Fluids help the body cope with a sepsis reaction, they are part of the treatment for it- the hydration helps keep the body out of shock as the sepsis affects every organ. Little man is on IV fluids every night with his TPN, I really have seen how this has helped his body cope through these reactions the past few weeks. And I am grateful.
Have you ever had an instinct? An instinct that begins as a gnawing...Then grows into a raging burn; a burning instinct that something is wrong...
Your baby continues to get sick from the very foods he is supposed to thrive on. I did. I am a mom of a little boy just diagnosed with FPIES.
And that burning feeling now? Extinguished. My instincts? Stronger than ever. Guiding me, with my faith, as we navigate through the murky waters of our new world created by something called FPIES.
"Faith is not about everything turning out OK; Faith is about being OK no matter how things turn out."
Saturday, December 31, 2011
Fluids help the body cope with a sepsis reaction, they are part of the treatment for it- the hydration helps keep the body out of shock as the sepsis affects every organ. Little man is on IV fluids every night with his TPN, I really have seen how this has helped his body cope through these reactions the past few weeks. And I am grateful.
Wednesday, December 28, 2011
1). Strawberry he ate one, next day had red-rashy cheeks all day. Refused to eat anymore strawberrys. Juiced some one day, coaxed him to take a sip- which he did and then looked at us like we had fed him poison, pushed it away and refused any more. That afternoon he was a complete bear. Enough, he doesn't want them, coaxing him to take some made him miserable. Shelve it.
2). Pork- ate 2 bites, got them down but struggled with the texture, refuses to eat again.
3). Potato- ate only a few nibbles and the following day had significant behavior changes- he has very little enzyme it requires to digest starches so unsure how far we can push this. We're trying again (now) with natural potato chips.
4). Cauliflower- refuses completely
5). Beef- refuses completely (won't even allow the plate to be next to him)?!
6). Pineapple- juiced, 1-2tsp in 6oz. of water and he loves it. No noticeable symptoms on first days! We have continued it and we do see some bloated belly, explosive stools; but feel this is related to his dissacharide deficiency. For now, we will call it a pass! We will rotate it and limit his amounts but feel good that symptoms do not build upon reexposure.
7). Eggs- was enthusatic about, but despite offering in a variety of ways, gets less and less excited about and has building symptoms from less and less nibbles (mostly behavior and sleep). We had been continuing egg all last week, he wasn’t interested in it as much as we had hoped he would be…in the past, for little man, that is a sign that he isn’t feeling well after eating it- symptoms that he can’t communicate to us but maybe include stomach cramps, achiness, mouth/throat itch; all confirmed after about the 8-10th exposure, he wakes up early one morning to fill my kitchen sink with vomit. It wasn't till bile, but it was significant. We don't call anything "FPIES fail" if it isn't to bile/diarrhea/dehydration....would it be so if we continued? Maybe. We're just not going to find out. We'll give it at least a year before we try it again. We're bummed about eggs, we had such high hopes for eggs- and it certainly increased the baking recipes!
8). Coconut - significant behavior symptoms from the flour, maybe it is just the fiber in the flour so I tried oil. I snuck it into one bottle (about 1/4tsp) but the next time I tried to sneak it in he noticed and then refused his bottle. I can't take that chance so tried it another way- coconut ice cream! He doesn't like it! We've tried coconut before- this spring, we got to day 4 with NO symptoms and then day 4 hit and he developed symptoms fast but since they were accompanied with a fever, I knew we had to try again since it could've been something else.
9). Quinoa- made him his "cookies" with quinoa flakes, he tried it one night but won't try it again.
9). What's next on the list? Carrots, kale, try pork again, try beef again, try quinoa again, ???
We need to keep introducing foods, it is helping collecting data for his varied reactions so we can assess if he has something else on top of his FPIES. The behavior symptoms are difficult as they can be from his dissacharide deficiency or his FPIES building. What behavior symptoms do we see that are concerning for FPIES? As the trials progress, he eats less and less but gets further and further away from baseline/ his “normal”- his tantrums increase (and get more irrational, any tiny thing will set him off), he has started hitting (hard!), he throws things (not good!), and he screams! his sleep is disturbed as well, and so is his appetite. He is just not our Little Man, he isn’t like this at baseline, he isn’t like this when we are not trialing foods, what is this beast inside of him? Why can’t food NOURISH him??! I can’t even begin to describe how this feels, to a mom….to not only not be able to nourish him from basic foods, but to have basic nourishing food turn him into this unrecognizable child.
I write this as I am sitting with Little man while he takes his bottle, which I do multiple times a day. My day is planned around it actually. Much like when nursing an infant, I would nurse and then know I had 1-2hrs. before I would need to be in a place where I could nurse them again- a private, quiet place- not shopping or out running errands, or driving; but able to meet their needs. To this day, we still make plans knowing how far we can go between bottles. He will eat while out now, just less- so we don’t have all day or hours on hours to be away from home or he is set back. He eats better if I am sitting with him. Maybe it goes back to our nursing days bond, maybe it’s just simply that I’m mom. Maybe he’s just very routine orientated. But it is what it is, it is what he needs, it is love, and I will love you through this.
