Adjustments

As we continue the Alimentum RTF via NG tube at home, we are not seeing any build of concerning symptoms and most of the other symptoms seem to be evening out.    We have not seen any more blood in his diapers, and his mood remains good (appropriate 2yr.old), his sleep is ok - and we continue to work that he can handle better overnight.  I suspect that his system is just simply struggling with the continuous drip all night long and wish that we didn't have that but there is no other way to get in that volume of formula.     He continues to drink his hemp milk well and enjoys his bottles but refuses any drop or mention of the formula.    He has been great with the NG tube though, maybe it is because of the PICC line and he is used to having a tube from his body.   The worst part of the NG tube is having to change the tape that keeps it anchored to his nose- he REALLY does not like us to touch it or mess with it at all (so he doesn't mess with it either!) 

Today, he ate peaches!  He asked for them at lunch time.   I cook and puree fresh peaches and store them in ice cube trays, so I can pop them out and defrost them in the microwave when he wants them, or when I want to use them for baking.   He ate 4 ice cube trays!   Licked the bowl clean.    He does like his peaches but he doesn't eat them regularly and he is not fond of puree....so this was really great to watch him, especially the licking the bowl clean!  

I continue to pray (and feel prayers for us) that he will continue to adjust with the formula and that the symptoms we have seen are simply just healing.   On scope just days before the challenge introduction of the formula showed that he still had some degree of villi damage....maybe we were seeing the symptoms because of that; but with the increasing feeds through his gut, we are seeing healing?  We are hopeful.   

We still do not know yet how we will continue to get the formula in him, if not an NG tube, than a G tube so that lingers on our mind...a decision that will not be easy to make...

He seems to have good days and then seemingly random off days.  We are concerned for his tolerance of the continuous feeding he gets through the night or is it something in the Alimentum itself?  Still not sure about it all because the other day was rotten and I am not sure what that was about- maybe he got "corned" (a term referenced in the corn allergic people when one is exposed to corn- because it is EVERYWHERE!)?  He was pale and yucky all day.  But he has been improved since; although he has random eruptions of skin coloring changes- not rashes but mottling, purplish skin, blue around his lips, etc....I can't seem to figure out what to make of all of that- doesn't have a history of it when not trialing foods (but presents itself when trialing).    He also has a serious "allergy ring" (diaper area).     We will need to work through these things over the next week or so -- before we decide to put a G-tube in for these feeds; we want to be sure he is 100% tolerating it.   

I try not to think too far ahead though, he is good today... and overall, he's doing really well- not as high maintenance and it feels good to have glimpses of a family routine and structure again that isn't completely around his mood and schedule.   

What is a Rare Disease?

From Rare Disease Day website:

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


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


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


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

Get involved!

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

About FPIES

When we began this journey, in the spring of 2010, the resources were few.   What is this FPIES?   When you looked it up online, the top posts were Kids with Food Allergies FAQ, BabyCenter FPIES support group, blogs, and Jacks YouTube video, alongside the dozen or so medical journal articles.  Of course this was all very useful and helpful information for us.   I read it all.  I started with the medical journal and research articles while we were still searching for little man's diagnosis.  I read as many as I could, and shared them with little man's pediatrician- who also read them to help us.   I looked up and read through every reference listed on these articles and read those as well- learning as much as I could about this immune and digestive allergy.  I then joined the support groups, and found a home among the moms on babycenter.   If you were to go back to May 2010 when I first joined, you would find dozens of my posts with question after question as I then tried to learn beyond the research and journal articles and into the every day aspects of this allergy from those living it every day....this new life we were learning to cope with for our son's benefit.  I learned from the other families, following what they were learning along their own journey's.  I learned what was similar to our experiences and what differed in our little man's FPIES.   I learned the importance of individualized care plans.  I learned that our little man has more chronic FPIES, and what we call "build" reactions before an acute reaction is observed, and I shared these experiences and heard them echoed among dozens of families.   I then began, like the families before me, to pass along what I had learned for our son.   


Maybe it was because of our struggles to find practitioners experienced in FPIES to help us with our son's FPIES or maybe it was the healthcare professional in me, but I couldn't help but see this from both sides- from both needs- medical professionals need support and shared experiences just as much as the families to help other families.  I began to see how useful it would be to have the information- this golden information in this amazing support group that I was learning so much from- to be collected, compiled and centralized not only for the mother in the middle of the night searching for answers, help and support but also for the medical professional to find a resource to turn to to help these same families. The support groups are a safe and comforting place for families and it is truly an amazing thing to be a part of- the families in these support groups are like no other community I have belonged to, the passing along of information, experiences, insights into what they have learned and observed and connected for their child, in the small hopes that it could help another new mom whose journey is just beginning, or a mom who has hit a bump and is struggling, or someone looking for a new recipe or tips to coping with this chronic illness. 


