Which way are we going?

We have no new foods, and we have put ProViMin aside.  Something was causing full allergic colitis.   We started by checking labs and stools and correlating the ProViMin (increased and decreased) intakes to the results.  The first weeks it was evident something was going on but, just as we were left guessing with his symptoms and ups and downs, the labs/stools left us guessing a bit too....was it leftover from Alimentum RTF?  Alimentum caused full enteropathy: reflux, villi damage, decreased ability to digest sugars, large stools, dumping proteins, malabsorbing calories and blood in stools.  It did not cause the vomit issues we have seen with his reactions, so it was either something trace or broken down and it didn't seem to be flaring his enterocolitis.  But enteropathy is just as bad- just not as sudden.  It took us months to determine that yes, he was in fact reacting to the Alimentum.  I didn't want that to happen again so we were taking the ProViMin cautiously but so many good signs were there too, initially.   ProViMin has less broken down dairy (from hydrolyzed dairy proteins to a piece of a full dairy protein - sodium caseinate) and yet his enteropathy was flaring down, his body was beginning to absorb nutrients again and his hemoglobin that was declining had picked back up....but his iron stores tanked again and his protein stores were not picking back up.   Did we just need more time?    Did we just need to push the ProViMin more?  

So, we came back from camping and I pushed the ProViMin- anytime he was sitting down or started acting up, I would ask if he wanted a bottle; I would sit with him if I needed to get him to sit still and take a bottle.   We did dream feeds (where we feed him in his sleep) and he would wake up 1-3x/night to eat himself.  He was getting ProViMin around the clock.  Things should be improving if he was merely recovering from Alimentum.   

My theory was that while recovering from Alimentum enteropathy, his body wasn't fully accepting the ProViMin either.  My instincts were right.  

His body has been having allergic colitis from the ProViMin.   I'm not sure when that kicked in, or if it's been there the whole time because he did seem to be doing ok on it at first, but then he slowly began to get more fatigued and have poor color, he gets this subtle cyanosis around his lips when his intestinal inflammation is "triggered" - it comes and goes if he is getting the protein throughout the day (such as in his bottle); or it will flare up for a few hours if we are trialing a food.  It's beginning to be a good red flag for us....but I can't stop the food on just that alone, I (unfortunately) need symptoms to add up.  

When we got back from camping, we were turning in samples and going to the lab- one Friday's lab results revealed a "spike" in his normal- he had Eosinophils in his blood and his hemoglobin was dropping, Ferritin was quite low as well.  His stools from a few days (week) prior had a significant jump in the markers for inflammation.  Something isn't right.    The GI called and she was alarmed at the results and felt that it confirmed he was reacting to the ProViMin.  Her thoughts were that we needed to stop the ProViMin and get him scheduled for an admission to re-start TPN (total parental nutrition via an IV).   She has no other idea's on why his body can not accept food right now.  It is the answer that she knows will keep his body alive and his brain fed the nutrients it needs.  I know she is just looking out for his needs- both in the now (stop the ProViMin because it's hurting him) and in the future (provide nutrients via TPN so his brain doesn't suffer later); but I just can't help but ask myself if we are doing the right things...do we look further? Do we go somewhere else for 2nd/3rd opinions?  Do we put him through new tests and hospital stays and who-knows-what-else?  Or, do we just do what we know to do and stay home.    I don't yet know that answer, I've been praying and praying about it.  I just can't see through this muddy dark tunnel.   

For the immediate needs, hubby and I talked about it and we decided a few things.   One is that he wasn't as sick as he has been in the past and we still aren't sure it is the ProViMin.   He seems to be getting more and more corn sensitive, so maybe he got "corned" the day of the stool tests- his diapers that he has always been ok with are now causing rashes (diapers typically have corn starch in the liner, he has always seemed to tolerate Pampers baby dry) so we switched diapers and wipes (7th Generation diapers- tolerates MUCH better) and Waterwipes wipes...which are on the fence of tolerance but a significant improvement over the wipes I've been using with all the corn-y ingredients.   Also, a few days before the lab draw that showed the significant changes, he had had butter and a lick of yogurt (planned introductions to be "push" dairy to see if that is what he was reacting to in the ProViMin and both I had hoped from my research, would be corn free but wasn't positive), he also ate a good portion of pork (same night as the yogurt).   A few days prior to that, he had LICKED a paint brush while painting!!  No idea why he did that, took us completely off guard because he hasn't done that since he was a baby.   Any one, or all, of these things could throw these test results.   The GI was calling me on a Friday afternoon, before she was to leave town for a week with this news.   So, we decided it would be best to wait out the week- continue the ProViMin but do nothing else (no paints!) and see what the next weeks test results would be.  It was a rough week, giving him something we weren't sure if it was safe and that looked to be more and more unsafe but we also talked about doing an endoscopy before taking him off the ProViMin if the blood showed eosinophils again.    Eosinophils in the blood doesn't automatically mean EGID (eosinophilic gastrointestinal disorder) but it could and I don't want to miss this "snapshot" of his body telling us something.   We waited until the next Monday (when the GI doctor was back) to do the labs.  That morning, middle of the night, I gave him a bottle with an increased dose of ProViMin- timed so that it would be about 6hrs. before the lab draw....to see if the snapshot could tell us something, anything.    

The increased ProViMIn did not spike his eosinophils again, those returned to normal levels in his blood.   So, the spike of labs in that direction seemed more likely from the butter, yogurt, pork or paints- not from the ProViMin, so that was a relief.    But, this blood showed a drop in Neutrophils (white blood cells that during an acute reaction elevate but during a chronic reaction for him, they decline- like his body is fighting something and depleted (chronically).  He's even been on neutropenic precautions at the hospital before when he's chronically reacting (because these levels look like a little boy susceptible to every illness- a body fighting hard to keep those illnesses out that it depletes it's stores, chronically).  His hemoglobin did pick back up a bit but his iron stores continue to drop and his protein stores are not picking back up.   The blood in his stool samples continues to worsen but the inflammatory markers are improved.   The GI and I agree that this is allergic colitis; that although it is easier to manage than enteropathy or enterocolitis (in comparison to the two), it is not something we can continue to subject him too- it will only continue to get worse; and any "hit" to his system will flare it so significantly, he will end up needing a blood transfusion.  We need to find a new path, a different path that he can thrive on....but where does that path lie?    

TPN is the answer from the GI.   New food challenges and time is ours.  Which one will be the solution, we are not sure.   

TPN would meet his nutrient needs, but TPN is not free of his allergens (corn and soy).  We could potentially leave the soy out (that is in the lipids-the fats), since he takes a diet orally- we can give him fats orally (which he gets in hemp milk and safflower oil); but we can not leave the glucose/dextrose out- it is the "rider" for delivery of the nutrients.  What does a corn allergic person do?  I'm not sure.  Maybe we will need to explore that, but first- we owe him to try a few other things....

But his allergy is a GI allergy and shouldn't be affected by the TPN ingredients, right?   Well, the whole time he was on TPN, he had villi damage....so his gut was getting "hit".   Also, he regressed, significantly.   I saw it happening while he was on TPN but I noticed it even more dramatically once he came off.  He has underlying sensory issues that come out (significantly) when he is reacting a food.   Well, on TPN they stayed around all day long and his body couldn't cope with it.  He began to be very specific in his needs, we had to keep the house dark, he didn't want anyone touching him, his language had halted and was starting to go backwards....everything was in a heightened state interfering with his quality of life, and ours.  It's not the ideal situation.  However, he did gain weight, his hair grew, he expanded his developments (even though his sensory issues kept him from practicing them, they were there).  I know that if his body can not absorb nutrients any other way, we will have to do it - we just need to know that is the right option.