Saturday, December 24, 2011
For awhile now, I've had theory's as to what little man needs for HIS plan, An Individualized treatment plan. Taking him down to elemental nutrition enabled us to see what he was still reacting to, corn fail and removing him from the corn syrup in the elemental formula helped to confirm that corn was a chronic FPIES trigger for our little man. Removing that from his diet enabled us to add hemp, safflower, arrowroot and shortly after peaches and millet. That is where we have been stuck. Following his patterns, we learned that our little man has chronic FPIES- that his body doesn't react severely on the first exposure although it does act as a toxin in the body, removing the trigger food, removes the symptoms; but his body struggles to get back to a "baseline" (depending on how far we pushed what was toxin in his body). Why would so many foods be toxic to his body? Food that is meant to nourish not only doesn't nourish him but is toxic to him.
Much of my research, and following little man's symptoms, reactions, baseline, his involvement in Dr.J's research, response to probiotics, anti-fungals, and antibiotics keeps steering me that his gut flora guides his reactions or tolerance. Gut flora is involved in immune mechanisms in the body, I think this is where little man's FPIES is tied. Influence and guide his gut flora, influence his tolerances? When I was building his formula, I didn't trial each ingredient for days on end, we didn't have time to- I added them every few days. He never had any issues with any of these ingredients. Peaches were also an easy pass. Millet puffs were easy, the millet flour was up and down and it did take awhile to settle that it was not going to FPIES but just finding a tolerance level for him. Removing the millet and reintroducing it helped clear that.
Little man is a "build reactor", but his safe foods have been safe from day one. But we've also had foods that look like they could be safe and then they gradually get worse- each dose becomes less and each day symptoms build. So, what if we shorten the the trial? Moving forward before his body builds the reaction, before his gut flora changes enough to induce that response?
The new plan includes just that, trial a new food for 3 days, but not pushing through any building symptoms. If Little man begins to show his body is struggling, we will shelve the food and move ahead; if he is not showing any signs- we move forward introducing new foods. What if he builds a reaction to a food? Well, we don't know that yet. It's been over a year since we have successfully introduced a new food so we need to try this plan....an individualized to Little Man plan.
Wednesday, December 21, 2011
We didn’t get to full FPIES vomit till bile (although we did see vomit);and yet we still had weeks of recovery time- weird diapers with mucus and blood and undigested food, terrible irrational behavior, decreased appetite, not trusting his safe foods, is he reacting to his safe foods?, whining, tantrums….and we stopped before the FPIES vomit. In the past, doctors not familiar with FPIES, or our little man, have previously advising us to push through until we get to the vomit (and not sure if we’d be advised to stop even then). With Little man not on an elemental formula, and missing nutrients in his restricted-unable-to-expand-diet; some doctors are sure any symptoms and reactions we encounter surely do not outweigh his needs for the nutrients those foods would supply. I know, from the outside looking in, it seems quite bizarre. It IS bizarre, that his body would not just reject food, but attack it in the way that it does!! It’s very bizarre. But that doesn’t make it not real, a very scary, exhausting reality that his daddy and I live with day in and day out. Unfortunately, we feel it is poor advice to tell us to push through symptoms that make our little man sick, especially without factoring in his illness complexity. There isn’t many other ways to put it- it is poor advice to not consider his allergic mechanisms when advising us to treat other symptoms. I have explained it this way to people- this is the logic we face….it is like telling someone with celiac disease that they need the vitamins in fortified bread so they must eat the bread, even though it contains the very gluten protein they are allergic to. The allergy isn’t anaphylaxis, so it will be fine. NO! It won’t be fine! A person with celiac ingesting gluten is doing chronic, and often silent, damage to their system. It is vitally important for a person with celiac disease to avoid gluten- in any form and in any contamination of. That is how our little man's body responds to corn, and many other of his triggers. His body suffers internal inflammation, oftentimes inflamed before he shows us overt outward signs (FPIES vomit till bile); so we watch his other signs- other signs that tell us that something is causing inflammation in his system. Will his body be able to cope? Will the inflammation subside? Or will his body try to cope, only to exhaust every internal anti-inflammatory mechanism until it is too tired to continue? And then everything starts to go “haywire”. That is what happened with zucchini, and countless other foods we have had to shelve….his little body just gets too tired to continue fighting- so instead of making it, watching as he gets weaker and more off character and unrecognizable, we stop giving that food.
We are now a few weeks past zucchini, ready for the next trials. But what we did learn was that an in hospital stay for a trial isn't going to work for our little man. The symptoms we see and the symptoms doctors are measuring are too different. We are responsible for keeping him safe, keeping him from being sick and increasing his nutrients.....and we remain his voice during these reactions- FPIES or not. Where does that bring us? We need a new plan.....