A clinical diagnosis can benefit from a collaboration of experiences, a foundation for FPIES.  And now, when you look up FPIES, the resources are many and growing.   Now, you can find an easy to read and understand (and share!) article About FPIES.  

About FPIES

A Collaboration Between The FPIES Foundation's Board of Directors and Medical Advisory Board

Food Protein-Induced Enterocolitis Syndrome (FPIES) is a type of food allergy affecting the gastrointestinal (GI) tract. Classic symptoms of FPIES include profound vomiting, diarrhea, and dehydration. These symptoms can lead to severe lethargy, change in body temperature and blood pressure. Unlike typical food allergies, symptoms may not be immediate and do not show up on standard allergy tests. Furthermore, the negative allergy evaluation may delay the diagnosis and take the focus off the causative food. Nonetheless, FPIES can present with severe symptoms following ingestion of a food trigger.
FPIES Common Symptoms:
There are two ways that infants or children with FPIES might come to medical attention.
		1. The classic pattern of an FPIES reaction is when a healthy infant or child develops symptoms shortly after eating a food. There is a characteristic delay of 2-3 hours before onset of severe and repetitive vomiting and eventually diarrhea. The child may appear very ill and sleepy (lethargic), and may become pale or blue. When evaluated by a doctor, he/she may be found to have low blood pressure, seem dehydrated, and have blood tests that mimic infection (sepsis); which in some cases can lead to sepsis-like shock. Many infants who are eventually diagnosed with FPIES are initially suspected to have a severe infection or sepsis. 2. The second common pattern of FPIES reaction symptoms occurs when infants who are ingesting a problem food (usually milk or soy-based formula or proteins in breast milk) as a consistent part of their diet might experience increasingly severe vomiting, diarrhea, and poor growth, possibly progressing to an illness mimicking a severe total-body infection. Please note that each child is unique and your child may experience their own range and intensity of these symptoms.
FPIES Common Triggers:
In the first months of life, FPIES reactions are most often caused by cow’s milk protein formula, and sometimes by soy. Proteins in breast milk may also cause symptoms in some infants. For infants experiencing FPIES with solid foods, rice and oats are the most common triggers. Current research reports other common triggers that include, but are not limited to, milk, soy, barley, poultry, peas, green beans, sweet potatoes, and squash. However, any food protein can be a trigger and some infants may be sensitive to other foods as well. In addition, some children may react to one or two foods whereas others may experience reactions to multiple foods.
FPIES Diagnosis and Testing:
FPIES is a non-IgE food allergy, which unlike classic food allergy, cannot be diagnosed with readily available food allergy tests such as skin prick test (SPT) or blood test that measure food IgE antibodies (RAST). These tests are helpful to identify triggers for typical food allergies that result in immediate hives, wheezing, and swelling and are characteristically negative in FPIES. An FPIES diagnosis is usually made by considering the history of the characteristic symptoms and exclusion of various alternative illnesses. A medical doctor, often an allergist and/or gastroenterologist, should be involved in making the diagnosis. Although Atopy Patch testing (APT) is often used for FPIES patients, it is not considered a validated test for FPIES diagnosis. Blood tests performed during a reaction may be helpful since the results often mimic the body’s response to infection. The most definitive test is a medically supervised oral food challenge (OFC)-where the suspect food is given to the child in a controlled clinical environment. An oral food challenge, however, is not often needed initially if the doctor has excluded alternative illnesses and the medical history is consistent with FPIES.
FPIES Treatment and Course:
FPIES reactions can be severe. It is important to get to prompt medical attention where treatment, such as fluids given into the vein to help stabilize blood pressure and treat dehydration, can be given in order to avoid sepsis-like shock. Although some doctors prescribe epinephrine to stabilize blood pressure before medical treatment, the main therapy is to get intravenous fluids; also steroids can be used to quell the immune reaction. Preparing a letter for potential trips to the ER, containing both FPIES information and a list of your child's triggers, may be helpful. Unfortunately, there are currently no simple tests for FPIES. The primary test, as mentioned above, is a medically supervised oral food challenge with the trigger food. The good news is that FPIES usually resolves with time.  Your child will need to be closely followed by his/her doctor to discuss what foods are safe to eat and when it may be time to determine if FPIES has resolved. With proper medical attention and a personalized dietary plan to ensure proper nutrition, children with FPIES can grow and thrive.
		Additional Useful Resources 1. Caubet, J.;Nowak-Wegrzyn, A. Current Understanding of the Immune Mechanisms of FPIES. Expert Review.Clinical Immunology. 2011; 7(3), 317-327. 2. Jyonouchi, H. Non-IgE Food Allergy. Inflammation & Allergy. Drug Target. 2008; 7(3): 1-7. 3. Nowak-Wegrzyn, A.; Sampson, H.A.; Wood, R.A.; Sicherer, S.H. Food Protein-Induced Enterocolitis Syndrome Caused by Solid Food Proteins. PEDIATRICS April 2003; 111 (4): 829-835. 4. Sampson HA, Anderson JA. Summary and recommendations: classification of gastrointestinal manifestations due to immunologic reactions to foods in infants and young children. J. Pediatr. Gastroenterol. Nutr. 30(Suppl.), S87–S94 (2000). 5. Giovanna M., et al. Food Protein-Induced Enterocolitis Syndrome by cow’s milk proteins passed through breast milk. The Journal of Allergy and Clinical Immunology. March 2011; 127 (3): 679-80.

