A rash + progressive weakness?

I have noticed for about 2 weeks now that he is weak- I thought it was related to anemia as he's had this kind of weakness before; although I was puzzled how he was maintaining his color- but thought it was just from the sun. And then after his CBC check at the time of his scope showed a stable hemoglobin- I started to really wonder what this fatigue could be from; but just thought it was from his body requiring extra calories to heal. Then this rash started and the weakness was really showing but thought it was from being sick,...

Monday was the doctor appt, with the labs to rule out viral infectious sources, tests came back on Tuesday clear...and Tuesday was a busy day and I wanted to observe him more....I was really noticing his fatigue, and started to realize he was weak, he joined the boys outside Tuesday morning while I was hanging out laundry and I watched him walk- tenderly- across the lawn...he was walking slowly- as if he had to think about each step; but he was determined to play with his brothers, He tired easily. I noticed when I put him down, he wasn't wanting to (or able to?) bear weight on his legs. My instinct is clawing at me....but I can't pinpoint what is going on.

I am so challenged by the continued challenges. NOT by the challenge itself, but by the constant place I am in...it feels like I'm being negative or dramatic. I am not a half-glass empty person, and do not want to send half-glass empty messages- with doctors, with family, with friends. I have no control over this, none. The more I want to, the more I don't. I hear God's message- I just struggle with it. Humility. I do not know what is going on with him, I have to ask for help. I have to plead for help from doctors who may not be able to, or want to help. Humility. I share our experiences to help share our story, to bring a voice to the atypical FPIES so many kids also share, to support and be supported in a new family connected through an isolating diagnosis.

I struggle...do I call the doctors? What symptom am I telling them? Am I just seeing this or is this 'just' his normal fatigue? What am I asking for? What do I need?

Today, we started out the day much in the usual ways, and I notice changing Sam's diaper that his legs seem weak, progressively from yesterday. When he finished his bottle, and got down to play- he clung to the bed, and then he grabbed for a chair, and then walked along the wall before he ventured out on his own- wobbly and shaky. This is getting really concerning. 
I called and the nurses agreed he needed to be seen.  After making the appt for later in the afternoon, I discuss his symptoms with a few more moms, and share the video of his walking, and discuss further with Little Man's dad (who is at work).  We decide to head to the ER, not really sure what to expect.  A nurse and Resident MD come in to do their assessment and vitals at the same time and then the Pediatrician on call comes in to do hers. She is very pleasant and she confirms my concern, not in a scary way but in a reassuring way- that my instincts to bring him in are on..."too many symptoms and can't pinpoint it except to say, something is wrong". She orders lab work, an IV placement and a some further tests (lumbar puncture).

Lab work, sure. IV placement, good. Lumbar Puncture? Ok, now I am getting really scared and I'm here alone with our 4 boys. I called Little man's daddy with the update, he knows he needs to ask to leave work and I confirm that by saying I don't think I can do a lumbar puncture with him alone, with the other boys here. The IV placement was difficult enough...the boys were in the room- the team had gotten there and was moving so fast, I wasn't thinking about having them step out, and I had a movie on for them to watch and at first they didn't notice the IV team coming in and then little man started screaming and W was squirming and climbing the walls and doing everything he could to not cry or reach out to his littlest brother.  So hard to watch him go through this. I, struggling to hold back my own tears that I am always able to hold back, give him an assignment of finding his brothers some stickers- right outside the room. The other 2 boys now notice the commotion- realizing their brother isn't just normal fussing.  J is frozen and crying...I ask him to leave, he can't move. With the help of one of the nurses, Thomas leaves to go get stickers- basically still oblivious to what is happening with Sam (thank goodness because Sam is sitting on my lap- my full attention needed to stay on him).  Yeah, an IV placement- they can't be anywhere near the room for the lumbar puncture!
We get the IV placed but it is clear we needed our tag team. Jeremy's co-workers are understanding of his need to leave and he is soon with us at the ER, arriving just in time for the LP....his turn to be with Little Man and this time I got the boys out before any commotion began....we're going to get a snack. They gave Little man some sedation and the LP went perfectly, and is over shortly after we get back from getting the cafeteria. I have never seen him so loopy! The sedation drug is one for pain and sedating so he doesn't move during the procedure. We have to wait until he is stable and then we move to a room upstairs.  He needs to be admitted for further observation and tests.