Wednesday, December 14, 2011
Our FPIES world has been difficult again lately as Little Man now turns 2 1/2 and we were supposed to be "growing out of it" by now....and in many ways I feel we are being pushed back to the beginning. Little Man is thriving on TPN (IV nutrition), and I am so.very.grateful for that. He has gained 6#- we are about to hit 30# now, as the geneticist that we saw last week pointed out, it is encouraging to see that his body knows what to do with calories, calories that his gut does not have to absorb (or malabsorb in his case). It IS good, it is all very good. But he is now becoming dependant on this TPN, he has less interest in even eating- how do we do trials when he only nibbles? He is 2 1/2 and has no positive relationship with food except to give food to his brothers and watch them eat. And I am struck with the "what if". What if he isn't outgrowing this? What if we don't find him safe foods? What if we find him safe foods that he refuses to eat, or doesn't know how to? What if his body begins to reject the TPN? What if there is something more going on that we are missing? What if there is some metabolic disorder that is compounding his FPIES that we haven't tested for yet? What if this new normal is our normal now? What if?
I trust in God, it is the thread keeping my peace....I have inner peace knowing that His plan is being carried out. My worry remains with what part I am playing in that plan. What does He want me to be doing? Am I doing enough?
Sunday, December 11, 2011
Too many times on this journey, we have found ourselves alone on this island. In the beginning, we didn’t know anyone else lived on the island and we didn’t know to look for ways off or invite someone else on for help. Then it became clear that our little man needed more than what we could give him, his illness was becoming out of my scope as a mother. That instinct I had all along that something was wrong, was now a burning fire, and the need to find more bridges to connect our island became the mission. We quickly learned that a clinical diagnosis, was easier to file under “mom not coping” (ie mom is crazy) than to say medical science did not know, understand, much less be able to help. We tried so many times to connect bridges between different islands, but the complexity of little man’s FPIES needed a strong bridge to a specialist….which, after much searching and persistence, we finally located that bridge. We were happy to finally use it and didn’t know if we’d need any other bridges.
Unfortunately, it soon became clear that we were in need of other things for survival on our island so we went in search of other bridges - only to be quickly sent back to our island- our little man has an illness no one wants to touch…..even though he has needs for these bridges to work, they do not. A corn allergy with an allergic symptoms to even an elemental formula is too scary for most to deal with, so it becomes easier to simply send you back to your island, even though this little boy still needs help. It is left up to the parents to find that way, keep searching for another working bridge. Some really, really long bridges are found- but the cost is very high and the journey long to use those bridges; especially when the shorter, closer bridges should work. Last week we found a bridge that other FPIES families have been using, we hope to be able to as well….maybe we don’t have to travel on the long, long bridges; but maybe we still will……
I don't mind the island, I just want off this island long enough to get what is needed for survival….which bridge is that on?
Saturday, December 3, 2011
That is why were supposed to be getting labs throughout the trial. He has a PICC line, enabling us to watch what is happening in his body so that he doesn’t have to go through this additional suffering; so we can match his first signals to what is happening in his body – his body fighting for oral tolerance or his body building to a reaction and each day less and less of the anti-inflammatory mediators are “winning” and more of the inflammatory cascade is taking over.
Through the zucchini trial, each day, he ate less and less and each day his symptoms build more and more. But his stools have evened out from all water/soaked in the diaper, to a thicker consistency but with some significant mucus in some of them. His symptoms are just all over the place. It reminds me of when we did the probiotic, and we pushed through and we got some great results (2# weight gain, increased appetite, happy playing boy) but then he hit a plateau on the probiotics; or he was building an intolerance to one of the inactive ingredients (which I am suspecting more now that he “failed” them after stopping them in August, and then re-introduction of them recently. And then my mind goes to- what if he was building an immune response to the probiotic ingredients all along and that is why he had so many complications this spring/summer? Why he had colitis with each food introduction, why he has had small intestine damage on the scopes, and why he has had persistently severe anemia. It was as if the probiotic was helping him with the gut flora but then hurting him with the intolerance to the extra ingredients. A change in probiotic to one with no added ingredients only made our picture so very messy as his system clearly did not tolerate those. And yet, my mind goes back to those beginning days on probiotics when we learned so much about how his body goes through so many FPIES-ish symptoms but that nothing was building or consistent, and it all evened off and we got great results (even if just for awhile). My mind keeps going back to where my original thoughts were, when he was just an infant….that there is something wrong with his gut flora- that any dramatic change in that alters his system and that too many times, his body can’t cope with those changes; because it doesn’t have the proper support. When he was breastfed, he had the support- again, another catch 22…where the breastmilk was giving him protective effects but it was still upsetting his system- and too much upset just tipped the glass over.
I didn’t WANT to think it is the zucchini itself. So, what is it then? Why the build? Why tolerate for a few days and then crash and roll? Why so many foods causing these symptoms and eventually (build) causing a reaction? Why can’t his body “win”? My gut says it’s his gut….
Is zucchini a full FPIES fail? We didn't get to full FPIES-to-bile vomit so we won't ever know 100% sure but he did have vomit, he did have diarrhea, he did have dehydration. Bottom line, he simply got to sick- from such little amounts of food, that he was increasingly refusing to eat....less and less ingestion causing more and more symptoms:
The first day- he had about 1/2tsp, he had some off symptoms but nothing that i could definitively identify as a reaction symptoms build as they were muddled with him being 2, and in the hospital.