Crap...

Well, literally.     

Little man has another diaper with blood in his stool - sent it for testing to reveal it was positive for blood but before that, I took a picture of it (yes, I take pictures of my son's diapers- YUCK, but trust me- it is a lot easier to analyze via a picture than standing over it poking at it.  And the pictures have helped the GI doctor see what I mean by what I describe....they know their poop!   She is concerned by it and wants to watch him closely- she wants to see him if he has anymore blood over the weekend.   He had a diaper today with no visible blood which is great but the neon green, slimy, mucus, smelly parts were not encouraging.   I am more concerned about malabsorption problems.  He is taking LARGE amounts of formula (Alimentum through the NG tube and Hemp via his bottles)- 67oz/day for the past 2 days!   

We worked out a feeding schedule for his NGT that we feel is something he can tolerate well- without overfeeding him.   It gives him ~43-45oz. of Alimentum/day while still allowing him to take his bottles- which we are not pushing but he ASKS for, and drinks every last drop of- his appetite is increased!  Part of that is because of not having the IV nutrition anymore.   His caloric intakes are about the same (1200-1400 calories/day) as they were on TPN and hemp milk.  So we are hoping to see a weight gain this week.  If there is no weight gain, and his diapers continue to be mysterious, we may have to look deeper into what is going on.   Maybe this is all just adjustment stuff as his gut gets used to the new "food".    He still won't drink the Alimentum though so we would be looking at a Gtube if we deem it safe after a few weeks via the NG tube.   

His mood has been poor; plays nicely but hits a wall and then just falls apart- so something is bothering him.   We just need to continue to take it one day at a time and see what tomorrow brings....

Motherhood is calling, an FPIES mom...

Last week, a fellow mom shared this letter she wrote to help her family understand what it is to be the mom of a child with life threatening food allergies:

To My Family by Beth Cowart

"My son has a severe protein allergy to milk & soy. This is not the same as being lactose intolerant.  This is not the same as colic.  This is a severe allergy that has almost taken the life of my child twice. This allergy is time consuming, life consuming, overwhelming and frustrating.  People seem to think it is as easy as just not giving him milk or soy, but it is not. 

It is label reading.  It is shopping at 4 different stores to get all of his safe foods.  It is frustration of not finding any foods he can eat because soy is in everything.   It is the disappointing trips to the grocery store, and returning home yet again empty handed for this child.   

 It is calling and being on hold with food companies for what seems like hours to be 100% certain that the vitamin E or natural flavoring is not soy based, because soy fat ingredients do not require labeling for allergens.  It is constant cross contamination research to check what foods are run on the same line as foods made with dairy and soy, because I will not let a drop enter his body.

It is constant research, constant planning, and constant thought about what foods to try next and in what order.  It is the facts about food constantly running through my head.   

 It is constant recipe search, trying to find new ideas for the same foods search, and recipe experimentation. It is the constant meal planning. It is food journaling.

 It is frequent trips to the store, since everything needs to be made from scratch.  It is the steaming, blending, storing, cleaning, and repeat, multiple times a day.  It is the not having the luxury to do "fast food" ever, even on those days where you just need a break from cooking.

It is trying to explain to his sister why she can't share her cookie with her brother.  It is the look on his face when he wants to try just a bite of what you are eating.  

It is expensive! All his foods need to be organic, natural, and whole.  It is having to give up the luxuries that are my sanity in order to pay for hypoallergenic formula, because each can cost $39 and one can lasts about 48 hours.  Although expenses are the smallest of the issues because his health is more important than anything money can buy!

It is the constant wonder if a behavior is due to an allergy or not.  He is fussy: is he tired or reacting? He is not eating:  is he full or is he reacting? He had 3 dirty diapers today:  just an off day, or is he reacting?  He is sleeping a lot today:  is he just tired, or is he reacting? He seems uncomfortable:  is it his teeth or is it his stomach? It is the fact that your heart always goes to the allergy, and your head has to try to talk your heart out of it. 

It is the poop checking.  Every day for 7months, poop check.

