times/day for supplemental doses to build back up his iron stores. The pharmacy compounded it for him and we break up a capsule and mix it with water (as little as possible) and give it to him via syringe. It looks, smells and tastes like rust...poor little man. It is giving him stomach aches. Our pediatrician wanted us to build it up to the twice daily so until today he was getting one/day- tonight I gave him the second daily dose for the first time. He threw up. It was not projectile or violent, it didn't even smell. I tried to give it to him on a full stomach- maybe it was too full. I hope that is all it was. He seemed pretty tired after throwing up but it was bedtime. I hope that is all it was (did I say that already?) Yes, I did. When Little man is reacting- small or big, I worry- my anxiety builds. It is a bit of a post-traumatic stress kind of anxiety....most especially when I don't know what it is from, or when/if it is going to stop. With this kind of symptoms- we can only watch and wait and see. See if it builds, see if it happens again or if it was just random, see if he sleeps well tonight.
We just take it day by day. It's all we can do.
Thursday, September 16, 2010
Little man, happy to be home from the hospital. He misses his brothers too much when he is there; and they miss him.
I haven't updated in a few days because we've been so busy, and worried, worried and busy- but mostly worried.
We've been working towards a baseline with Little Man, we moved away from Neocate and began to see what he looks/acts like when not in pain all day and all night long. Then, we tweaked his 'new' formula (the one I make in my kitchen with hemp milk and arrowroot starch) by adding hemp protein powder and we found not only a pain free little boy but a content little boy. The end of August, he was doing so well. The past few weeks he started to slide- he was cutting his 1yr molars so was a little fussy/whiny -but no where near the magnitude of his GI pain, so we were dealing with it day by day like any other "normal" teething kid. Then, he caught a cold his brothers were passing around- which brought him down a bit. Of course, all of this made me nervous- is this a build reaction or just "normal" 15mo. old everyday things? Gah! How crazy to be nervous over such little benign things! We pressed on, he continued to get millet puffs daily- he enjoys playing with them more than anything and we have intentions to do a full trial soon. I added the protein powder. Then we find out his hemoglobin/hemotacrit is falling- and his iron stores are already low. His iron has always been low- we (me) assume it is part of his malabsorption he had for so many months while on foods that were triggering his inflammation response. The hemp milk does not have ideal amounts of iron in it, and the iron it does is non-heme (meaning not from blood- so vegetarian). I have a 15mo. old on a vegetarian diet. He needs double the iron and good sources of Vit.C- both of which he does not get.
Walgreens was so helpful in compounding his pain medications, now it was time to move the possibility of a vitamin supplement to the top of the list. I had asked them over a month ago and they could not find a way to make a multivitamin compounded with the vitamins but none of his allergens. I spoke to them again, and we decided to just to an iron and vit.C supplement- both of which come in powder form so they knew they could compound this. We waited a week, their supplier was out of FeSo4 (elemental iron); they kindly suggested we try another pharmacy in town. We took it to the hospital pharmacy, it took a few days for them to order the FeSo4 and assemble the compound and then a day to let it "set" and finally, last Wednesday it was ready. I gave little man his first dose as soon as we got home from picking it up. I thought he had good energy right away, but then he had trouble sleeping that night. I attributed it to an off schedule day and more teething. The next day, I had to work so little man was home with his daddy- who called mid day to say he was on his 3rd nap for the day. Odd, could this be his body's way of resting and breathing. One could hope, right? Thursday night he had more disturbed sleep and Friday morning brought the mucous filled, reaction smelled, diarrhea diaper. "He's reacting!" Little man's daddy added, "he had that same diaper yesterday afternoon". Ugh! What? What is he reacting to? The iron supplement? Iron is constipating, why is my little man having diarrhea with mucous? Ugh! Is it the Vit.C? I had called right away and asked them to be sure it was corn free. "It's corn free" they assured me. I didn't think to ask what it was if it wasn't' corn....I will next time. We called Friday to ask them to check what the Vit.C WAS made from, they got back later that day -- tapioca!! Tapioca that he had such violent dumping diarrhea that landed us in the hospital in July? Tapioca, really? Who would have thought?! Or, is it build from millet, or is it build from the protein powder that has Vit.E derived from sunflower oil. Can little man tolerate this? Everything extra, everything we weren't 100% sure on was stopped. No more millet puffs, no more protein powder in his formula, no more Iron/Vit.C supplement. He remained sick all day Friday, we remained nervous and concerned- would he get more sick? Can his body really keep handling this? Saturday he ate less and was tired and cranky, I sent a stool sample that came back positive for blood. Ugh, again. Sunday he was so, so sick- but what do we do? The ER wouldn't' recognize what was going on, their recommendations would probably be something that his body is sensitive to. So, again- we wait it out...moment by moment with him, holding him and watching him closely. Monday morning we call the doctors. The GI doctor wanted his labs done right away, they came back by noon and revealed a very low hemoglobin/hematocrit....he needs to be admitted.