The next day, his body was having some low blood sugars (before we even did the zucchini); and then he went into acidosis (low bicarb and continued low blood sugars) after the zucchini; and had a full blown "episode"- full tantrum where he was irrational and inconsolable, and he was drooling (not ok, not normal); and choking. He took about 45min to calm down (this is a classic type tantrum when his body is struggling). The next morning, his neutrophils were declined and his hemoglobin fell an entire point. All of these labs were written off to be related to something else; so we were encouraged to push forward. Although Sam was not interested in eating any more zucchini. So, we switched to baked in form.
Day 3- he ate only nibbles but seemed to be ok; no repeat of symptoms from the day before and his labs were all re-cooped. It appeared, at this time, that his body was trying to tolerate the food- and winning. We were optimistic. We went home the following morning.
Day 4- he had some "cookies" (zucchini/millet/oil/peaches) at home, did fine but was having some "poop soup" diapers but not really any other symptoms- and was just happy to be home.
Day 5 - things start to get a little muddy, poop soup diaper again, starting to get whiny and cranky off and on.
Day 6- I give him a Tsp of boiled, purred zucchini again- thinking if he was building to a reaction, we would see it from this controlled amount (vs. baked in). He had no concerning or building symptoms that day, he slept well overnight- doing well overall.
Day 7- eating zucchini/millet flour/oil biscuits and loving them- doing well all day; gagged on a piece of zucchini (insisted on holding a piece of raw zucchini I was cutting up to prepare to freeze and bit a teeny-tiny piece off)...or at least I think it was from the zucchini. It was enough to cover his plate but he was fine after. Although he has choked before on foods (due to texture) and not thrown up like this, so….
Day 8- slept well overnight, poops are no longer loose but do have some mucus in them (but that isn't so out of the normal for him that it would alarm me)- was doing well in the morning but started to really get "off" today- lots of whiny/cranky, looks so tired, and pale.
Day 9- eating less and less - of these baked millet/zucchini biscuits, whiny and clingy, more mucus and smell in his poops, seems to be having low blood sugar episodes,....
Day 10- rough day, decreased appetite, decreased wet diapers, looks terrible (purple around eyes), disrupted sleep (crying in sleep a lot- for naps and night). Had some millet/peaches waffles (no zucchini- made biscuits but barely nibbled them)- loved the waffles!
Day 11- same as yesterday but worse. Each day he eats less of the zucchini but gets worse. He is declining. Sleep is disrupted. Mood is terrible, whiny and angry- hitting and sensitive...
Day 12- today is day 12 and he's a mess. There isn’t' anything significant happening in his diapers. He had this weird drooling this morning, put his hand in his mouth, gagged and threw up (caught me off guard as he doesn't do that when he's at a baseline) but don’t' know what to make of it. Feel he had low blood sugar episodes again today. He hasn't played AT ALL today- he's been in bed watching movies all day, or in my arms, or sleeping. He is taking his bottles better today (had been declining for a few days).
Day 13: He slept ok through the night, except when he woke up at 5am, crying, I notice that his diaper is dry- completly dry. He has been hooked up to IV nutrition for ~8hrs, and he has not urinated. I calm him down and we go back to sleep (he does not want to drink anything). When we woke up at 7am, he had soaked through his diaper....something caught up and "clicked"...whew. But that was weird, he is always wet through well before 5am- between drinking 30-40oz./day and getting IV nutrition through the night; he is an overly hydrated little man. So, any signs of dehydration- even temporary, concerns me.
This morning is his weekly lab draw. This lab draw will help me decide if we will continue to challenge zucchini or if we need to move on. If his internal body shows me the signs of struggling that we are seeing externally, we will need to stop. If his body isn't showing any signs of struggling, we will challenge with a full dose today and measure labs 4-6hrs. later and the next morning so we can get a more clear answer.
I keep him hooked up to his IV nutrition until the moment the home health nurse draws the blood. The home health nurse takes a few notes and then takes his blood to the lab to be analyzed and we wait. I am nervous about "challenging" him today with zucchini as he is just a mess. He is whiny and clingy and clearly not feeling well. A few hours later, we get the labs and they reveal a very low neutrophil count (white blood cells)- a pattern for Sam following a reaction that we have observed in the past is this neutropenic look to his white cells. His hemoglobin is also falling again. And, most concerning- his blood sugar is quite low (45). His body is struggling to maintain his blood sugars- and that is while he is getting nutrition infused into his blood stream! His platelets are on the rise but not out of range of normal, although we have never seen them too high since starting TPN- but he gets heperin in his line to keep it from clotting over, so I would imagine we won't get true readings with that; so trends of increasing is what we're watching for. Another lab that we would see if his body was struggling, another one that is in his pattern, is his bicarbonate levels- he becomes clinically dehydrated BEFORE the vomit/diarrhea. I suspect it has something to do with the sepsis state his body is in while "fighting" this reaction. His bicarb is not low....but I am susicipious this is being masked by the IV nutrition (thankfully!) -- remember the dry overnight diaper at 5am? That tells me more accurately what the IV nutrition might be masking.
It's enough for me, the outward appearance and suffering of our little man and now his pattern of labs indicating that his body is struggling. There is no reason to include zucchini in his diet, we won't be able to move foward with other foods, he's not thriving he's surviving.....no.more.zucchini.