It is the not having anything in common with your friends anymore, because you eat, sleep, and breath this allergy.  It is talking to people in online support groups that you have never met before more than talking to your friends, because they are the people that truly get what you are going through. 

 It is the prevention and worry of cross contamination in your own home. It is the little decisions about food that have turned into monstrous ones.  It is the selfishness of wanting to introduce more foods then you should for convenience, and then the guilt of even thinking of that.  It is the constant thoughts of food running through your head that have taken all the joy and fun out of introducing them to your child.

And the worst of all is the constant anxiety that you are going to put your baby through a reaction again if you don't do everything right.  It is the constant fear that overwhelms you every time you put something new into your child's mouth."

I reflect on what she is writing because so much of it are my very own thoughts.  There is a connection between FPIES moms because we can so easily relate to one another with this journey we have been thrown into before our babies are even past the infant stages.  Just when you think you're settling into a routine past the infant days, you are thrown into the life of a chronic illness.

A mother is created to nurture, a baby is born completely dependent on that need.  A baby soon grows into a toddler, preschooler, and before you know it, they are off to school.   Children grow up so fast, life happens in the blink of an eye.

Motherhood is calling, and when it calls an FPIES mom it takes nothing less than a woman made of steel- one that can exist on what seems like no sleep to stay up all night comforting a crying baby, one that can push past their own fears to feed a baby food that may end up making them sick in hopes to expand their diet and nutrition, one that has to chart food logs and keep poop journals, one that has to search for a doctor knowledgeable in this allergy -even if it requires them to over advocate for their child, because not every doctor is trained in it and a clinical diagnosis benefits from experience, one that has to put on a strong face when their heart is crying as they watch as their child undergoes procedures and allergy testing to help doctors carve out the individualized care plan their child will need to thrive through this illness,  one that has to stand strong and kindly tell a begging toddler "not for you" when they ask for a food that is unsafe or contains unknown ingredients, one that sets aside their own needs and sits with a sick child for hours or even days just to be next to them because the loved ones touch is the only comfort they have after a chronic reaction and the gastrointestinal stress it does to their body.

Motherhood is calling, an FPIES mom.....

One day at a time....and celebrating the small victories along the way

The plan for readmission was to do the challenge dose, wait 12hrs, and then begin his 24hr.dose of 50oz. of Alimentum via NGT feeds.   That dosage begins at 12am Tuesday, and he receives it through the night and every 3hrs. throughout the day.  He did drink some hemp milk formula bottles on top of that- as that remains a comfort item for him and we want to maintain that.  He played SO well today, SO much energy and smiles and giggles and barely any meltdowns and no other signs or symptoms.

With an NG tube inserted, and formula looking like a pass; the other thing that needs to be done is removal of his PICC line.  His PICC line that has been in his chest since mid-August is now not useable for IV nutrition, and the risks outweigh the benefits of keeping it in.  It feels strange to think of his "lines" not hanging from his chest, very strange.   But we are anxious, and glad for this moment.  Little man recognizes the walk to the treatment room and his bottom lip begins to quiver.   That look, that look of understanding and comprehension that he knows, that look of fear and "mommy please tell me it is going to be ok, and you'll be with me" look in his eyes.   I swoop him up and quickly tell him how proud I am of him- he is being so strong and brave.   He is such an amazing little warrior. 

It was a quick procedure, actually oddly quick- scary how fast and easy that would come out of his chest.   A small scar will be present but likely it will just disappear into his chest as he grows, as his arm PICC line scars from Dec.2010 have.   

We anticipate being discharged the next morning- he is doing SO well!!  They will start his home regimen doses through the night Tuesday night to keep us on track for Wednesday at home feeds.    Except we forget to calculate that he doesn’t need a full nights dose, after already getting a full 24hr.dose.  He got 30oz. overnight from midnight to 7:30am- a lot of volume! Too much!  Back to back full doses resulted in a vomit session on Wednesday morning.   

I get to the hospital around 8am, and he had just been cleaned up and was happily playing in the playroom when his daddy broke the news to me….”he vomited this morning”.  I stop dead- NO!  What?  He looks ok now but that isn’t new, he’s had reactions before where he played fine after.   They have not done labs yet and I’m concerned as we do know that blood counts can return to normal quickly following a reaction- within hours is what the literature says.  Although his electrolytes will still be “off” as his body fights the dehydration….but after just getting 30oz, I’m not sure we’ll see dehydration signs!