Labs follow up

When we saw our pediatrician a few weeks ago, we had decided that the Hemoglobin of 8 (normal is 12) was cause to watch things closely.   That is until 2 days later and the Ferritin (iron) levels came back at an all-time low for him, at 2 (normal is above 24, but he's rarely been that high ever). So, our GI changed the plan and we went ahead with the IV iron infusion as he was symptomatic and we really wanted him to feel better and stop that cycle (while praying the new ProViMin formula was doing it's job - not only restoring his levels but not reacting to it causing him to dump the proteins (and malabsorb vitamins/minerals).  The re-check was this Thursday- a week after getting the IV iron and 2 weeks after his last check.   

And it was GOOD news!!  His hemoglobin is now up to 11!! From 8 and within a week- this is a good sign that the IV iron helped and hopefully that his body isn't reacting to the ProViMin.   His Ferritin came up a whopping 17points and is now 19!!!  WOW!  Although this could be a "false" rise as it will appear higher right after IV iron therapy.  I am SO glad we got the IV iron for him, despite the week + symptoms he has been having from the corn in the dextran the iron is delivered with, his hemoglobin is up- he is feeling better, appetite is improved, and sleep is improved. Although he is still tiring easily and having mood swings (usually only when he is overtired and a lot more likely in the evening) that are not at his "baseline"....so I am still playing the guessing game to what is causing that.  I don't know that it is related to food; for one thing- we aren't doing any big trials....except the ProViMin.  

The other thing that I am worried about is another lab that we got checked, from an appointment we had a few weeks ago with the geneticist - a recheck of his carnitine.   He is low again but not low enough to trigger a clear deficiency so we haven't gotten a call back but I am wondering what his symptoms now correlate with this lab value....and what we should do about it.  The geneticist did say he wanted to supplement it (although admitted it may be difficult finding one that is corn free) because he fears he just isn't getting it in his diet.   I'm not sure why the formula wouldn't have it,but know his hemp milk doesn't (carnitine comes from meat and dairy sources).  We still haven't gotten to the bottom of this and it is either simple (not getting the source) or it is complex (a metabolic disruption disorder).  For now, we need to concentrate on finding a safe source of carnitine for supplementation as carnitine has a role in gut health.  

Something isn't quite right, just not sure what it is or if we are moving in the right direction with each day or getting further away....we need some follow up's with GI and the geneticist and he is (over) due for his 3yr.well child visit.  But, I just want to enjoy being at home and summer starting and spending time as a family, and celebrating his brothes 5th birthday, and going camping....and FOOD trials, and not dealing with medical issues and appointments and more lab draws and interfering food trials for supplemental trials.  So, I guess I've been playing Ostrich a bit.   Maybe tomorrow I'll start to make some calls....for now we're going with the flow- taking time to cuddle and lay low when he needs it and making the most of when he is feeling better. 

IronMan

Little man got his IV iron infusion 2days after his 3rd birthday at the PITC (Pediatric Infusion Therapy Center).  It is a 6hr.infusion because the iron has to be infused with a sugar- sucrose or dextran.  We've tried the sucrose but that has given him bloody stools.   FPIES is defined as a delayed food allergy of the gastrointestinal tract, so doctors often struggle that allergens via IV or on the skin, or even airborne, could cause reactions.   We've seen them all when he is exposed to corn.  It makes me wonder if he will ever outgrow his corn allergy.....  We keep learning as we grow with it, on ways he is "corned" (as it is called in the corn allergic communities).     Did you know that many corn allergic can never go to a movie?  or shop at Target?  Or walk through the detergent isle in the stores?  The strong corn smell is too much and induces an allergic reaction.   I think I've seen these in him too....my instinct tells me I have....but I'm still trapped in that "that is crazy" feeling fighting my instinct.   This past weekend, hubby bought some scented laundry detergent- which I have been avoiding but didn't tell him that- Little man's color was very pale that evening as the house was engulfed in fumes from the laundry scents.  Today, his color was off again - he hasn't eaten anything new- but hubby used some strong cleaner on some stubborn spots on the living room floor and also on the kitchen sink.     

Now, most of these reactions don't completely interfere with his quality of life and can go almost undetected (hubby doesn't notice them like I do); but it is hard to differentiate if the symptoms are from a new food we are trialing or being "corned" so we have to be careful --- even if it isn't going through his gut. 

So, the IV iron sucrose is a 45minute infusion...would be so much easier if he could have that but it is not safe and the benefits do not outweigh the risks.   The IV iron dextran produces symptoms but they are manageable and the benefits of the much needed iron outweigh the risks of corn-tamination from the IV dextran.  But it's been a long week....working that dextran out of his system is taking a long time.  He isn't sleeping well (won't even sleep in his bed), has stomach aches, doesn't want to eat but will take his bottles (thank goodness), is tired a lot- achy maybe? touchy and moody.    Over the week, we played the guessing game- did he get something, is it from this or that, is this getting worse or better, is his iron still critically low?  But now it is clearer to see that it was all from the dextran and I'm feeling de-ja-voi, that we've been here before and even discussed it with the GI who agreed that we need to try and avoid the IV iron (ie, not let his iron stores get so low so that he needs it).   But with a Ferritin of 2 (normal is 24 and up), we had no choice unless we did a blood transfusion.  

We got to the PITC and Little Man knew exactly where we were....and wanted to go home.   He was very brave though and got distracted by watching "Cars" while we waited for the lab to show up to get his IV started.   We were happy to see one of our favorite nurses there too.  We were giving her an update on how he has been (they love to see how much he has grown and changed!).  She was talking to him and trying to distract him as they got the IV ready and he had his SuperMan pants on (pants with a patch) and she asked if he was Superman.  I told her how one day, he looked at me and said "Sam Ironman, me Ironman?" ;)   She thought that was cute too...and replied...."well, today he is".   

The day was long but hubby and I split the "shift"- I take the first hours where he gets the IV and gets settled in and then hubby takes the rest, where they can play and nap and get through the long 6hr.infusion.

Anemia....again

Little man has been hovering over his spiral for a few weeks now and helping him on our own has been just out of our grasp- right at our fingertips but not enough for us to get a good hold on it.  

This past weekend, he was really showing signs that his anemia was back and his Gtube was really hurting him.   So, by Sunday we decided we needed to call in right away Monday morning and get him evaluated further- for both anemia and Gtube issues (not that they are directly related). 

We call and leave messages with the GI and try to get in to see if his Pediatrician on Monday.  There are no appointments available but he can see him on Wednesday morning and in the meantime, the GI ordered for him to have an Gtube study (barium contrast upper GI) and some lab work to assess his anemia.    So, Tuesday afternoon was spent at these appointments.  

We got some of the results that evening, not good.....Hemoglobin (normal is above 11) is back down to 8.   Not good (down from 12 a few months ago).   It explains a lot though, and was what I expected - as he has been tired and had disturbed sleep and now lately, his lips are the color of his cheeks, which is not good because his cheeks are not rosy.   