Tag Team

Little Man was in the hospital for a week, with the efforts of his daddy and I - we were able to maintain some sort of order on the home front because we have such a system down by now.   All part of learning to cope with a child with a chronic illness -having a plan in place when he needs to be in the hospital to maintain order in the other children's lives.  His brothers are very well aware that he is in the hospital and that either me or daddy are there with him (while the other one is home with them) but they have needs too and this can't be forgotten.   Maintaining routines and order for them helps them cope better through it.   It also helps when family is able to come and help out, so his daddy and I can both be at the hospital or have an extra set of hands, and entertainment to 3 energetic boys at home.   (Thanks again Aunty K!)

We had a system, we coped through it; but we were glad to all be home under the same roof again.   Friday night was uneventful, little man even slept fairly well back in his own bed.  We continued to imagine how nice it will be to have soy as a "non-trigger".   I made a fresh batch of formula, 1/2 Hemp milk and 1/2 soy milk (with the fortifiers of safflower oil, hemp protein powder and arrowroot starch) for Saturday morning and the morning went well.  I also restarted his iron supplement he has been taking since mid-September.  The pediatrician at the hospital explained he would still need his daily iron, even though he had just gotten "loaded up" with red blood cells because otherwise his body would not make more red blood cells because it would think it could take a much deserved vacation!  So, I wanted to get that restarted right away.   He played all Saturday morning and went down for a nap at his normal time.   He awoke from his nap screaming and clearly upset.  I picked him up and immediately could tell he had a fever.  I took his temperature and sure enough, a fever.  Clearly he was uncomfortable so I went to get his ibuprofen (this is compounded for him to be corn free).  We had taken it with us to the hospital and it wasn't unpacked yet, however I had a new bottle of a prescription I had filled a few weeks ago (to have on hand instead of waiting until we ran out because compounding can take a few days).   So, I went to open the capsule (it comes in a powder, sealed in a capsule that we break open and mix with water, using a syringe to give it to him) and the powder came out orange.   Now, his iron that we got from Walgreens at the same time (second week of Oct.or so) also has been orange but I assumed that was because it was elemental iron and this suppliers was orange....but now, why would this ibuprofen be orange if it is just ibuprofen and calcium carbonate?  The other one wasn't orange.   He was screaming, so I gave it to him and then immediately thought- "why did you do that, you don't know what the orange is"!  I immediately called Walgreens and was relieved when the kind pharmacist that worked so well with us in the beginning with his compounding answered.   She looked up the information on this particular prescription and sure enough, they had added aluminum/red dye #40 to it!!  This is used as a "lake" and it helps keep the medication compounded.  They had wanted to put it in his original prescription compounds -- I remember because it was one of the many times they called to double check to see if they could add this ingredient or that.  I have been wanting to steer clear of food dyes.  Little Man's daddy was allergic to food dye as a young kid and his older brother has some issues with them as well, and I had observed in the past that he may also have these same issues.  I don't know if they would be FPIES related or not- I think just more of an intolerance/typical allergy.   But the pharmacist that did this newer refill did not know that, did not think to check because I had not spoken directly to him/her to discuss his food allergies and we do not have food dye's listed on his chart for allergies.  So, we have been giving him red food dye for 2-3 weeks in his iron.   Could this be the reason his hemoglobin feel from 7 to 5 without us noticing?  Could this be the reason he was having problems sleeping (hyperactivity is a symptom of a food dye intolerance) despite being so over tired?