Two hours after the vomit session, we finally get a lab draw.    I’m concerned over the timing of this because it was supposed to be immediate but with further discussions with his daddy, we don’t feel this was an FPIES vomit session- it just didn’t seem like it to him.   It was a lot of vomit, and he emptied his stomach contents but it wasn’t retching vomit and it wasn’t to bile…it was stinky Alimentum (and I can still smell it on him- both of them, which isn’t normal with the bile vomit- that has a different smell, an acidic smell vs. this putrid sick vomit smell).  We are all encouraged that he is happily playing but now vomit and we’ve seen 2 blood in his stools….I want to be sure we are doing everything we can to collect what we need to analyze this as we move forward.   We wait for the labs to get back to read and request a consult with our GI for her thoughts on if we should have further inpatient evaluation or not. 

The labs come back and they ALL look good- hemoglobin is good, white cells are good, electrolytes are good.   The on staff pediatrician comes by to relay the good news.   We speak to our GI shortly after that and are reassured that she is following closely and although she remains concerned over the blood in his stool, she is encouraged by all of his other progress.  She agrees with our thoughts that we simply overfed him and tells me to slow his rate down tonight so he doesn’t get as much overnight and we don’t risk repeated vomit sessions.   We are set to go home- all of our equipment for an NG tube.   We have follow up appointments next week with the dietitian, pediatrician and GI to assess where we are at in a week and to make plans for next steps (tweak formula volumes, try another formula to get higher concentration, discuss Gtube vs. getting him to drink a new formula)…so much to think about, so much to process.    For now, we are home- re-adjusting to our schedule and adjusting to this new "feeds" system which he seems to be tolerating well, he is playing well and enjoying being home.  He even had a FULL bath!!   He hasn't had a bath since August because of the potential of introducing infection into the bloodstream with the PICC line- hard to not get his dressing all wet when you are two, splashing in a bath, so we’ve stuck to sponge baths.

One day at a time....and celebrating the small victories along the way…

Challenge for Chronic FPIES

From The Current Understanding of Immune Mechanisms ofFPIES “FPIES manifestations differ in the chronic and acute forms…”Removal of the offending allergen should lead to rapid and complete resolution of symptoms within a few days. This chronic initial phase can be followed by an acute phase if the antigen is removed from the diet for at least 2–3 days and subsequently reintroduced, with pathognomic symptoms starting approximately 1.5–2 h after ingestion. Severe, repetitive vomiting generally occurs 1–3 h and diarrhea occurs 5–8 h after feeding [13]. Associated features may include pallor, lethargy and cyanosis”.

While we celebrate that our little man has not had an acute reaction the Alimentum RTF (from the casein protein), we are still concerned for a chronic reaction, based on Samuel’s history of chronic FPIES (dairy through breastmilk, soy, rice and corn triggers were all chronic FPIES before we pulled them from his diet and reintroduced and confirmed the acute FPIES.  

Little Man had an acute reaction of projectile vomit, and diarrhea to Nutramagin at 6mo and again at 7mo.  Nutramagin is also hydrolyzed dairy proteins- like Alimentum is (just different company).  So, we did not try Alimentum at that time. Two years later, a reintroduction of this same hydrolyzed protein did not result in an acute reaction is a VICTORY for our little man!   A sign of his FPIES triggers healing, one by one.  A sign that by avoiding milk in any and all derivatives from the beginning helped his body lose the Tcell memory that it once considered it an antigen (something to attack).  We are very encouraged by that.   But since he has more of a history chronic FPIES and since his body has not tolerated tapioca starch or soy oil before (other ingredients in Ali RTF), we want to be sure we have taken all the measures to be sure his body is accepting this nutrition.   

After a weekend rest, we are re-admitted on Monday morning for the challenge dose of Alimentum (5oz. via the NGT); and we will wait 12hrs. to observe for a reaction.  In that 12hrs, he will get his hemp milk formula.   We are admitted around 9:30am, baseline labs and assessments are done and Sam gets his dose of Ali RTF at 12noon.   He isn’t settled enough for a nap but at 2pm has a diaper that has blood in it again.   This is not encouraging and this one is more than the first one….but he didn’t have them all weekend and now with the challenge dose he has it again.   The stool is not diarrhea, but the blood has us concerned.   He has a fairly uneventful rest of the day and 6hrs. comes and goes, then 9hrs and then 12 and NO reaction.    NO vomit, no diarrhea, no symptoms (other than the blood in his stool).   I breath a sigh of relief and collapse into sleep- I’m exhausted….trial days are exhausting as you wait and see if the body is going to react or accept the food. 

Pressing through and ready for a break...

With the picture muddy, we decide to increase the dose to help clear things up.   The increased dosages start in that afternoon (Wed), and he begins to perk up even more, improved demeanor but he looks more pale and tired, and has some blue (cyanosis) around his lips.  I am very encouraged by his improved mood but still confused on the paleness and cyanosis episodes. 

We anticipated some symptoms, Little man’s body has not accepted new food in so long- and the plan was to press through….we will push through to tolerance or reaction (vomit/diarrhea/dehydration).  We need to know.   This isn’t a typical trial- it’s more of a challenge but not a challenge we can guess on, a challenge we need to be sure.  This formula could help sustain him for the next 2+yrs as his body outgrows the level of reactivity it has had, as his gut heals, as he grows.  We will press on.  We need to know.  