We go to the Pediatrician appointment on Wednesday morning and he assesses that yes, his skin/lips/etc are  pale but his heart rate is good and his activity level right then in the office was good.   So we decided that at this time, it was worth trying to see if the ProViMin would  help him catch up and replenish his stores.  We scheduled a follow up lab check for 2weeks and said we'd be in touch.  The other good thing about his appointment was that his weight is UP!  For the first time in months, his weight is UP!   Alimentum RTF formula did nothing for his weight and just a week on ProViMin and he's gained a pound a half! He is now 31#8oz.    We all agree that we don't want to see him HAVE to get another blood transfusion and would prefer to avoid IV iron infusions as well.   IV iron is infused with a sugar - dextrin....which comes from corn.  It doesn't go through his gut but his body still reacts to it- so we would rather avoid it....if we can.   

Later that afternoon, the rest of his labs came back- another lab they checked was his Ferritin, which is the iron stores in his blood- we needed to find out if his hemoglobin was dropping because of blood in his stool/losses alone or if his stores were also affected because the last time they were checked, they were already low.   Now, they are very,very low.   We are going to have to intervene and not wait to see if the ProViMin will restore his stores quickly enough.....realizing that his iron stores are so low, we know we will need to do something more aggressive to help him.  

His GI called this morning and confirmed those are her thoughts and recommendations, so now we are waiting for her to connect with the Pediatrician and come up with a new plan (his plan yesterday before the Ferritin levels were back were to do a wait-and-see approach (to which we agreed to).   But now realizing that his iron stores are so low, we know we will need to do something more aggressive to help him.  

The GI doctors also mentioned that she is really upset that he is bottoming out again, that she doesn't understand why he didn't tolerate the Alimentum RTF- or why he "seemed" to tolerate it for a few weeks and then didn't.   I clarified to her that I don't feel he ever did tolerate it, we just wanted him to so badly so we kept pushing it -- and that he CAN be stable if we didn't have to keep pushing foods that his body is not ready to accept- full blown FPIES or not, he goes backwards faster if we push him when he shows us signs that he isn't tolerating something- if we push him just to get to vomit because that is the only evidenced based signs.  IF the ProViMin continues to work out, I don't want to push him - I want to just take things slowly because he should be getting all he needs in the ProViMin and if he needs gut rest or time to heal or a food that is not tolerated taken out of his diet- then we need to listen to his body.  Without a source of complete nutrition, we have not had that luxury.

The GI starts talking about long term hospital admissions, extensive tests and consults at other facilities with other more FPIES familiar GI doctors....she admits she is frustrated and losing her patience and needs to just know if his gut is use-able or not, or if he needs long term TPN to thrive....   

These thoughts overwhelm me and we ask all prayers that Sam will tolerate the IV iron infusion and that his body will have the catch up it needs and he can thrive on the ProViMin, and we can take things slower.   

Oh, and the tube?  Both the Ped and GI do not want to discuss taking it out yet....even though surgery has said that is their recommendation.   One good thing is that it has stopped oozing and draining and we noticed a significant correlation with the Alimentum feeds and intolerance to the increase of the gtube drainage.   

Trying not to get too discouraged....or, too excited?!

Today was some of the same symptoms as yesterday but not a build in symptoms; actually if anything- they were less. I am still confused by the symptoms at all, if this is going to be a pass but I am so encouraged that things didn't build. Maybe what we are seeing is left over from the Alimentum he had this weekend? It wouldn't be the first time a trial got muddied by being too close to a fail or just from a food that caused symptoms.
 He got 3 doses today (2tsp at a time, in 3 different bottles throughout the day). His symptoms to note are: crankiness when waking this morning and from nap....but I have to remind myself that is not abnormal for him- he really struggles with this transition and I really wish I knew why.
 But the good news, other than that- he was his normal mild-natured self all day. His cheeks aren't as rosy/chapped looking but it is waxing and waning still a bit. The concerning symptom of the blue coloring (cyanosis) around his lips and eyes is still there- there was a point today I just couldn't get over how terrible he looked.....but he was acting fine! He took a good nap, and he has fallen asleep easily tonight. His bowel movements have been great (yesterday and today)- no mucus, no blood, no increase or liquidy diapers, no diaper rash, no allergy ring; but there has been hiccups- twice. I know, seems silly right? Well, he's gotten hiccups before a fail before. He's also gotten hiccups randomly just like anyone else so who knows if this is a warning sign or not.
 Either way, we press on. We press on encouraged because his appetite was good today- he ate his millet "cake" for lunch, and he had snacks through the day and willingly drank his bottles, and had dinner even! I made mashed potatoes and he LOVED them! I mixed them with his safe oil (Safflower) and his hemp milk. He ate a few tablespoons, and then he followed that with gobbling down 3 mini millet muffins and a whole cup of peach juice (peach puree + water = his safe juice). He then had a bedtime snack of 6 mini muffins. 

The next day started out well, actually really-really well. The two little boys played SO well together! And then as the late morning wore on, we started seeing him crumbling...little things were upsetting him, he wanted mommy or daddy right next to him, he was getting obsessed over things (he has odd obsession behaviors when he isn't feeling good), he had 2 bowel movements - that although they didn't have any reactionary look to them, 2 in a day is out of his norm, plus that "allergy ring" was showing and some spots around his diaper area. What is this about? I actually didn't go right to the ProViMin in my head....

This is where it gets really hard....when you start seeing symptoms but you can't be 100% sure if they correlate with the trial or from something else....accidental exposure, cross contamination, etc. My head went to the Potato Stix he had eaten that morning. We initially found Pic-Nix potato stix at a Fleet Farm on our way up north last weekend. Everyone was getting a treat and I picked it up and read the ingredients: potato and cottonseed or palm, or safflower oil, and sea salt. Safflower oil is his SAFE oil! Right under the ingredients it states that it is manufactured on a dedicated line- no line cross contamination risk! The risk with it though is we're not sure if cottonseed or palm are safe....and actually have had suspicions that they might not be (from other foods with them). But, I took the risk anyway...we're traveling, he's not eating.anything. on the drive....I wanted him to have something to re-stimulate his appetite and was excited to give him a snack, just like this brothers were getting. I took the risk, for him. He ate the entire can over the course of the weekend, no symptoms to note. On the way home, we didn't go the same way but discussed that maybe we should have - since that can was seemingly safe, we should stock up!! Although we do have a Fleet Farm down the road from our house. I ran some errands earlier this week and decided to get some more, from our local Fleet Farm. I brought them home and he wanted some, took a few bites and then didn't want to eat them. Huh, well- maybe his "jag" is done and now that we're home, he wants something else "new"....or maybe it is a different batch with a not-so-safe oil and he can tell? My radar goes up a little. But, he has been asking for his "star cake" so I've been making that everyday (millet muffins made with a small star silicon pan). With the addition of pure maple syrup and ground CheeCha puffs (for potato starch/flour), the texture holds up better than his mini muffins ever did and he's gobbling them up- by the fork-ful! I'm amazed at how well this little boy is using a fork and spoon!