It might be, but it could also be a build reaction to soy.    Saturday we stopped any medications with food dye but continued soy.   Saturday night was disturbed sleep, Sunday brought more fevers, more crying, more needing to be held all day, and a loose-soak-into-and-thru-blow-out-mucous diaper.    There is fiber in his soy milk, so maybe it is because of this increase in fiber in his diet.   Maybe he is teething, and sometimes stool can be a little more runny from that.  Maybe it was the red food dye reaction.  I was starting to get nervous about the soy....

Sunday night brought on less sleep (for everyone).  Monday morning, he was still running fevers so I put a call into the pediatrician to let them know how he had been doing and get advisement on if he should be seen and evaluated.   We weren't as worried about the fever itself but what it was doing to his body and how far it would go before he "snapped" out of it.   With his history of such prolonged and rough "falls" after a reaction, we began to worry.    I gave him his "safe" iron dose and within 30min he was screaming, screaming, screaming.  Inconsolable crying, agitation, mottling, drooling, hunched over-legs drawn up stomach pain, screaming.  I left a message for the GI doctor as he was now crossing over into concerning symptoms that she wanted to know about if he started to have.   I waited for return phone calls while trying to analyze what was going on and what next steps I need to do.   My mind automatically goes to next steps...what can I do?  What can I do to stop his pain, or at least ease it some?  What can I do to make sure this doesn't happen again?  What plan can I make for the other kids if I need to go to the ER if he should start vomiting?  Who can help me decipher through these symptoms?  So, yes- my anxiety was building.   I do my best to not let it show to Little man, he picks up on it so easily.  So, I hold him and hug him and kiss him and dance in the kitchen, and watch Baby Einstein, and try to get him to eat, and see if he wants to play...nothing is distracting him from his pain and the more I try, the more upset he is getting -- almost as if he was mad at me (maybe he is, maybe he is mad that I gave him the things that are making him now hurt).  I called his daddy at work to let him know the assessment of the morning.  He knew by my tone that things were not good, that things were all-too-familiar from previous reactions.   He came home immediately, his assessment- the same as mine....clearly reacting to something.  So, either red dye from Saturday (when his fevers and agitation started) or a build up to soy.    The good news is, Little man calmed down some for daddy....as long as he didn't try and put him down.   So, he cared for him while I dumped out all the soy formula and made a new batch of "safe" hemp milk formula.   Little man had already been starting to refuse to eat - is it the soy?  He will typically refuse something that he correlates with giving him a tummy ache.  Now he is mistrusting his hemp milk formula (something I've been very afraid of as I look for fortifiers for it).   He has a large blow out diaper and we decide he is going to need to be seen, even if our pediatrician hasn't called us back yet.  So, we see someone completely new.   All we wanted to know is if his fevers were related to a viral illness or not so we could continue to assess the situation and what we needed to do next.   She checked his ears, nose and throat and everything was clear.  There were no signs of a viral illness- although that doesn't mean there isn't one.   She wanted to help more, so she orders a hemmocult screen for his next stool per his daddy's request/insistence--which he kindly gave us a sample for when he got home from the appointment. 

Tuesday morning and the GI doctor called with results from the stool sample....positive.  With the symptoms since Saturday and a positive stool sample- she wanted him readmitted right away.  I agreed that I was worried about him but since he had not stooled yet that day, and seemed to be "holding" - we were trying to be optimistic that he would remain holding.....because Tuesday was his biggest brother's birthday (again, we try to maintain a quality of life not only for little man but for the whole family, through his illness).   GI doctor said to make sure to have little brother hug big brother-birthday boy because he was saving him from a hospital admission that day.   We were to keep her posted, if he continued to do ok- we could continue working on getting him back to a baseline at home before proceeding to next steps of a vitamin trial and follow up with her at our appt later in the week.   Our optimism didn't keep little man from going downhill that afternoon....