Monday was our 13yr. wedding anniversary. What a way to celebrate, a trip to the ER for a hospital admission with a sick, sick little boy. But,these are the times I am so very grateful for my husbands strength. He was at work but he came home immediately and took Little Man to the ER while I stayed home and made some more formula and packed (hey, at least we got to be together for our anniversary...sort of). Thankfully, by the time we get to the ER- Little Man had a good nap and was perked up. They agreed he could wait until he was admitted for a possible transfusion- but that he needed continued monitoring for signs/symptoms of anemia.
So, monitoring is what we did- while the doctors checked all differentials for his sudden decline in hemoglobin/hematocrit. Little man kindly made us a stool sample the next morning and then blood work was done. Blood work revealed his H/H are stable- so confirming that his body was coping and holding; his chronic anemia has enabled his body to know how to utilize his stores well enough to hold. His stool sample came back negative for blood, the intestinal bleeding has stopped. So, the signs of him having more energy and more smiles and less crankiness with these lab results tell us he may not need the transfusion just yet. We wait to hear the doctors recommendations, while we try to think of the best decision for little man's health. "He will feel better" is the benefit they tell me. This doesn't seem like the benefit outweighs the risk. Do I want him to feel better? Of course!! That pulls at my heart strings like nothing else!! But do I worry that he will have a transfusion reaction? Do I worry even more that someone else's red blood cells in his body will incite his inflammatory responses? YES! Little man's daddy and I talked it over, and we just couldn't justify it. We know that if he drops low again, becomes lethargic and "blah", and a lab draw would reveal a drop to 6 or below- we would have to strongly consider this transfusion, esp if he was containing to lose blood (intestinal). But we are not there yet and Little Man deserves the chance to let the supplemental iron build in his body. Upon that decision, after 24hrs of monitoring to confirm his body is coping ok, Little man was discharged and we headed home. A discussion with our pediatrician today, and a plan is made for his specific needs. We will have to monitor him closely, and food trials are certainly on hold. I have added the protein powder back in, and he has responded well to it. I think not having this extra protein boost (also has iron) for his body did make a significant difference in how his body coped over the weekend. We will keep millet out and we have added tapioca to his list of allergies on his chart. We are hopeful we have identified the "trigger" correctly and he will continue to improve every day back to his baseline (that he had for such a short time).
He is so sensitive, so fragile but at the same time so resilient, so amazing. And he has big shoes to fill.
Monday, September 13, 2010
Little man is back in the hospital. We hope it will be a short stay but his hemoglobin/hemotacrit have dropped to low. He may need a transfusion. For tonight, they are holding and monitoring and will decide more in the morning. He has had a low iron ever since we started checking it but recently dipped too low, and now has dipped below ok.
Not much time for a long update. Will update more tomorrow when I have a computer.
Friday, September 10, 2010
GI appt was on Wednesday. GI doctor agreed with us that we were ready for a food trial, we've already discussed first foods to be in-hosptial trials. Little mans' reactions have been long-drawn out and make him very,very chronically ill for weeks. The doctors want to do what they can to learn about what a food pass or food fail looks like and learn what they can do to help his recovery. They will monitor labs, symptoms, diapers, intakes, and weights, and anything else that comes up.
Wednesday was a busy long day. 2nd day of school for 2 older boys and Little Man's appt in the middle of the day. We finally got his compounded Iron supplement, that we had compounded with Vit.C as well. Vit.C will help his body absorb the iron (in the supplement and in the hemp milk). I was so excited to give it to him, I got home and immediately mixed it up for him- a small syringe full of rust went into this mouth....and then I realized I didn't get clarification on the Vit.C origins!! I called the pharmacy and they assured me it was corn free. I breathed relief. My baby boy was going to get the iron his body so needed. He spent a lot of time napping that afternoon and evening but thought it was from the long and busy day. I worked the Thursday (the next day) but gave him his iron supplement before I went to work. A call from hubby later that day revealed Little Man had already taken a few short naps. One could hope this means his body is just getting the rest it needs, but my radar went up- what is going on with that? I get home from work and he is whiny but he's been whiny- he's been cutting his bottom 1yr molars so have had to write off his whining to this. But could it be from the Iron/Vit.C or is it from the protein powder that has sunflower seed derived Vit.E? Adding anything....Any.Little.Thing. to his diet always has to be a "trial". He's had the protein powder in large enough quantities- we would think we'd have seen more build symptoms by now but I will worry about this for another few weeks probably. Maybe I should just trial sunflowers to be sure?