Ok, this is getting long so I better get to the point! Yesterday, he asked for his "potato chips" first thing in the morning. I hesitated- thinking of how he hasn't wanted them and what if they contain a unsafe ingredient and we're in the middle of the ProViMin trial,... but he has been wanting to eat before his morning bottle and I don't want to discourage that! So, I let him eat some- he didn't eat many and then he wanted his bottle, we decided to up the dose of the ProViMin yesterday- he has been SO pale and he needs the nutrients so his body can make iron if we have any hopes of not having to have another IV Iron infusion or blood transfusion. He took his 8am bottle and his 10:30am bottle well and was playing well all morning and then he had a full lunch of peaches, a potato pancake, 4 mini millet muffins, and some more potato stix. After lunch, he took another bottle and he started to get really crabby, we hoped he was just tired and an hour later he took another bottle and finally went to sleep. He did fine all evening, although his appetite was decreased and he was a little clingy to daddy. We got home late and he went to sleep easily and then he woke up around 3am SCREAMING!!! Yelling and screaming and thrashing and then hitting and kicking and screaming! He woke up everybody in the house, he was just crazy!! It's 3am!! We finally got him calmed down but he stayed awake for at least another hour. The next morning he was very crabby all morning- everything causing tears...all out of sorts. He seemed to be getting better as the afternoon wears on, so I am still hoping my instincts are right and that it was the potato stix and that will just have to work it's way out of his system (the can went into the garbage!) and not the increased ProViMin (went from 2tsp/bottle to 3tsp/bottle- upping his intake to 1/4cup/day (end goal being 1cup/day of ProViMin). I am also worried about his anemia and fear we have to face it and bring him in for an evaluation soon- he is so pale and has been for at least 2 weeks now, and this disturbed sleep (sleeping but not acting rested and then being up in the middle of the night) are familiar anemia symptoms. Also, his tube looks terrible!! I'm ready for it to just be taken out...we can't use it and he's now complaining again of it hurting during the day, and who can blame him? It's so red and inflamed, it looks like it is going to push itself out!! Time to address it. Surgery has given their recommendation - to pull it out, that it is causing more pain and adverse symptoms than it is helping (for sure!). His doctors are not sure if we are there yet with it and still hope to utilize it. We are just not sure. We continue to pray that the ProViMin will work out.

Transfusion

Even with the decision made to stop these push trials, little man was still having vomit sessions (sunflower suspicions) and now he’s falling fast- his all too familiar spiral...a sick little boy…

So, we get the labs done on Tuesday morning and we go in for the weight check Wednesday morning- along with a blood pressure and heart rate check because the lab draw reveals his hemoglobin is now down to 7.1.   It is protocol for a hemoglobin of 7 to be evaluated for a blood transfusion….we didn’t catch it soon enough for an IV iron and now he’s falling too far, too fast…we've been here before, why are we here again?

It was a rough week.  We’re nervous that he isn’t sleeping well, he is eating very poorly, and he spends most of the day in the chair (mommy holding), he’s very pale.  He’s also running low grade fevers. …something isn’t right...this isn’t supposed to be happening again, we were supposed to be watching him closely through these trials; but our pediatrician wanted to slow down his lab checks to 2x/mo. instead of weekly.
   

A blood transfusion was necessary, and although I am very, very sad that we have to be here again – him being so sick, feeling so miserable and exposing him to blood products- I was anxious for him to feel better.

He is doing so much better since the transfusion!  Saturday was a rough day, we were at the infusion center from 1:30 until 6:30pm but he did really, really well.  His energy level picked up and his cheeks pinked up.  He was happy to be home and feeling good.   The following days,  he played and played and played- it is dramatic the difference….kids are so amazing.    Daddy took him to the Nature Center just to get OUT (when he’s sick, he doesn’t want to go anywhere or do anything- so lots of sitting and rocking and movie watching).  He doesn’t regress when he’s sick but he sorta goes in shell- and it’s like a veil is lifted when he’s well again- it is SO fun to see!  He plays a lot of catch up between illness setbacks.  He amazes me all the time.

Busy week

Well, we've been home from the hospital for a week now (seems longer- it's always busy trying to catch back up after being in the hospital!).


Last Sunday, we went to the pumpkin patch, Little man was having a rough sort of day- he enjoyed playing but was tiring easily. We suspected it was from not getting his TPN the night before. Big mommy and daddy mistake, part of being in the hospital for a week- no milk in the fridge for the other boys’ breakfast; and no tubing for his TPN administration. We hadn’t realized we were short! We had the TPN bags, but the wrong tubing was sent! It was a weekend, we didn’t expect that anyone would be able to get us the tubing we needed until Monday, so we upped his formula intake (of course) but we were unsure how that was going to work out for him.

By Monday morning, it was becoming more clear that something wasn’t working out for him- but now we had a few things in the differential. A water bottle on Friday, found to have “gunk” in the sip valve; missed TPN for 2 nights, a line that wouldn’t flush the antibiotics through and we had to “push” it through (potentially putting bacteria into his system- not enough for full sepsis but…), or was worse yet- was he reacting to something like his new brand of hemp protein powder. Ah, the fun times of FPIES guessing games. A delayed food allergy, you have to look at a minimum of a 24hr.window. It gets muddy sometimes!

He had labs drawn on Monday, and my concerns grew as his labs confirmed that something wasn’t right. I continued to watch him through the next day- I was concerned that he may be heading downhill. I contacted his doctors and decided to continue to watch him through the day, and get his Thursday labs done on Wednesday to help assess- and make a plan if needed.

Thankfully, by Wednesday he was showing improvements and his labs confirmed this. We still don’t know for sure what caused this, but just thankful it was short-lived and his body re-cooped quickly. Thursday and Friday, he began to be more and more himself. He is still getting IV antibiotics 2x/day (morning and night). This will continue until the 26th; and then we will get cultures to see if the long treatment of intense antibiotics has worked to rid his blood of the bacteria. Good signs are that he is looking good, acting well, and his line that had redness around it now does not. I pray this treatment has worked.

One other thing noticed in his lab work is that his hemoglobin, that was dropping last week, has started to climb up….for the first time on its own- the numbers are going UP! I’m puzzled by this, an am remembering some research I have done on “iron loving bacteria” and wondering if that was happening to him. Well, now he is on IV antibiotics and his hemoglobin is going up- on its own. Coincidental? Maybe. Maybe it is a silver lining amongst this sepsis cloud. Everything does happen for a reason, that I do know.

The next step is to start some probiotics. Antibiotics kill bacteria in the body- good and bad. It may be helpful if it is killing some negative bacteria that is iron-loving; but regardless, his body still needs the good bacteria. Probiotics are found to be helpful during antibiotic treatments. Maybe this is the time when we get ahead of his gut dysbiosis that we know has compromised his gut health and complicated his FPIES?

So much to blog, so little time!

We've had a busy summer.   The house has a "lived-in" look, the pool is getting good use, the kids have tan lines, there is always someone opening and shutting a door, there is constant hustle and bustle....and I love it.    My boys love it too, my eldest has asked if he can be home-schooled because he thinks summer vacation routine is pretty nice!

We've gone from Catechism (church school) classes to a lake vacation, back home again for swimming lessons and then a surprise birthday weekend for "Papa", now back home and Nature Camp week.  Next week starts summer vacation.   What? Wait? Didn't it start 2 months ago?  Doesn't school start in a few weeks?   Yes, we did it to ourselves.   We've kept our older boys busy and involved.   And they are thoroughly enjoying it. 

Little man, well- some things just never seem to change.   We keep cycling through anemia, and the effects that come from that.   He had the blood transfusion and really perked and pinked up at the Lake the following week (as I previously blogged about), and he really seemed to be doing well otherwise.  A few symptoms here and there, and without the anemia- we would've just written them off to other things.   But there is the anemia.  

We just do not know where it comes from.  The Hematologist we saw in consult last fall feels it is a combination of things: chronic inflammation, low iron in the diet (which isn't the case), and poor absorption/utilization of it.   I dont' know how to describe it, except to say it is my instinct but she is wrong and there is more to it.    I wrote to Dr.J (Immunologist at the PCRCD) and inquired if she saw any patterns with protein intolerant kids and anemia.  She replied back that she does not necessarily note that; and then offers some speculation that, with Little Man, it could be something related to macrophage activation or something of this nature.  Something to rule out.   The hematologist is not interested in our outside opinions.   