So, the first day we gave him a tiny slice of steamed zucchini; he had some symptoms that afternoon that were notable but nothing to write home about, but note and move through....so the next day I got him to take a tsp of boiled, pureed zucchini (that I pureed with some of the water I boiled it in- was that bad?) - he let me feed him a teaspoon and then would not take anymore. At the 4hr.mark, he had this terrible-terrible and classic-for-him irrational tantrum...the kind where you can't let them out of your sight because they may do something to hurt themselves because they are being so irrational (but he wouldn't let me hold him)- so he is having this tantrum in the bathroom (of the hospital) and he is drooling excessively (doesn't normally do that when he cries and he is 29mo.old so drooling isn't an everyday occurrence around here).....another FPIES mom helped clear this up in that it is uncontrolled reflux, which makes a lot of sense. He isn't necessarily crying as much as he is screaming- no tears, just screaming...then he starts to choke (not on his saliva, almost as if he is throwing up in his mouth and swallowing it down- you know when you recognize that sound)...it goes on for at least 30min. The plan is to take labs if he is symptomatic- so I ask (ok beg for these labs) and they are taken and found to be "off". He finally calms down and then is ok for most of the day- until he has some cookies later and then about 5hrs. after that, he has another one of these "mini" tantrums- this time he has clear mottling and his feet are purple when being held (again, classic for him when he was an infant and symptomatic). But then, he recoups from that and is fine again, he sleeps ok that night. Then we did baked zucchini the next day-Friday (so boiled and pureed and then baked into his muffin/cookies) and he doesn’t' have any symptoms to write home about. We got him home yesterday and he had his cookies again at dinner, and 3hrs. later had a poop soup diaper; but no other symptoms- sleeping good, eating ok, has a red rashy spot on his right cheek, behavior is ok,.... The plan is to continue the food trial for a full week- now at home, getting labs as needed. Until poop soup diaper, I was feeling confident that we were going to be able to work with just baked zucchini into his diet....and not even 'test' the boiled zucchini again (why make him miserable?) but now I worry that all we're doing is drawing it out and going to end up making him sicker in the long run vs. just knowing.
He's been, the past 2 weeks (since probiotic/sorry board game ingestions/symptoms) a little on the slower side for dirty diapers, only going every other day, and it being more play-dough so now to have soup...it's confusing... The labs that were off were his blood sugar and bicarb- he fit the criteria for acidosis (which he has had before with reactions); and his neutrophils (white blood cells) took a huge dive (very classic for Sam following a reaction, and why he always gets a cold after- (he's susceptible because his WBC's are down), and his hemoglobin dropped a full point (and didn't recover)- again classic patterns we’ve (I’ve learned) for Sam. I'm nervous but we need to know.....it seems to me that his body is having these dips of problems so if we helped his body (by baking it)- he could tolerate it? But....this could be his body adjusting or it could be building to a reaction....I'm trapped because we are supposed to call it a pass or fail after 7days but if he doesn't get enough of a serving- it's going to be hard to call it, but if we do too much- and his body is just adjusting we risk pushing his limits versus teaching his body to tolerate the food (which obviously is the goal).
My theory is: what we are seeing now (and the labs that correspond with his symptoms), but then we see him re-coop. I am curious if this is his body training for oral tolerance or if it is his body teaching to react? So, do we push it so we know and not continue to make him more chronically ill; or do we take it slower so we continue to teach his body oral tolerance?
Wednesday, November 23, 2011
Recently, we had a fundraiser to raise awareness, a month later and I am still overwhelmed at the response we received. Money raised, in Little Man's name, for The FPIES Foundation, to help future families in more ways than one. To enable The FPIES Foundation to provide assistance and programs, and educational materials (such as a flyer we distributed at a recent Food Allergy Fair, more on that later) to further raise awareness and support for FPIES families following in the same footsteps as we have on this journey. A treasure beyond measure, the gift that will keep giving.
Kindness, empathy, thoughts and prayers for strength and healing....all among the richest gifts we have received from family, friends, neighbors, acquaintances, even complete strangers. God has mysterious ways of showing us how beautiful His world remains- because of the very people who have shared our journey, who have provided us with this support.
God never promised there wouldn't be trials and troubles in this world, He only promised he'd help us through it. This diagnosis can be ugly, but God is giving us the graces to get through it- the strong instincts to know what to do next, the drive to do it, the graces to perservere through adversity, and the support from others. Assurances we are on the path He intends for us and Faith that there is something at the end of this journey.
Tuesday, November 22, 2011
This past spring, my sister held a benefit for our little man. It was extremely successful in that it raised a lot of awareness in our home town community, 3 local newspapers did articles, people wrote to us from reading those articles, and of course people attended the benefit to meet our little man and ask their questions and learn about what FPIES means to him, and our family. At that same time, in the spirit of raising awareness, I also booked "Tip Night" at a local Pizza Ranch to raise awareness in the community we live in now. Unfortunately, we had to reschedule that as Little Man's needs were too high at that time, so we postponed it for this fall. It became scheduled for Oct.24th. Of course little man's needs have not slowed down much, but I decided we needed to go ahead with it anyway. We were blessed to have friends that were able to help- help advertise it and help work at it; because little man was actually in the hospital the week prior to the fundraiser!