Last night, Little man went to bed but was awake screaming twice before I even fully went to sleep. Thankfully, little man's daddy wasn't working today so he was up with him rocking and comforting him. After those two times, he wasn't awake again until morning so again- tried to think it was "just" teething or random awakenings. That is until I opened his bedroom door that morning to his cries in his room....and that smell is un-mistakable, the rotten smell of what we now coin as "reaction poop". Mucous, slimy, liquid full diaper reveals he is reacting to something. Is it the Vit.C? Did the pharmacy check close enough to be sure,sure,sure it is not from corn? Is it a build from the sunflower?
I called the pharmacy, they did some checking and the Vit.C (ascorbic acid) they used indeed does not come from corn but we were surprised to find out it does come from tapioca! Tapioca? I had read that as a possible source but I thought, what are the odds? Well, had he not had such a violent dumping diarrhea reaction from tapioca (what led up to landing him in the hospital last month). We still aren't sure tapioca was a FPIES "trigger" (no violent vomiting) but either way, his body did not tolerate it- and caused him to be very,very,very sick. So, tapioca in his Vit.C/Iron supplement would not be ideal for him. I am not sure where he will get Vit.C from, will need to find a fruit he can tolerate shortly after lamb. Ugh- sure wish I could just feed my son food....
So, we have postponed his hospital lamb trial- pushed it back a few days and if we don't see resolution of diaper symptoms (or if he has weight loss or blood in stools)- we'll push it back further. It is a BIG disappointment of a day....the emotions become difficult to contain when a food is failing my son; when a supplement made specifically for him that his little body needs fails as well, the load gets heavy to carry. How much can one little boy take? We were so ready for some things to start/continue going well. There are other plans...everything happens for a reason.
Stay tuned for food trial news...
How can you feel empowered in any sense when everything normal you know is ripped out from underneath you from something as simple as feeding your baby the simplest things: rice, oats, formula, soy (in everything!), corn (again, in everything!), the list grows and grows and all triggers are unknown -- until you feed them to your child.
FPIES reactions vary in severity, just as a typical food allergy can vary in severity. In a typical food allergy (that most are more familiar with) - one child allergic to peanuts has to avoid foods that contain peanuts because it causes hives, or some mouth itching; another child goes into anaphylatic shock from any exposure of peanut residue. FPIES allergy is the same. One child can go into vomiting/diarrhea/hypotensive induced shock hours after minimal ingestion, and another will cascade down a myriad of symptoms for days before the body gives a full rejection (violent vomiting and shock symptoms) of the offending protein. With FPIES, there is yet to be a way of knowing what foods will cause this reaction. You have to feed your child food that may end up being poison in their bodies. You have to make your child sick to find food for them to eat and sustain on. How does such an awful thing even exist? The underlying stress this puts on a parents shoulders should never be underestimated. The roller coaster ride of emotions can be quite overwhelming. And yet, most of us are walking around without adequate support when we need it most....when our little one's are sick.
FPIES triggers are everywhere. Kids put everything in their mouths, so not even bath soap is safe. Paper has soy and corn. Crayons, paints and crafts have wheat, soy, corn. Baby food is contaminated with dairy, soy, rice. Medications have soy, dairy, corn, additives, colors, in them. Your child is in pain and you can't even give him a medication to take away that pain, you can't do anything to help with the pain but wait for it to pass. Your only consolation? They are too little to even understand, they are also too little to know the difference. What kind of a consolation is that? Think of when you had a stomach flu, or a hangover, or food poisoning, or if you know anyone who has Crohn's Disease or Ulcerative Colitis- ask them how much pain they go through. I imagine these things are close to how it must feel for an FPIES child. But, what can a parent do about it?
What do you do when your child is sick and their symptoms puzzle you or you don't know how to make them better? You take them to the doctor. What do you do when your FPIES child is sick and the doctors don't understand it? You are forced to deal with it on your own. Looking for support from the medical community that knows very little about this rare illness and not finding it, only makes the load heavier. You carry this load every day; some days it is heavier than others (days of reacting, days recovering from reactions, ER visits, doctors offices where you are not heard); but you keep on carrying the heavy-heavy load. I accept my load, he is my child. I am not asking for anyone to take away his FPIES.....just asking for a little bit of support.
We, the FPIES moms in the trenches can continue to make a difference. Continue to work on being heard and raising awareness. if not for our children- for the children following us.