So, here we sit- monitoring iron and hemoglobin levels closely.  The last time they fell, his hemoglobin went from being within normal limits to dangerously low, requiring blood transfusion- it fell 5 points in 6 week (from Mid march to beginning of June).   In that time, he had reactions to probiotic strains, reaction to banana, a mystery reaction possibly related to cross contamination or banana, reaction to blueberry, and also to elk.   With reaction, I refer to a variable spectrum of symptoms but each time he had colitis (blood in his stool) for 1-2days after.    What is causing this?  Are these losses enough to explain the sudden decline in hemoglobin and iron stores?   Is there something connected to the immune/inflammatory response that affects the iron availability or absorption in the body? 

Today, we had his CBC (complete blood count) checked.  Hemoglobin June 4: 10.5g/dl, June 14: 10.6g/dl, today 10.7g/dl.   He's maintaining, which of course I am grateful, but I don't understand it, more to research....

Decision to scope....

With Little man falling into severe anemia in such a shorttime frame (March-June), we returned from vacation (July) ready to investigate.   A lab draw revealed a declining pre-albumin, combined with the iron stores we were already watching decline, as well as no weight gain in months, and Little Man's history of villous atrophy, we were highly suscipious of something again.  

But the main concern is his severe and persistent anemia.   Dr.J (PCRCD) has speculated that he may have a macrophage activation causing his body to chew through his iron.  It sounds like a logical place to investigate but our current hematologist isn't interested.   The feeling is that he simply does not get enough iron in his diet, combined with chronic inflammation and bleeding- he has iron deficiency anemia. Period. No further work up needed or necessary.   This simply doesn't add up to me, and my instinct tells me there is more going on.   We need a new consult, we need them to look further; so we decide to proceed with the scopes (upper endoscopy and colonscopy) to rule out the GI involvement for the low iron and persistent anemia.

Our thoughts are that we need this to help further investigate the anemia, if we find inflammation or villous atrophy than we need to evaluate that and we need to consider that is the source of his anemia. But, on the flip side, if he doesn't have any inflammation, we can be cleared to do a food trial! Eggs?? I found corn/soy feed free chickens!

Little man has gone multiple scopes and each one teaches something more about his course of illness.  I agonize over putting him under yet again, for yet another scope; but his FPIES mechanisms are so atypical and we are losing direction on what to next- because of the recurring anemia.  We can't keep giving him blood transfusions!

We proceeded with the scope....

Phases of a 2yr.old or stages of FPIES?

FPIES or 2? Again, my thoughts are put to pen by a fellow FPIES mom.   These two little boys and our families share many commonalities and, as it seems, we have yet another.   These words, her thoughts,in this blog post: FPIES or 2? ...."When do I worry and when do I let it go? When do I correct the behavior and when do I cuddle it? These are behaviors that are not normal for [him].  We saw a lot of these as a result of internal pain that he wasn’t able to express"  hit so close to home. 

It is hard for me to see little man acting out or being cranky without associating it with something from his FPIES.   Something that does help me (other than being with him 24/7) is to keep logs/journal/notes- whatever you want to call it.  I take note of things that I suspect may be out of the ordinary, even if it could just be 2yr.old tantrum.  Then, I know if it starts to show in a pattern- I can look for that.  It helps a little....when I have time to review the logs! 

Little man has primarily a very mild natured, even tempered, take-a-lot-of-hits-before-he-loses his patience, kind of personality.   A personality I knew was there as an infant...hidden underneath all the pain....I knew he was in there....this is the little boy that I waited to come back to us.   And now, we have hit the temperament and independent, coupled with frustrations of two....and we walk a fine line of phases of 2 or stages of FPIES? 

Thrown in for some extra added confusion in his "atypical FPIES" is the mix of Anemia...and we didn't catch it soon enough, will we next time?  And why is he still struggling with such severe and persistent anemia?   I have been polling other FPIES families among different support boards, and there does seem to be a commonality of anemia happening.   Thankfully, most not as severe as little man's.   There must be something connecting this commonality among FPIES kids as well.   Dr.Jyonouchi, whom we consulted with at the Pediatric Center for Rare and Complex Diseases has a theory on it, and we're hoping to consult with a new hematologist about that soon.   But, first, we need to rule out additional intestinal involvement- does he have villous atrophy or inflammation that would not allow his body to absorb the iron?  Does he have active colitis, or something more involved in the colon that he is having acute blood loss?   Or is the plant protein based iron in hemp not bio-available enough for absorption in his body?  Many questions to answer and I feel like we're in a race to answer them before we land with needing transfusions again.   We sit and try to assess current symptoms, are they related, are they developing into a pattern?  Do they look the same as his previous patterns of inflammation/villous atrophy/severe and persistent anemia? 

• His cheeks are still somewhat pink, he still has his tanline from vacation. 
• His mood is all over the place, but he is two.  
• His energy is up and down, mostly down. He wants to be held and carried more every day.
• His sleep is only a little disturbed, but when he does wake up he is MAD and it is difficult to console him back to sleep.  
• His diapers have a "glossy" look to them but there is no blood detected (tested for 6days last week). 
• He is gaining height but has not gained weight in over a month, or longer.
• His appetite is up and down, but we still manage to get in ~1100 calories/day, in a 24hr.period.
• All of this we could do the "wait and see" approach but that approach has never ended in good results in the past when things start adding up like this; but mostly we can not because of the severe and persistent anemia. 

Something is still not right, but what is it?  What is going on?  How do we get to the bottom of this?  IS there a bottom?   

From Pale to Pink!

It took a few days but the effects of the transfusion combined with the sun at the lake and Little Man went from pasty and pale to pink and perfect! We don't notice it as much day to day but.....such a difference.....

The transfusion brought up his hemoglobin, and he was able to be an active toddler for a good solid week. Transfusions don't do much for iron stores and his are very low, so it shouldn't come as a surprise as I start to catch the symptoms of a declining hemoglobin again.    Fatigue, he loves to play outside, wants to play outside but his fatigue keeps him watching from the window, or asking to be held or ride in his stroller (around the yard!).   Today I caught him licking on wood again...it is a sign of his PICA when his iron deficiency is rearing it's ugly head.  It has barely been three weeks!  But then he will have hours where he is happily playing- with no indications of fatigue or a chronic illness.   So, am I reading in too much?  Or am I catching the early signs because we've been here before?  

His color still looks good, he's not pale and pasty....yet.    We will get labs and weight check again tomorrow and keep looking for clues for the next steps....stay tuned as I attempt to keep things updated here!

Transfusion....again

It was last October when Little Man's hemoglobin dropped so severely that he needed a blood transfusion to recover. A quick glance back of that time can be read: here.

Little man had 2 IV iron infusions, but his hemoglobin barely moved and was still very low when we rechecked it.   Iron stores remained low as well.   Why is this happening?  It is becoming more evident that he needs a full anemia work-up (re work-up) but not this week as we were planning on leaving town for a few days.   A discussion with the GI and we know he needs more treatment, he is going to need another transfusion. His iron stores got too low again and his body simply can not make enough hemoglobin or make it fast enough with the stores he has.   We decide it is best for him to get another blood transfusion.  My heart sinks and I melt.  I can't help it, I am crying....I ache for my little man.   He has to endure so much.  Is it something I missed?  Something I did, something I allowed him to have that is making him decline again?   I don't even know and can not think about it.  No time for a full melt down, he needs my strength.   And we need to get to the infusion center so they can begin the preparations for his transfusion....