We also need to significantly increase his doses, a discussion with the dietitian reveals he will need ~50oz. to meet his Vit.C needs;  which he does not get in his hemp milk formula, the only thing providing Vit.C is his diet is peaches which he doesn’t eat daily.    The Alimentum will also provide some B vitamins that his vegetarian protein source (hemp) does not.  The B vitamins help his body turn the iron into oxygen rich hemoglobin and further help prevent his anemia.  We are quickly overwhelmed by this realization that the Alimentum will not be a supplement but pretty much needs to replace his hemp milk (in volume).

Wednesday night he continues to receive the feeds through the night, he has increased gassiness in his sleep and is tossing and turning a lot through the night….nothing that wakes him out of his sleep but definitely different than how soundly he has been sleeping for the past many weeks.  Thursday morning  brings a cranky and whiny boy in the morning but he eventually starts playing and plays well through the mid-morning.  During rounds with his pediatrician today, we discuss how we want to increase the Alimentum to get closer to what he is going to need and see what that does for his symptoms.   So, the first day (Tuesday to Wednesday) he got 8oz. of Alimentum (mixed with his hemp milk formula) via the NGT, the 2nd day (Wednesday into Thursday) he got 12oz. of Alimentum (mixed with his hemp milk formula) via the NGT and now, the 3rd day (Thursday afternoon to Friday afternoon) we want to increase to 24oz. Alimentum mixed with his hemp milk formula via the NGT (we divide this increase into 12hr.segments- building him up slowly). 

I also discuss that because of the symptoms he has been having, that are still not clear (don’t seem to be building but are still present as we press forward), we will need to try and rule out if what we are seeing is a chronic FPIES.  A recently published article outlines the chronic FPIES: “The CurrentUnderstanding of Immune Mechanisms of FPIES” (page 4).   Little man has more chronic FPIES reactions than acute FPIES.   Once we realized FPIES was what was ravaging his body, and removed his trigger proteins we saw dramatic improvements in his gut symptoms (chronic vomiting, diarrhea/constipation, etc)….reintroduction of these proteins results in the classic acute response (profound vomit followed by diarrhea and dehydration).  His worst trigger remains corn.  You can almost set your clock by a corn ingestion- even trace/contamination amounts.   So, to be sure he is tolerating the Alimentum, I propose we remove the potential trigger protein- going home for the weekend, get back to a baseline (be sure what we are seeing is stress or hospital related) and we reintroduce a dose on Monday – a challenge dose; followed by a 24hr.”feed” of the 50oz. he needs for his nutrition (to be sure he can digest this volume).    The pediatrician agrees to this plan and we press through Thursday, increasing the Alimentum by Thursday afternoon. 

That afternoon, he is very tired and cranky after his late nap, mostly just wants to be held, noticed cyanosis on his legs again, and also a “lacy pattern” red flare color to an area on his arm, and his skin is mottled looking.   He clearly isn’t feeling good but it is not yet clear why.  Then around 7pm, he finally has a bowel movement and there is clear streaks of blood in it.  We get it tested and it is positive for blood.    Blood in a the stool of a child with Enterocolitis is concerning but we press on; this will be evaluated later, especially if he continues to have them.  Our GI has been clear with us that she wants to assure he is fully tolerating the Alimentum before committing him to a Gtube – which is why we elected to the NGT, so we can do 2weeks full strength and then assess where he is at, with as sensitive of a system as he has- we know to proceed cautiously

Going into Friday, we’re getting ready for a break.   We are ready to go home.   We are still concerned about the blood in his diapers and other symptoms but hope that this break will help us see things through a clearer light. 

He is SO excited to be home!  He even asks for his peaches and eats about a 1/4cup of puree peaches!!  He is a little clingy in the evening and struggles to calm down to sleep but he slept well overnight, and awoke looking more rested than he had been in the hospital- maybe it’s the lighting?  Maybe it’s just the comfy bed at home vs. hospital atmosphere.  This little boy is glad to be home and plays well with his brothers.  His diapers do not show any signs of blood through the weekend.  By Sunday evening, we are seeing the effects of not drinking enough of his hemp milk formula.  He used to drink 64+oz/day before TPN, meeting his 1200-1400 caloric needs/day; but he’s not used to having to drink that much since TPN provided him 800calories/day, he dropped down to ~30oz.; so now he is only back up to ~45oz. and it is clearly not enough.  If he were to stay on hemp milk formula alone, I’d have to go back to setting my alarm and waking at night to feed him through the night to get him back up to his daily requirements (I did this for 2mo. after his initial TPN in Dec.2010, until we started probiotic and his appetite increased during the day….I would do it again if need be.    But we are heading back on Monday to rechallenge the Alimentum RTF.  