More research is needed, better protocols for how to even begin to help these family's. Help doesn't come in the way of a "cure", many FPIES kids outgrow this by the time they are 4-5yrs old, we are fortunate enough that our children have a diagnosis that has (in the words of another FPIES mommy)"a light at the end of the tunnel, it's just a really long tunnel!". We know (we hold on the hope) our children will not have this forever and we know one day we can look back on it under a different light but that doesn't take away from how tough it is RIGHT now. Help comes in the form of symptom relief and support for the family behind the FPIES child.
Support is one of the biggest "treatements" of this beast diagnosis. And yet, so many of us struggle to find it. This is a chronic illness of a baby/a young child. Their needs are 100% on the parents, a simple "how are you doing" in the beginning of a appointment would go a million miles for me. A reassurance that even though my child doesn't have a test that can 'read' his symptoms- that someone believes and trusts me because they are listening and care about my child, that even though there is little know about this diagnosis- they are willing to learn; to learn so they can help the next time he is sick.
Support is needed, for the family of a child with a chronic illness, and to make sure the family is being HEARD. Coordinated care is essential. Coordinated care -between GI, Allergy, Nutrition, and Pediatrician. Also coordinated care between in-patient and out-patient doctors because we all know hospitalization happens for these kiddos.
Bottom line to how to empower a parent with a child with FPIES? A willingness to learn and to listen to the parents.
Monday, September 6, 2010
Little man is enjoying his "new" quality of life - ie a baseline. He still has a chronic illness, that hasn't changed. We still have to be very viligent about his diet, his growth, his illness. But, for now- we can smell the roses a bit.
Sunday, September 5, 2010
I have 4 brothers and 2 sisters. They mean the world to me, and I know they would move mountains for me if I needed them to. My sister's have been invaluable to me through this last year. My youngest sister A.(almost 14yrs separate us!) came to live with us this winter/spring when things were so very rough with Little Man that I was barely keeping up. The morning she arrived, she did more in 3hrs. than I had been able to do in 3 weeks- laundry, dishes, games with the big boys. Taking care of little man had become a full time job. Right before his first hospital admission, his daddy and I had both been calling in sick to work because it took both of us to care for the boys throughout the day because little man was so sick and in constant pain. A. stayed with us for 3mo. and it was a gift. My other sister K. has been available, almost at the drop of a hat....she lives closer and visits often (never often enough though!) ;)
It is our hope that our boys always have the strong bonds they are forming now and carry with them, for each other, for life. Our eldest struggled with Little Man crying episodes, when the "colic" started at 2 1/2mo. old - he wanted to help so badly; he is a responsible leader. Our second son cringes at the thought of Little Man getting his blood drawn, an IV placed, even a blood pressure check makes him hurt for him- he has a sense for other people like no other, he is a protector. Our third son is too young to fully understand what is going on, and has started feeling the pangs of jealousy as this journey goes on and on and on; but he has been so patient- so loving.
Their bonds will be stronger than they would have been had Little Man not gotten so sick.
With Little Man's illness, we have had to strengthen from deep within, a strength that could only come from Above - to perservere through the many days of crying and crying with nothing to do, nothing to stop his pain, no direction to go in, trapped. It has been a very, very difficult year and I will never forget the long days and nights. But I am not one to dwell on such things for long and choose instead to focus on our blessings.
We celebrate a year, Little Man's 1st year, a year to a diagnosis. We celebrate the gifts Little Man has given us....patience, perseverance, advocacy, empathy, love, knowledge, faith, closeness, trust, blessings, support, strength...
Faith, Family, Friends....
Saturday, September 4, 2010
As I mentioned in my post yesterday, he also has a bit of a viral bug. Judging by how it is affecting other members of the family - it is making him feel just a bit icky-achy and off sorts...and actually he is coping with it better than his 3yr.old older brother! But, again, I still worry when he is "off" his baseline. Yep, I said BASELINE!! It is like a beautiful symphony - a baseline for an FPIES child with multiple intolerances/triggers is a difficult thing to come by. Making a formula he can thrive on when one of those triggers is clearly corn is the next difficult thing.....but we're finding it, and I'm doing it. I have optimized his formula well for calories, protein, fats and all the carbohydrates he can tolerate for a 14mo. old --although he is essentially a 14mo. old on a Vegetarian diet. It can be done. And it can be optimized by an Iron supplement made with Vit.C, which we will get on Tuesday! The hospital pharmacy here has the Iron and Vit.C powder and will compound him a supplement, that "will taste like rust" and it will be ready on Tuesday.