I pack some bottles and some toys, blanket and off we go.   We get to the Pediatric Infusion Therapy Center (PITC) at the hospital and Little man already is unhappy with where we are going.  This has been too much- needle sticks, lab draws, IV infusions...too many this week already.   They call for the IV team, and lab draw - "difficult stick" she tells the technician on the call.   They arrive and get to work- assessing veins.  Veins that are hiding and mad.  Surprisingly to all, he is not as bruised as one would expect him to be, given his severe anemia (one of anemia tell-tale symptoms is bruising).  The think they have one in his left arm...poke- nope, it jumped.   Get out, try the right arm (little man is getting difficult to hold down), poke- nope, it deflated. The blood is thick and not coming.   They need a lab draw before his transfusion to type and cross his blood, the line is not accessible.   The lab RN is ready to "call it" (sometimes they will call the Peds trauma team to get a difficult stick).  A short discussion on what I would like them to do.   Is going home and pretending all of this isn't really happening an option?   No, no it's not.  I'm holding a tired, anemic, growing little boy who needs adequate hemoglobin to have ample oxygen being supplied to his brain.  "What about his heels?" I ask, cringing at the thought.  "Will you let us try a heel?" the RN asks kindly.   "f you can use a heal for the transfusion, and can get a vein...then yes- please try", as I remove his socks and shoes.   Immediately they found a viable vein.  Poke, bingo.   Now, a new problem is securing a line in a heel of a 2yr.old who wants this stuff "off?!".   We manage to secure it and distract him, and then wait for a few hours while they type and cross and then prepare his blood.  The transfusion takes 3hrs, it's ordered for 2hrs but even the technician that comes to run it acknowledges that might be fast for a little guy with his sensitivities.   We did 4hrs. last fall and he tolerated it well and responded well to it so we decide on 3hrs....it's late in the afternoon already and it's been a long day.   We're tired....and we're leaving the next day for vacation!

"I am SO Two!"

Reads his t-shirt, handed down from his oldest brother, worn by each with such true meaning.  There is something about being two!

Little man had his 2nd birthday recently, but that is not what this post is about- that will come soon though (as I'm still working on organizing/downloading the pictures...hey, we've been a bit busy!). 

Just a week after his birthday, and we are finding ourselves sinking into anemia traps again.  His hemoglobin seems to have spiraled down quickly.  The lab draw reveals a Hemoglobin of 6.1 and Ferritin stores are quite low again as well.  There isn't enough iron being absorbed to make iron rich hemoglobin with.  All that we know right now is that it is a combination effect of:
1). colitis flares with food trials as evidenced by blood in diapers for 1-2days following small ingestion's of foods, or triggers (sucrose in IV iron 6 weeks ago) and unknown days of microscopic blood not visible. 
2).  Not enough Vit.C to absorb the plant based source of iron (despite his hemp supplying almost 3x what his body needs)
3).  Missing micronutrients (B vitamins) to synthesize iron.
4). Acute inflammation following reactions
4). Gut flora involvement??

Peaches supply Vit.C but he doesn't eat them consistently enough to get enough of it from them.   We just started B vitamins and it looks as if he is tolerating them well (we are 1 week in!); so once we restore his iron levels - will the B vitamin supplementation to his vegetarian diet be enough?   We can only hope. 

But right now, the question remained if he needed a blood transfusion or could proceed to straight IV iron infusion.  And for that, he needs to be evaluated.   We say good-bye to Daddy....which he is not happy about and head to the Pediatric Infusion Therapy Center and await the Physician's Assistant to come and evaluate.  If his heart rate is too slow, or too rapid- they will need to do a blood transfusion before they do iron infusion.  

It takes 10-15minutes to get from our house to the hospital parking....he's been screaming the whole way- more like yelling.  As if he is saying "I know this drill and I do NOT like it....you don't take me with when daddy is home unless it's to the doctor, take me back home, take me back to my daddy".   He kept trying to wipe his tears, they were just an annoyance getting in the way and he was trying to tell me something.    There was a point, as we pulled into the parking garage that I just wanted to turn around and go home.  What would be the point in his if he is going to be screaming like this?  But, no- he needs this, he needs to be evaluated and he needs treatment.   Wasn't he just so tired a few hours ago that I was carrying him around the house?  Where is the energy to have this tantrum coming from? 

He continues to carry on, stopping for a split second to pout his lip as we enter the parking garage.  I breath a sigh of relief- maybe he's resigned and we can walk into the hospital without them thinking I am hurting my child!?   Nope, he takes a breath and begins again.   We go up to the infusion center with little breaks from the objections.    The nurse tries to take vitals, which only makes him more upset and clearly accurate assessment of his vitals is not going to be possible in this state.   The physician assistant comes in to exam him and the tantrum escalates....does he even remember what his tantrum is about?  No, but he knows exactly where we are at.  

I have witnessed tantrums to this caliber before but not from him.   He is adamant that no one is coming near him, and he barely will let me hang on to him- he is not happy with me either.   I called his daddy and said "you've got to come, he won't calm down, he needs you".   I knew he would be happy enough to see his daddy that he would at least calm down and catch a breath so we could better assess things.   In the meantime, the PA and I discuss his symptoms and observe the energy it is taking for him to have this tantrum.  It is decided that we can hold off on a blood transfusion and proceed with the IV iron.   But it is already 3pm in the afternoon, the IV dextran takes 6hrs. to infuse and the Infusion Center closes at 4pm. 

They ordered IV Iron Sucrose.   The one he got SO-SO sick off of, the one no one understands why he reacts to....a corn sugar injected into his veins where T cells have to encounter it at some point?  I don't know why it is hard to understand but I do know what we see happen to our little man.   I wait until his daddy arrives a few minutes later and ask if he would want Little Man to receive the IV iron sucrose.   I barely finished my sentence, and he says "NO!....absolutely no way is he getting IV iron sucrose, we will not put him through that again- he was so sick".     The decision is noted and understood, we will return in the morning for the 6hr. IV iron dextran infusion.  

We all go home exhausted.

Tired of Anemia

....tired FROM anemia.   I thought it was the heat., then maybe it was daddy's extra shifts at work that was making him cranky.   But then his sleep started to get more disturbed, and his appetite was declining for everything but wood.   And then he didn't have the energy to walk up the stairs, or play outside with his brothers, and his lips were the color of his pale cheeks, it seemed to spiral quickly...or the days smashed into another, or I didn't monitor my journal close enough to see the building symptoms that were becoming more clear familiarity of anemia.

We had discussed a lab check with the pediatrician at our 2yr.well child just a few weeks ago; but we decided to hold off- unless/until we saw symptoms.  Well, with all of these symptoms, it would have to be done sooner than our planned 6week follow up.   A lab draw right away on Friday morning revealed a Hemoglobin of 6.1 (when normal is 11-14 for this age).   Sigh, tears, sigh....phone calls and messages to his doctors (our clinic has an online patient portal for checking labs and sending messages, it works well for the needs of chronic illness) and it is decided he needs to be evaluated right away, to check in at the Pediatric infusion center where he will receive appropriate treatment.   A blood transfusion may be necessary if he has a too low, or too high heart rate. 