We turned in another stool sample that the GI doctor wanted in follow up to the positive blood one; and we did some blood work.   He had another rough night and clearly wasn't getting better,only taking in half the amount he is supposed to for formula for 3days now, and making less wet diapers and his lips were dry....our concern for him was growing.   So, this morning we called the GI doctor again and asked her to call us back right away.  She got the message and called us back, we reviewed the symptoms and all agreed he would need to be readmitted for monitoring to try and re-establish his baseline before proceeding with a vitamin trial and a re-trial of soy.

I am home now, tag team in effect again.   I needed to come home to make formula so we agreed that daddy would stay at the hospital with little man tonight....although he did ask today if this formula is something he can learn to make.  He can....but it's kinda like making candy - it is all in the feel of the stir....it is a delicate process and I have made a few batches that went down the drain. So, we'll save that for another day and for now- I am home to make the formula and be with the other boys.  Little man is at the hospital, with Daddy, and being observed....maybe today was the "peak" and he will do better overnight and tomorrow but if not- he is where he needs to be and I can rest my mind, even if it is just for tonight.  

Home from the hospital.

Little man, happy to be home from the hospital. He misses his brothers too much when he is there; and they miss him.

I haven't updated in a few days because we've been so busy, and worried, worried and busy- but mostly worried.

We've been working towards a baseline with Little Man, we moved away from Neocate and began to see what he looks/acts like when not in pain all day and all night long. Then, we tweaked his 'new' formula (the one I make in my kitchen with hemp milk and arrowroot starch) by adding hemp protein powder and we found not only a pain free little boy but a content little boy. The end of August, he was doing so well. The past few weeks he started to slide- he was cutting his 1yr molars so was a little fussy/whiny -but no where near the magnitude of his GI pain, so we were dealing with it day by day like any other "normal" teething kid. Then, he caught a cold his brothers were passing around- which brought him down a bit. Of course, all of this made me nervous- is this a build reaction or just "normal" 15mo. old everyday things? Gah! How crazy to be nervous over such little benign things! We pressed on, he continued to get millet puffs daily- he enjoys playing with them more than anything and we have intentions to do a full trial soon. I added the protein powder. Then we find out his hemoglobin/hemotacrit is falling- and his iron stores are already low. His iron has always been low- we (me) assume it is part of his malabsorption he had for so many months while on foods that were triggering his inflammation response. The hemp milk does not have ideal amounts of iron in it, and the iron it does is non-heme (meaning not from blood- so vegetarian). I have a 15mo. old on a vegetarian diet. He needs double the iron and good sources of Vit.C- both of which he does not get.

Walgreens was so helpful in compounding his pain medications, now it was time to move the possibility of a vitamin supplement to the top of the list. I had asked them over a month ago and they could not find a way to make a multivitamin compounded with the vitamins but none of his allergens. I spoke to them again, and we decided to just to an iron and vit.C supplement- both of which come in powder form so they knew they could compound this. We waited a week, their supplier was out of FeSo4 (elemental iron); they kindly suggested we try another pharmacy in town. We took it to the hospital pharmacy, it took a few days for them to order the FeSo4 and assemble the compound and then a day to let it "set" and finally, last Wednesday it was ready. I gave little man his first dose as soon as we got home from picking it up. I thought he had good energy right away, but then he had trouble sleeping that night. I attributed it to an off schedule day and more teething. The next day, I had to work so little man was home with his daddy- who called mid day to say he was on his 3rd nap for the day. Odd, could this be his body's way of resting and breathing. One could hope, right? Thursday night he had more disturbed sleep and Friday morning brought the mucous filled, reaction smelled, diarrhea diaper. "He's reacting!" Little man's daddy added, "he had that same diaper yesterday afternoon". Ugh! What? What is he reacting to? The iron supplement? Iron is constipating, why is my little man having diarrhea with mucous? Ugh! Is it the Vit.C? I had called right away and asked them to be sure it was corn free. "It's corn free" they assured me. I didn't think to ask what it was if it wasn't' corn....I will next time. We called Friday to ask them to check what the Vit.C WAS made from, they got back later that day -- tapioca!! Tapioca that he had such violent dumping diarrhea that landed us in the hospital in July? Tapioca, really? Who would have thought?! Or, is it build from millet, or is it build from the protein powder that has Vit.E derived from sunflower oil. Can little man tolerate this? Everything extra, everything we weren't 100% sure on was stopped. No more millet puffs, no more protein powder in his formula, no more Iron/Vit.C supplement. He remained sick all day Friday, we remained nervous and concerned- would he get more sick? Can his body really keep handling this? Saturday he ate less and was tired and cranky, I sent a stool sample that came back positive for blood. Ugh, again. Sunday he was so, so sick- but what do we do? The ER wouldn't' recognize what was going on, their recommendations would probably be something that his body is sensitive to. So, again- we wait it out...moment by moment with him, holding him and watching him closely. Monday morning we call the doctors. The GI doctor wanted his labs done right away, they came back by noon and revealed a very low hemoglobin/hematocrit....he needs to be admitted.