The FPIES Foundation helped in making an announcement flyer for it, that a neighborly friend helped hang up around town.
It was a great event. Pizza Ranch has a program built in for non-profit fundraiser events such as this. It is called "Tip Night". You bring the waiters, you earn the tips.
Monday, November 21, 2011
We've chosen to start that with zucchini. Well, I've chosen. I chose zucchini because we have never tried it before, he is not sensitized to it, and it's been over 18mo. since he's even had anything in the squash family. It is a good source of Vit.C which he needs more of in his diet. It is easy to digest and easy to bake in to foods (since his favorite food right now is his "cookie"). It is low in sugar and lower in fiber (skinned and seeded), it shouldn't cause any symptoms much less an FPIES reaction. But if it should, it's only zucchini and not available all year (although I do have a freezer stash). It WAS in season when we were going to orginally start these food trials so it was perfect (food trials were delayed a month due to a PICC line infection and treatment).
So, here we are at zucchini. Today is Day 6 and today went well; but we're holding our breath because we've been cycling through some concerning symptoms. My hope and prayer right now is that these symptoms are merely his body demonstrating that it still struggles to find Oral Tolerance and to caution us to continue to be careful in the foods we chose next and how we prepare those foods, and how we help his body accept those foods.
So, how has the trial been going? What symptoms are concerning?
11-16 Zucchini Trial at 11:15am= 1 small slice and ½ a “muffin” with zucchini in it. Fussy before nap, itchy ears, red cheeks, smelly gas, refusing bottle before nap. Woke up from nap pale but pinked up, and played well all afternoon/evening; very tired looking, drinking sips of his peach‘juice’. Slept ok through the night, some tossing and turning. BM diaper is "normal”- has been having them every other day, thicker.
11-17: Zucchini at 9:30am- boiled/purred- 1tsp. 12:30pm had bites of a cookie made with pureed zucchini. Got D10 for low blood sugar at 10:30am. Refusing bottle a lot today, looks more pale this morning but happy throughout early morning, had some “blah” and fussy times. Found low blood sugar results from early AM lab draw,after getting unhooked from TPN; took 4oz.at 10:30, then had lab draw right after finished bottle. 1:30pm- irrational tantrum, drooling, choking, screaming (not crying-screaming),noted mottling (but was playing with cold water). Settled down and had a short nap, woke up crabby; better after bottle but still “blah”, evening tantrum noted to have mottling and feet turned purple when being held (cleared when put down), rash on right cheek, looks so tired, smelly gas. Decreased oral intakes, and decreased wet diapers (didn’t wet through the diaper last night as typically does). No BM diaper today.
Concerned that boiled zucchini caused the “episode” but could even have been from the D10 he got for low blood sugar. Slept well overnight.
11-18: Zucchini at 5:30pm baked in cookies-ate 3, got approx. 1tsp zucchini. Looks pale, purple around lips, lips look paler; but otherwise in good mood this morning. Started asking for his bottle again, and increasing intake mid-morning, took a good nap- woke up crabby but calmed down easily, played nicely all day. Willingly ate his “cookies” at 5:30pm- enjoyed them. 6:30pm noted rash on cheek flared up again, and smelly gas otherwise good mood. BM diaper had mucus surrounding thicker stool (playdough consistency).
Slept well overnight, but had to be near daddy. Woke up twice but didn’t eat.
11-19: Nibble of zucchini cookies at dinner 6pm. Fasting for AM labs to check blood sugar after TPN cycling, tantrums while waiting for 10am (fasting) lab draw! Ate well after that and played well- dismissed from hospital and happy to be home, ate “cookies” for dinner. Rash on cheek flared up after, otherwise no other symptoms- good mood and played well. BM at 9pm- “poop soup” (took pic)- watery/soaked in surrounded by thick pieces. Slept well through night. Eating better at home.
11-20: 9am bites of cookie with baked zucchini (same batch from last night). Asking for bottles, eating better today. Increasingly whiney all morning but playing. Rash on cheek flared again, itchy ears.
11-21: 9am BM diaper, blow out/liquid. 10:30am 1tsp of skinned/seeded/boiled/pureed zucchini- doesn't like it. Fussy off and on today as yesterday, but slept well and increasingly better mood throughout the day, increased energy.
I’ve been in an FPIES funk the past few days. It isn’t the reason I’ve been unable to keep up with my blog, that started with just enjoying our baseline (shhh.don’t tell FPIES), which was kept busy with the work on The FPIES Foundation, but then led to anxiety about upcoming food trials and landed us square in getting an upper endoscopy last week and proceeding to the zucchini trial we are in now.