Little man has already had a long morning, an IV stick for a lab draw....correction, 2 IV sticks for a lab draw- he has tricky veins.   We've learned enough about his veins and draws that if they stick him and can't get blood immediately, to just pull out and try another site.   Some lab techs do not appreciate being told how to do their job, because that is what they are doing- their job.   But it is my job to remind them that this is a little boy, and he also happens to be a little boy who has to endure many IV sticks - we do not need to make them more traumatizing then it needs to be.   We have stood by and watched them dig and poke and prod around under his skin looking for that vein that "was just right there" enough to know that once it jumps- there is no finding it.  Get out, find a different vein.   So, we switch arms, and positions and this vein comes right away.  They get the blood they need while Little Man screams in objection.  He doesn't like to be distracted, he doesn't like to be told "it's ok"- he just wants it done and he does not take his eyes off the needle until it is done.     They bandage him up and he sits up.  Tears done.  He is such a trooper.  He looks over my shoulder at the tackle box that carries the prizes, "quack, quack?" he questions, he knows he is due for another rubber duckie.  He picks out what must be his 30th duckie by now, and we head out.  He stops me half way out the door because we passed by the stickers.   I try to tell him he already got a duckie, he doesn't need a sticker.   He quickly informs me that he does need a sticker, he picks out a puppy one and has to have it stuck on his shirt right away  - it is his badge of courage, and he wears it proud.   "Bye?" he says now.  And we go home to wait for the test results and decisions on treatment....

Two Year Well Child

Little Man's birthday is next week, so we got an appointment scheduled and completed yesterday.   First on the agenda: weight check.  Little man is now 24#8oz. and 34" tall.   He is in the 19th%tile for height and 12th%tile for weight.   Holding his own curve, although it looks like a bouncing ball all around the 10th%tile line- he does stay around 10th%tile.   One the growth chart, it is more about the curve than about the number.   A pound or two at this age is all that separates from one end of the curve to another.   At his highest (at 8# at birth, he was 57th%tile, he hovered in the 40th%tiles for those first 6mo, and then took a plunge from Nov.2009 to January 2009, this is after we tried soy for the first time and when began to multiply quickly, except his weight.   He maintained his own curve again until after corn fail when he hit a 5%tile low, not dipping that low again until after soy challenge fall of last year.   The patterns present themselves when you look at it all laid out on the growth chart graph.  I follow his weights not because I want to obsess over every ounce but because the patterns of intakes and outputs can tell us a lot about what is going on in his gut- healing or inflammation? Absorbing nutrients or malabsorbing them?  

Height taken, it is off somewhere because his height curve is a bouncing ball too....that doesn't make sense.  People don't shrink like that.  But there are a few weights like that too- done in off-pediatrics floor appointments, where I want to just say "don't record that, you didn't do it right anyway and his growth chart is enough of a mountain range".   He is much shorter than his brothers at this age, after all their daddy is 6'3".  But maybe he simply will take after his French ancestors (my dad), so then he's right on track.  

The nurse enters his height and weight in the computer, and prints me the growth chart. She hands the paper to me and Little Man grabs for it, takes it and "reads" it- as if he is studying it.  This gives the nurse a good chuckle.   He does this a lot with books at home as well, full intent into the book- as if he is reading it.   I admit, it is super cute.   The nurse is surprised that he does that a lot.  I comment he does it with writing too- uses a pen/pencil with paper and will look and draw as if with much intent.   She asks to see him do it, so I hand him a pen and he promptly takes it and starts to "draw"- complete with tongue sticking out!  It was priceless and made her day.   "See you later" he says to the nurse, her part is done and in comes the pediatrician.

Quick discussions about his weight gain, and current measurements.  He is growing proportionately, and appropriately at this time, and that is encouraging.  Next is his development.  I check off on a "scoring" sheet for things he should be doing at this age.   He "scores" at 2yrs.3-6mo. on everything which is good that he's meeting base milestones but I already knew that.  It continues to amaze me that he isn't further behind, with everything he goes through.   That said, I don't agree that he is in the 2yrs.3-6mo. milestones category, not if the category's were written with his brothers milestones in mind.   But I do feel the things he may be a few steps behind on, he will catch up.  Here again, he's following his own curve and developing appropriately.  We discuss his speech specifically, I am a little concerned, or more like watchful, that his speech has "exploded".  There is a point with toddlers where they go from a few dozen words to so many you can't count.   Little man has his few dozen words, uses them well, learns a new one every day or every few days (depending on how he's feeling) but he hasn't exploded his language yet.  I'm not overly concerned, just watchful.  The pediatrician feels the same- not over concerned but agrees to be watchful, if we haven't noticed his "explosion" of language within the next 4-6mo., we can move for further evaluations.   I agree, this is a good plan.   The pediatrician adds that he feels little man is just a calm natured little boy, and will talk when he has something good to say.   He is probably right. And he is right that little man is calm natured, we've been getting a lot of comments on that lately and his daddy and I can't help but to think of the first year of his life when we were told it was his "temperament" that made him so fussy and non-sleeping, and crying all the time.   I know, in my heart and soul that Little man was a calm spirited boy- hiding underneath all that pain.  

We wrap it up with discussions of his current diet, current goals for expanding his diet, a peek at his eczema that popped back up after sucrose reaction and hasn't completely quieted down yet.  A complete exam, for which little man is so cooperative for- he has this doctor visit thing down!

And then an anemia discussion.  Last check was one month ago, do we feel he needs to be checked today?  No.....although he had a strong bloody diaper after blueberry (I will spare you the picture I took!), and has started some wood chewing again and has been more pasty looking some days, we had 3 days of Elk going strong, maybe we can replenish his stores with real food and avoid the offset he gets from IV Iron infusions?   The Pediatrician agrees we can do a wait and see, being that his physical exam looks good except his pale skin and somewhat pale in his lower eye lid.   We plan a weight and lab draw for ~6weeks from now, unless something else comes up in between.  Having a plan is good. 

Now, on to planning that birthday party!!

Anemia

Little man has always had low hemoglobin, the first blood work we did (around 6mo.) showed a low hemoglobin and low Vit.D.  It was one of my confirmations of the intestinal inflammation I suspected he had, that matched his ever-growing symptoms.   A change from breast milk to elemental formula where we could be assured he was getting the iron his body needed, but his hemoglobin continued to teeter and his iron stores continued to fall.   Iron is needed for the body to make hemoglobin.  We got him started on some oral iron pills- even compounded to avoid his allergens (corn), initially he seemed to respond to them and we had hopes it would do the job- slowly but safely.   He had a lot of weird symptoms at that time but we attributed to iron upsetting a sensitive stomach and resigned that it would be part of the side affects he would have to deal with....to avoid a blood transfusion.   A few weeks later and he was no longer responding (even reacting to) his iron pills, his iron stores were severely depleted and his hemoglobin dropped severely low.   By the end of October, he was admitted for a blood transfusion....a very scary time for us.  He responded well to the blood transfusion and his hemoglobin raised nicely; however it did little for his iron stores and within a few weeks, he was quickly declining again.   After the soy challenge, it went very low.    That is when IV iron was decided to be the best course for him, along with IV nutrition.    That was almost 4mo.ago.   We have been checking his blood labs frequently, with two checks in a row revealing a wonderful hemoglobin within normal limits.   We last checked March 15.   We had a follow up appointment on April 15 with our GI doctor, it was time to check his labs again....he was chewing on wood, he was fatiguing easily, his appetite was declining, he had had accidental ingestion reactions, he was spiraling....the blood work came back and we were all surprised to find a low hemoglobin once again (8.8)!  Back to IV iron....