Monday was our 13yr. wedding anniversary. What a way to celebrate, a trip to the ER for a hospital admission with a sick, sick little boy. But,these are the times I am so very grateful for my husbands strength. He was at work but he came home immediately and took Little Man to the ER while I stayed home and made some more formula and packed (hey, at least we got to be together for our anniversary...sort of). Thankfully, by the time we get to the ER- Little Man had a good nap and was perked up. They agreed he could wait until he was admitted for a possible transfusion- but that he needed continued monitoring for signs/symptoms of anemia.

So, monitoring is what we did- while the doctors checked all differentials for his sudden decline in hemoglobin/hematocrit. Little man kindly made us a stool sample the next morning and then blood work was done. Blood work revealed his H/H are stable- so confirming that his body was coping and holding; his chronic anemia has enabled his body to know how to utilize his stores well enough to hold. His stool sample came back negative for blood, the intestinal bleeding has stopped. So, the signs of him having more energy and more smiles and less crankiness with these lab results tell us he may not need the transfusion just yet. We wait to hear the doctors recommendations, while we try to think of the best decision for little man's health. "He will feel better" is the benefit they tell me. This doesn't seem like the benefit outweighs the risk. Do I want him to feel better? Of course!! That pulls at my heart strings like nothing else!! But do I worry that he will have a transfusion reaction? Do I worry even more that someone else's red blood cells in his body will incite his inflammatory responses? YES! Little man's daddy and I talked it over, and we just couldn't justify it. We know that if he drops low again, becomes lethargic and "blah", and a lab draw would reveal a drop to 6 or below- we would have to strongly consider this transfusion, esp if he was containing to lose blood (intestinal). But we are not there yet and Little Man deserves the chance to let the supplemental iron build in his body. Upon that decision, after 24hrs of monitoring to confirm his body is coping ok, Little man was discharged and we headed home. A discussion with our pediatrician today, and a plan is made for his specific needs. We will have to monitor him closely, and food trials are certainly on hold. I have added the protein powder back in, and he has responded well to it. I think not having this extra protein boost (also has iron) for his body did make a significant difference in how his body coped over the weekend. We will keep millet out and we have added tapioca to his list of allergies on his chart. We are hopeful we have identified the "trigger" correctly and he will continue to improve every day back to his baseline (that he had for such a short time).
He is so sensitive, so fragile but at the same time so resilient, so amazing. And he has big shoes to fill.

So quiet....

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

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

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

Hospital....

The last week has been a whirlwind. Little man was admitted to the hospital on Tuesday morning.