The upper endoscopy showed mild reddened area’s that are familiar to some past scopes. The plan was for a food trial to begin- zucchini; but the biopsy results back the day we started the food trial showed villi blunting, again. Do we proceed with the food trial? What is the villi blunting from? Is this his normal? Is it related to FPIES or something else? Could he have small bowel bacterial overgrowth, and is this why he did so well on the antibiotics when he had the line infection a few weeks ago? Is every reaction causing villi blunting? Was this from the probiotic? And why? Those ingredients were “safe” for so many months – why would it now cause this? Or was it from the Sorry board game ingestion? We certainly saw symptoms from that. Or, is it from something else in his diet? In his world? A cross contamination from the soap I use to wash his bottles? The exposed sheetrock downstairs (gypsum is made from corn)? The potential cross contamination in the Bob’s Red Mill products I use (they process corn meal on the same lines, anything is possible)? An intolerance to the arrowroot starch in his daily bottles, feeding his small bowel bacterial overgrowth? If this is happening this frequently- without glaring outward signs, then no wonder we’re struggling with passing a food, and no wonder he has persistent and severe anemia. If the reactions aren’t causing just inflammation but causing villi blunting- it would be more severe, and take longer to heal…..and no wonder we are always cycling. It certainly makes sense for why.
Still so much to process, still so much as we continue through a food trial- zucchini….
Saturday, November 12, 2011
I worried if these recent reactions were going to compromise the scope findings. The Sorry board game was Nov.5th, the scope was scheduled for the week of the 7th. We have already lost a month, from the line infection; and he isn’t going to be able to have the PICC line for forever so we need to keep moving forward. We need to utilize the line to draw labs and watch what is happening in his body as we try to get his body to accept a food. Is it something we can change? Is it something we can help his body with? Are the symptoms related to his FPIES or something else? So, we can’t delay any longer but he is still having some symptoms. Ironically, our GI couldn’t do the scope the week that we had planned and put it off a few days. We would do it on Monday the 14th instead; and then be admitted for the food trial on the 16th for 3days. We decided to go with this plan, as we have been waiting months to get to this point.
Sunday, November 6, 2011
Little man was on a 10day course of antibiotics after being discharged from the hospital for the line infection. That course was set to finish Oct.26th. I began to think about how this long course of antibiotics, plus the multiple antibiotics during the hospital stay, would be robbing his body of bacteria- good and bad gut flora. Ridding the body of the “bad” gut bugs is a good thing, now how to ensure the “good” gut flora flourishes so that there is homeostasis in his gut: where the beneficial gut flora reigns, the “bad” gut flora remains to do its job keeping the fungal’s at bay.
We had stopped the probiotics he has been on since March, in August after a switch in brands caused some questionable symptoms and coincided with the scurvy he developed. The beginning of his probiotics last March was very favorable for him, he gained weight, his appetite increased, his labs leveled out, his hemoglobin was stable, he was at a baseline we hadn’t seen him at in a long time. However, he quickly hit a plateau so I set off to find a better fit- first I gave him the probiotic in 2 doses, a morning and an evening and that seemed to help; then I thought if that was helping, I wonder how he would do with the probiotic in his formula so he was getting a continuous “feed” of it all day, and so the probiotics were in his gut in an environment (with the food directly) that would support their growth at optimal levels. I can’t change his diet to encourage that (with foods referred to as ‘prebiotics’) so this seemed to be a good answer; and he again responded well to it. I don’t remember exactly when he seemed to be having a plateau or something again, but I thought he needed new strains- maybe the strains I was giving him was creating more dysbiosis within the beneficial bacteria. I tried adding a strain (bifidi) and he had too many symptoms to continue, I tried adding a new line (ProBioGold)and he developed symptoms, fevers and even an ear infection (his first and only!). Back to the CD-Biotic (3strain probiotic from Kirkman labs). Then, following advice from the Functional Medicine doctor we saw, he was concerned over this combination of probiotics and felt Sam needed custom probiotics; he had a guy (the “bug guy”) that did this very thing; so we switched to Custom Probiotics. That brought us to August when he had symptoms and then developed scurvy.
Now, the antibiotic brought us to a great baseline; all to see it slipping away with reintroduction of the probiotic? Does this make sense? Is this die off or is it a reaction (maybe one he was having chronically all those months) to the filler ingredients in the probiotic or even a reaction to the strains? Initially I started with the CD-Biotic, which is the one he responded so well to in March and the one we felt he did well on for a long time- even putting it in his formula mix. He had symptoms and I worried the long course of antibiotic was too much and 3-strain probiotic was too much to start him off with; so I switched to a single strain I had gotten to try and add to his 3strain a few months ago, or try in place of it since something seemed off. Dr.Jyonouchi, the research MD we saw in NJ (in Feb) had said he would need to be on a low strain (single would be preferable) as his system was so severely dysbiotic. After trying a few days of CD-biotic, with these symptoms of uncertainty, I thought I’d give it a try this time around….