His weights are stable, he has not had blood in his stool for the past 2 weeks check, his tummy pain is gone, his diapers are "normal" and consistent, he is content, his lab check this week revealed his hemoglobin/hematocrit is holding steady (the minimum we were looking for- for it to not be dropping) and it will rise with the addition of the compounded iron this week, and some REALLY good news (for me!) is that his other labs are all picking up -- on my homemade formula.
When we first went to the pediatrician for help with Little Man's "issues", we got some initial lab work done at around 7mo.old. I had requested a Vit.D level to be done. There is much emerging research that is showing that many people are low on their Vit.D and even deficient- these low levels can have many varying affects on a body- especially a growing body of a baby. We were all a little shocked to find his levels VERY low, well past low cut-off for his age range. We immediately started him on a supplement and they climbed nicely. We had to stop that supplement because, despite it helping his Vit.D levels- I suspected it was giving him tummy aches (later learned I was right as there is corn based ingredients in it). Another check a few months ago (right before going to CHOP) revealed his Vit.D (and iron) were dropping again. Another indicator to me that despite exclusive elemental formula diet- little man was continuing to have intestinal inflammation. A recent check of Vit.D revealed it was climbing again- all on it's own. Sunlight? Hemp milk?
Another lab that we were alarmed was low was his Pre-Albumin. Pre-Albumin is a marker for protein status in the body. This lab was also below the cut off for low normal. It told me he either was not getting enough protein (breast milk), or that his inflammation was impeding him from absorbing it; or a combination of the two. We later confirmed he was indeed still reacting to components in my breastmilk even after giving up dairy/soy, and wheat; we now know he was reacting to rice and corn that I was eating in larger quantities to make up for the no wheat. The lab value for pre-albumin this week? Within normal limits!! Within the range of normal!! Hemp milk and decreased inflammation and his body is getting and utilizing adequate amounts of good protein for the first time in probably a year!!
We are not out of the woods yet, but each day we get closer and closer to winning this war- to successfully navigating these murky waters of our FPIES world.
Friday, September 3, 2010
I have continued to tweak his formula and now have added Hemp high protein powder to his current formula. This bumps up his protein and calories well, it also makes it a bit thicker so I am still playing with the right ratio of ingredients to make it most palatable for him. He has had varied intakes over the past week/week and a half and it always makes me nervous when he doesn't seem interested in his bottle -- especially when we are moving in such a positive direction for him. I am so nervous about him being sick again - not only losing ground but backtracking. I just wish everything could continue to go in this direction all the time.
He has had some 'typical' toddler things this past 2weeks- he is teething his 1yr.molars, tonight I felt a sharp tip on one side so those are close to coming through. Then, we are a sharing family- and that includes the germs and Little man has quickly developed a cough. He was diagnosed with asthma during our consultation at CHOP so we have the usual medication profile for an asthmatic. Except, how do you give an inhaler to a 14mo. old? Well, when he needs it- you give a nebulizer treatment instead. Little man got one last evening because daddy didn't like the way his cough was progressing through the day. The albuterol in the treatment made it difficult for him to sleep last night....well, at least that is what we are hoping it was from. Sleeplessness has been a part of his previous reactions to foods. Is he reacting to something in the Hemp protein? Is it too much protein for him- afterall he does have Food PROTEIN INDUCED enterocolitis - what if I make him intolerant to hemp? What would we do then? Please, please, please - let this just be a cold!!
My updates are few lately- we've been busy!! Busy doing what? ENJOYING our Little Man!!! This little boy is SO full of life, SO happy to be pain free, so loving. He plays nicely on his own, plays great with his brothers- now getting in on the wrestling and games, "asking" them to play, "joking" with them. This is a beautiful thing to watch -- and one of my favorite things as a mom....just to sit back and watch them play....watching from a distance so they don't know I'm watching (and not asking me to referee every 2 seconds) but watching them interact with one another, help one another out, big boys caring for the little boys. It is what fills my tank....so, so, so many months going on "E" - now my cup runneth over and I find myself wondering if I can really believe it, really trust it. When that lingering thought tries to come in, I just dive into some hugs and kisses from Little Man. It's as if he knows- he smoothers me with kisses and comes running up to me and throws his arms around my neck- squeezing in a hug! I can't get enough of that.
His brothers are starting to trust it more too- this is the longest we have seen a baseline, a pain-free and happy/content little boy since he was 2mo. old. They now fight over who gets to be his buddy for the day, who gets to show him this new thing, or teach him that new thing. It warms my heart beyond what words can express. When I can't express the words and I worry about the memories escaping with time, I photograph moments. Here are a few....backyard swing time, popsicles (hempsicles for little man), a trip to the local zoo, and a Eagle attraction center. We've been busy- we've been "normal" family of young children.