The effects from the starch intolerance led him to drink only half of his hemp milk, which was already low in nutrients (mainly carbohydrate source). He was just so sick, we knew it- we just didn't know what to do about it. We trust in our team we have put together for Samuel- his pediatrician, dietitian, allergist, and GI doctor; we brought him in for appts right away Monday morning. I knew he had lost weight, getting a weight at the pediatrician's office confirmed this- now down almost 8oz. since corn fail. He has had lots of periods of no gains, but this is the largest loss he has had. His little body has been struggling so much to catch back up since corn fail.

His pediatrician helped get him a last minute appt with his GI who agreed he needed to be admitted to the hospital to work on his nutrition as well as try and schedule a endoscopy for further evulation of his continued intolerances. She gave us instructions to come to the hospital for admission right away on Tuesday morning. I was still a little concerned to get to Tuesday morning. That night, little man's daddy slept with him, so he could be close in case something should happen...he was so lethargic. He was not dehydrated because he was drinking enough of his hemp milk to stay hydrated. Little man has always been so strong and so resilent through all of this, it was so hard to see him like this- so very, very weak.

Tuesday morning we were admitted to the hospital and the doctors began their assessments. He was given an IV and blood tests were taken to assess his current state. His blood sugar was very low (in the 40s)!! We were of course concerned, but not surprised as this would explain his weak/floppy that was getting worse with each day. His body had was going into acidosis, which meant that he was burning muscle to make glucose to feed his brain- not good. Little man was so, so sick; we were glad to finally be in a place to get him help. IV's were started and his blood sugar picked right back up to an acceptable level (90s); he began to perk up as well. He continued to eat just plain Hemp milk until he was made NPO in preparation for his procedure the next day. The GI doctors wanted to do his upper endscopy and lower sigmoidoscopy right away, so this was scheduled for Wednesday mid-morning. Little man was given a bowel prep of Miralax....only it didn't work. He was in SO much pain most of the night (only getting barely 3hrs of sleep all night long). We finally had to give him an enema to "clear him out", which worked nicely (finally). The procedure went well and multiple biopsy's were taken to help further evaluate Little Man's continued intolerances.

We kept him on hemp milk for the rest of that day, which he drank like a champ. It was then discussed and decided the next morning to re-introduce the Neocate to assure his brain gets the nutrition it needs (avoiding the low blood sugar). This is where it is very helpful to have kept logs on Little man's eating and symptoms. We had started to see significant improvements in little man when we had started to move away from Neocate. We seem to have found a thresh hold were we saw little symptoms when he kept his Neocate intake to less than 20oz./day. I had noticed that when he had 24oz., he would have random throwing up and discomfort. So, we decided this would be a safe place to go back to to get him thriving again. This was started on Thursday afternoon. He has been drinking it well and doing primarily well on it.

Test results are coming back in, biopsy's have revealed that his intestinal inflammation is not changed, and may be worse, than it was on last scope 5mo. ago- this after 7mo.on elemental nutrition. It doesn't make sense, unless you consider that he has been reacting to the trace proteins in Neocate as we suspect. The GI doctors agree that it doesn't make sense, but we differ on the why. They ordered more tests to be done, testing for an auto-immune enteritis. Good news is if they find this, it can be treated with steroids to get the inflammation to finally subside and we can begin to introduce foods. The bad news, this is an auto-immune disorder - from infancy.

This is the course of FPIES, ruling out other possibility's while you treat the intolerances. It is a difficult road.

Little Man will stay in the hospital for the weekend, and Monday will bring more decisions and next steps to take.

As for the rest of the family- we are holding on. The 3 older boys are doing well, they understand that their little brother is sick and needs the extra care right now. We try to keep their routine as "normal" as we can so one of us stays home with them while one of us is at the hosptial; with the exception of when we have help to come stay with us. This week, that came in the form of my sister. The boys always enjoy visits from their Aunt, she knows their routine and their needs and that gives them much comfort and stability in an unknown circumstances.

We remain thankful for the continued support from family and friends....it gives us much comfort to have prayers for Sam's health and our strength for endurance being sent our way. We have a deep Faith that allows us to put our worries in God's hands.