Muddy Picture

We knew we’d have things that would come up in the trial/challenge to muddy the picture, we did not anticipate Little Man completely refusing the formula and the decision made for us to starve him to get him to take it.  We did however anticipate the NGT (nasogastric tube); we had discussed it previously with the GI- how he may need it to do the trial, to be sure he consumes enough for us to call it “safe”, we also discussed a Gtube (a feeding tube surgically inserted into his stomach) if the Alimentum RTF was an FPIES pass but if he does not accept it…we know it will at least need to be a supplement in his diet. He has hemp milk that is providing the calories and protein and fats he needs, but it is lacking in some nutrients- the nutrients the Alimentum would be able to provide.   We haven’t done the math yet on how much he needs of the Ali. 

The Alimentum RTF trial continues and Little man continues to get round the clock “feeds” via the NGT of the 1oz. Ali+3oz. Hemp milk mix. He appears to be tolerating it well and in the first 24hrs. with 8oz. of Alimentum in.  This is a good amount, but because of the events of the day before , prior to the NGT, we are no longer at a baseline  and we’re not sure if it’s the Alimentum slipping him downhill or simply his body trying to recover from the previous days’ events.    

We are optimistic of his progress, Wednesday morning his cheeks are rosy, he slept well overnight, wakes up ready to play, and enjoys some playtime  when his brother visits.    So, we began to discuss the next 24hrs.  at rounds on Wednesday morning, we express and discuss how we feel we need to increase the Alimentum he is getting- because of the symptoms he is presenting with that are unclear if they are building FPIES or leftover from the stressful beginning days.  I am hopeful it is just the stress, and am encouraged that we haven’t seen any of his “classic” building symptoms of pain, agitation, decreased appetite and disturbed sleep.  But I am troubled that we jumped right into lethargy, pallor and cyanosis.  But, even though these are seen during an FPIES reaction (acute or chronic), they alone (without vomit or diarrhea) are not defining criteria; and especially not now for little man because of the stress his body has been under.   So, we press on as planned but decide that we should continue to increase his doses to see if that makes the muddy picture clearer.  We will increase to 2oz. Ali RTF + 2oz. hemp milk for the next 24hrs. 

He'll eventually get hungry enough....

Little Man is admitted for the formula trial, and the hopes to find him a supplement to his hemp milk formula.   We have decided on Alimentum RTF for that trial because although it contains casein (dairy) hydrolysate and tapioca starch as well as a tiny amount of soy oil, and also DHA/ARA that is becoming common place to have in formula’s….it doesn’t have corn.  And believe it or not, all of these risks are less than his risk to react to corn.  He has become more sensitive to corn instead of less over the past 2yrs., but the hope is that he is becoming less sensitive to some of his other original triggers.  The trial is a challenge and will be done in the hospital.   Little man is a chronic reactor (delayed reactions) so we also want to document any symptoms he has along the trial, even if they don’t lead to an acute FPIES reaction.  We get admitted on Monday and get all settled in, reviewing the plan with the doctors, and attempt to begin giving him the formula.   We decided a 1/2oz. mixed in 3oz. of formula may disguise it enough to test it out.   I was wrong, there was no disguising the taste of this formula and little man noticed it immediately and was not happy about it!   It is a long afternoon as he becomes more and more hungry and frustrated, and keeps asking for his bottle – to which we give him the 1/2oz. Ali RTF and 3oz. hemp milk mix.   His daddy arrives for the night and I am hopeful a new approach will help.  Daddy tries Ali hot, cold, over ice, in a special sippy, through a straw little man drinks about few swigs before he realizes and then refused.    We try to be optimistic that he even took the few sips but we are concerned over his refusal and hunger.  It really isn't something we are comfortable with, it isn't why we are here (in the hospital)

We request to speak to the Pediatrician.  We've seen little man starve himself before and we've watched him spiral down fast....of course this is after a reaction when he's already very sick; but we're still very worried because of his yet unexplained hypoglycemia (low blood sugar) issues he's had in the past.   If they have to give him dextrose to keep his blood sugar stable, he may not feel hungry and the dextrose (being from corn) could "muddy" the trial (he's had it in the past but with questionable symptoms).   So, we are advised that "he'll eventually get hungry enough and eat".   Well, I know that is an old saying for "typical" kids who are being picky; but it doesn't work that way for kids whose food causes pain.  We have had hospital admissions for this very reason, "he'll eventually eat when he gets hungry”, they feel he will eventually accept the Alimentum mix if it is his only source of food.   We do not agree with this recommendation because of what we know about Little man but we agree to give it overnight.  The main concern beyond him being hungry is that this isn't what we came for- to starve him, to dehydrate him, we came to challenge a formula- to find him food- not take it away.  