Why are his iron stores always so low?   Is it his diet?   From the outside looking in, it would be an easy culprit to blame; but a quick glance at all his lab data and you can see his iron stores progressively declining from 6mo.until he got TPN (IV nutrition with IV iron)- even through getting 5+mo. of elemental formula.  IV iron has been the only thing to bring his levels back to where they should be.   His DRI (daily recommended intakes) for his age is around 7g.- he gets around 15g/day! He is missing vital B vitamins (gets some but not all) so essentially he is a 22mo.old vegetarian. The typical recommendation for a vegetarian is to double up the protein sources to assure adequate iron intakes since non-heme (plant) sources of iron are not absorbed as readily by the body as heme (meat) sources are. So, we're doing that and still he is unable to maintain his iron.


His gut is not absorbing his iron enough- regardless if his diet is complete in it or not.    But why?    

http://www.loveyourbelly.com/resources/gut.html
"people with damaged gut flora often have particular groups of pathogenic bacteria growing in their gut, including iron-loving bacteria (Actinomyces spp., Mycobacterium spp., pathogenic strains of E.Coli, Corynebacterium spp. an...d many others). These bacteria consume whatever iron the the diet provides, leaving that person deficient in iron. Unfortunately, supplementing iron only makes these bacteria grow stronger and does not remedy anaemia. To treat anaemia, the person requires all the nutrients we have mentioned, many of which healthy gut flora supply"  
I found this reference recently, while trying to read more about some information I ran across a few months ago that would explain his anemia more....have found this connection interesting.
I've also since learned that beneficial bacteria actually synthesize/produce B vitamins (which helps the body with iron).  

He has continued blood in his stools, from what we are told is from a fissure (superficial, bright red), his diet is double his iron needs for his age but from plant source, his diet is low in B vitamins, and Vit.C, he has dysbiosis, and I am now really thinking the above reasoning (gut flora) is behind this anemia.    We see in his blood work data from Dr.Jyonouchi that his gut dysbiosis is behind/at the root of his complexity of responses.   

Stool samples are sent, probiotics are on board (trying to find the right "fit"), B vitamins are ordered and will be "trialed".....I continue to fight this fight for him.  But I'm not working fast enough....

Bringing him in last week to get an IV placed for the iron to be administered was rough, emotionally and physically exhausting.    He is so little, and he goes through so much.....I just can't seem to research enough, there are not enough hours in the day to get far enough ahead of his condition to know what next step to take.   Someday, the doctors will know more about FPIES, someday treatment protocols will begin sooner and infants and toddlers do not need to suffer these co-factoring illnesses on top of what FPIES already is.

Hematology follow up

Little man has went through oral iron supplements- responded well too and then did not respond at all, and then actually seemed to have a reaction to (a new formulation Rx), hemoglobin levels dropped to 5 in October- blood transfusion necessary.  Hemoglobin rebounded to 10.5 following blood transfusions, wonderful!  Continued oral iron supplements, and a mere 1mo.later, hemoglobin back down to 6 (following a soy-challenge and discovery of villous atrophy).  Hematology consult, with recommendations for IV iron therapy for 10treatments (2x/week for 5 weeks).  That's a lot of IV sticks, PICC line placed instead and given parental nutrition along with iron therapy via the PICC line for 4 weeks, followed by 1 more week of IV iron.  

The last treatment was one week ago, a lab draw a few days later revealed a hemoglobin of 10.8!  His body responded well to the IV iron therapy and rebuilt his iron stores and red blood cells.   Anemia will always be a component of his diagnosis and he will receive IV iron periodically until we can get him past the curve on his tiny menu to some more iron-rich food sources (ie- MEAT!).   

With the news of the restored hemoglobin levels, we were finally able to remove the PICC.    Little man had the same PICC line for 38days, we kept it clean and intact and NO infections!  Whew! And it is now out - he got a treat last night, and then one just for the heck of it this morning- a full-soak-up-to-his-chest- BATH!  A no-soap bath, but still, playing in water, soaking bath is a treat.   We had given him a few baths over the course of the weeks, but with an arm wrapped in plastic, it was more of a chore than a relaxing activity.   Sponge baths were easier.  

We will do lab checks every 2 weeks for the next few months, as well as IV iron therapy once a month for the next 3 months.  We will not let any slippery slope land us back to a PICC line again.....we hope, and pray.  

Now, onto food trials....soon....

"Atypical" FPIES

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

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

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

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

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

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

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

Pink cheeks

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

How is Little Man?

I wish I could answer this and know the answer I gave was accurate.  But I do not know.   His anemia is keeping him laying low most days.  We will likely get his levels checked again sometime this week to check on it.   I'm not sure if this is what is causing all his random off baseline things we have been seeing, or if it is from food trials - although we see this even if he hasn't had food that day as we are taking it super-super slow with the food trials.   We stopped millet- it was looking non-FPIES but may have had some digestion issues but seemed better with the cooked porridge but then he didn't want to eat it.   So we stopped, took a few days off and then started his plum trial.  Plums are more questionable than millet was!  But he likes them better!   I want to be able to call them both a pass -so I can put the plums in the millet and flavor it, giving him something fun and tasty to eat that is also adding to his nutrition!  I am trying to be patient.  Patience is what I hear from the other moms, slow but sure and watching closely.   Also "met" a mom with a 5yr.old little boy with FPIES online this weekend; she has great wisdom to share....sharing that using the most liquid/puree form of the food and then working the way up as tolerated -- and slowly!  And if there is a digestion issue, than stop the food.   FPIES or not, if he can not tolerate it, he can't tolerate it.  I agree- no child should have to have so much pain just from eating such small amounts of food.    The question is: is it from the food trials?   Is it from teething?  Is it from the iron?  Is it from the anemia?  I believe the disturbed sleep is from the iron or the anemia;  I think the decreased appetite is from the anemia - he takes an average of 700cal/day- at least 300 less than what he needs to gain weight!  Although he expends a lot less energy. We are frequently carrying him around, he plays well on his own but he doesn't run as much as he did a month or so ago.  I imagine he is just tired.  I don't like seeing him like this but the benefit still does not outweigh the risk for a transfusion, but I will be glad when he beats this and he can feel better and run and play more.    We took him in for a weight check, as we do this often throughout his illness.  It has been one way to monitor his reactions and how long it takes for him to heal, and if he is eating enough- calorically, or absorbing enough nutrients.   The good news is he hasn't lost!  Even with 2weeks of eating 300 less calories than his needs;  he hasn't gained much but has a tiny bit....and he hasn't lost!
Today, he woke up stuffy and with a low grade fever, remained "clingy" all day and then threw up this afternoon.   We have no idea what caused it.  It wasn't violent so whatever it was it was only "mild".  He felt better after his nap.    What is causing all this?   How do we proceed when we are barely moving and we are getting all these mysterious signs/symptoms.   Can we attribute it all to his anemia?   What if he is slowly building towards a reaction?  What if he is having blood in stools again?   His last positive stool samples, we couldn't even visually see the blood- it was microscopic.  
We see GI on Tuesday, an Allergist (this will make #4 for Allergists, #1-no FPIES, #2, absolutely no doubt FPIES, #3- confirms FPIES, #4 - ?).  We need this 4th Allergist because he is the one actually in the clinic where the rest of his team is.  We only ask that he be open minded, and willing to learn so he can help Little Man. 
We will proceed with food trials tomorrow, I think....