Thankfully, his daddy does get him to "dream feed" in the middle of the night- he takes some of the mix in his sleep.  THAT is a good sign and we are hopeful; but it wasn't enough.   He has one wet diaper in the morning and then not again all day, his lips are getting dry and his eyes are looking sunken.  And he is NOT happy.  He refused any attempts or mention of the bottle….and we fear he is gaining mistrust in something he has trusted for so long- his one stable food source, and a true comfort item.   By the time the doctors rounded (late morning) it now had been 22hrs and he's had 6oz. to drink (1.5oz. of Ali RTF).   

We discuss the nasogastric (NG) tube.  Our GI discussed this with us before we came to the hospital- how we may have to do the trial with the NG if he refuses to drink enough; we would then know if it was "safe" and see if he would drink it (while he maintained his NG); if he would not drink it; we would have to do a Gtube (surgically inserted into his stomach) to assure he gets the nutrition he needs.   This does seem drastic when he DOES eat by mouth, he takes his bottles VERY well and can drink up to 60-70oz./day without a problem, it is adequate in calories but lacking in some nutrients.  All he needs is supplemental nutrients- that he can DRINK.  But we have searched and not found these in 2yrs.....

This hospital stay was already becoming more complex than we had anticipated (and we anticipated complexities!).   So, right now, we need to focus on today.   Today, he needs nourishment.  Today, we need to find out if the Alimentum RTF is "safe" for him. Today, we need to make the decision to have the NG tube inserted to give him Ali RTF through it and let him have plain hemp milk in his bottles for his comfort.  

We make that decision and we head to the procedure room with the nurses to insert his NG tube.   After it was placed, we had to get it checked on xray to be sure of the proper placement, during that time- we offer him a bottle (of plain hemp milk) and he, thankfully, accepts it and instantly starts to (finally) feel better and drift into sleep (for a nap)….HUGE sigh of relief.   He's been through SO much already and we've barely just begun.  

X-ray showed that the tube was not placed in his stomach, so back to the procedure room we went.   This time was successful and the first feeding of 1oz. Alimentum and 3oz. Hemp milk went in around 3:30 on Tuesday.   The plan now is to continue "feeds" of 1oz. of Ali + 3oz. of Hemp milk every 3hrs for the next 24hrs. and see what/if symptoms present.  

The other thing that the X-ray showed is that his PICC line is not sitting in a good place, it is decided that it is no longer safe to use for TPN.   We will keep it placed in case he needs it for hydration while we work through this trial/challenge but plans are made to have it removed soon.  

Alimentum RTF Challenge, are we ready?

Ok, so if you follow my blog you're probably wondering why I haven't updated.   I'm not superstitious by any means but there is something about FPIES and writing something out....I like to be sure before I put into words what 29,000 people could potentially read.   And we've had a busy and sometimes muddy 6weeks.  (this post is backdated to reflect the accurate timeline)

The decision is made to challenge Alimentum Ready To Feed (Ali RTF), it is a challenge because he has reacted to both casein (dairy) and tapioca before...although we've never been sure if his tapioca was FPIES or dissacharide deficiency related, we've simply avoided- it made him very, very sick and necessitated a hospital admission to recover.  We plan for the hospital challenge, to observe for both acute or chronic reactions, since Little Man has more chronic reactions than acute and this isn't a food we can shelve for varied symptoms, we need to be sure we looked at everything and that his symptoms are logged carefully if needed to look at them retrospectively.

We prepare for the hospital stay, a planned 3 days to assess all symptoms and tolerance.   We plan on 3 days but we prepare for more- knowing little man always seems to throw us some curve balls.    The days before, we start to get nervous, nervous about making him sick, nervous about his failing PICC line and what we'll do if this doesn't work.  The PICC that was scheduled to be converted to a port just a week prior, a decision based on his inability to tolerate the food trials we had done at home for the past 2months; but a line infection scare left us rethinking things at the last minute, deciding that the benefit of tolerating the Alimentum RTF is worth the risk of a reaction being that it has been 2yrs. since he has had a dairy derivative (in a similar formula).

Excited? Yes, that if he passes Ali RTF- we could have a supplement to his hemp milk formula and we could just BE- just take food trials longer and only when he's ready instead of pushing him.   We could make plans easier, we could see little man at a baseline for more than a week before we move on to the next step.   We can just breath.

Nervous?  Yes!  Little Man hasn't had a food pass since the summer of 2010!  His body hasn't accepted a new food for 18mo.! (another post).  Will it now?  If he isn't ready for dairy or tapioca, do we risk re-sensitizing him and having to wait longer to trial it again?  And of course, there is the fear of making him sick and having nothing to build a formula from.  And....will he even drink it?