Which brings us to Halloween screams. I had given it to him that morning for the first time (I worked over that weekend and I didn’t want to do anything while I wasn’t home). I thought it was attributed to him being upset that he couldn’t have the candy he saw us pass out to the trick-or-treaters. It was a bit irrational and extreme; when he had it the next night over not letting him dump water on the floor- was starting to wonder if we were seeing a pattern? That something simple was setting him off because he was having some underlying pain/inflammation/whatever-happens- in- his- body- during- a- reaction. I skipped the next day and he was fine. So, I gave it one more time- somewhat reluctantly…and, like clockwork, ~8hrs after he consumed the probiotic, he is screaming and screaming and screaming. Irrational, inconsolable. Other things I notice is that he is sweating (from the fit) but cold, also he is gagging every few minutes- kinda like you would hear from a typical kids’ crying fit when they are sobbing so much they begin gagging on their snot…only he doesn’t have snot and he’s not crying as much as he is just pure screaming. And the gagging is more like he is throwing up in his mouth and then swallowing it. And even his brothers recognizes the screams and the pattern, and say “first it was the Halloween candy, then the next night over the water/ice, and now this? What is going on?” Also, in the night on these nights- he is crying in his sleep and even crying out “help”, it takes a lot of rubbing his rock hard belly and patting his back to get him consoled for sleeping.
I didn’t give it to him yesterday and he was great all day; until he sucked on the Sorry board game when I wasn’t looking (or expecting)…and 8hrs (at 4am) came the screaming – that was fun. And today he is a little clingy. Most cardboard products like that have corn and potentially soy in them- and it becomes more clear that the probiotic is the same pattern of symptoms. His pattern of symptoms. Now what?
This got a little long, and complex- but this is my thought process as we go through the day(s)….everything needs to be trialed, anything could cause reactions. I’m tired.
Thursday, November 3, 2011
Prayer Blankets are sewn with love and prayers for those who receive the blanket. This prayer blanket is a symbol of our faith sharing community “covering with prayer the individual who receives it”.
The Prayer Angels of St.Joseph parishes, commit to prayer as they fulfill their part in construction of the blanket. The process involves shopping for material, washing, coordinating fabric, cutting, sewing, ironing and tagging. After the blankets are complete, they are placed on the altar at a Healing Mass and blessed by the priest. Those who are to receive the blanket are held in prayer. These beautiful acts truly transform the blanket into a “PRAYER BLANKET” whose very threads become interwoven with pleas for healing on behalf of the recipient.
Each blanket bears a tag that reads ‘You are covered in Prayer.” This blanket has been prayed over and blessed at St. Joseph Healing Mass. Be Not Afraid, for the Lord said, “I am with you always, until the end of age”. (Matthew 28:20).
This gift of prayer originated at the MD Anderson Hospital in Houston, TX where the chaplain who gave the blankets to the sick found that they so uplifted the patients that she began to deliver them on a regular basis. Blankets were given to hospital patients, the sick at home, the Veteran’s Hospital and cancer support groups. Missionaries presented them to the orphaned, very elderly, and those who were sick in Guatemala and Volograd Russia. The gift was received as another wonderful way that people are doing God’s work.
The Prayer Blanket brings comfort to the suffering and peace to the fearful. When one sees and touches these blankets, they know they are not alone – that they are covered in prayer and there are those who do care about them.
God has used this beautiful ministry not only to touch the lives of countless people who have received the Prayer Blankets while they are going through extremely difficult times in their lives, but the lives of those involved in the ministry as well. We are blessed to have so many people who give abundantly of their time, talents, and treasures, making Prayer Blankets possible.
Wednesday, November 2, 2011
September 1 Little Man was 11.1kg, which is 24#. Today, 2 months later, with 8 weeks on TPN, he is 12.76kg, which is 28#! He has gained 4# in 8 weeks! This weight will help us see how he is doing on food trials as well; although he will have the "back up" of TPN during the food trials to keep him from losing - we may still get indicators of his inflammation as calories are not readily absorbed from the gut, and his body needs extra calories if his villi (small intestine walls) get damaged. His growth chart is full of these types of losses. His growth curve is a picture of a bouncing ball. He would gain, and then fail a food and either lose or not have any gains for weeks. Just in September, when he was reacting to the Vit.C supplement; in a week and before we started TPN he lost 1/2kg which is a full pound in just one week! (so technically he has gained 5# in 8 weeks on TPN- having to regain a lost pound!).
He needed this weight gain, for catch up growth. He is now in the 35th% for his weight. I didn't think to get a height check on him too, and should have because looking at his growth chart, I am reminded that his last height check put him in <3% on the chart. He is much shorter than his brothers were at this age (judging by their growth charts on the wall), but he is proportionate for him. He is finally out of 24mo. clothes, and even moving up a size in diapers. From failure to thrive, grow and gain weight, to catch up growth. My heart smiles.
Tuesday, November 1, 2011
The set back of the food trials is on my mind a lot, we were all set to go in 2 weeks ago; and that we didn’t- I feel like we’re in limbo, waiting to go to the hospital for the inevitable and yet looking forward to getting it done…and potentially getting some food for his tiny menu from it, or the very least- learning more about how his body is working and why we’re struggling so much with so many foods.
We got blood cultures last week which tell us if the antibiotic course has helped his body fight the PICC line infection, to test for any signs of the bacteria in his line remaining after the long course of IV antibiotics. We got the results today- negative! The line is CLEAR of bacteria, no lingering bacteria to cause an infection! This is such great news and now we can proceed to the plan of food trials, where we were a few weeks ago before all of this happened. I have thought (and wrote here about) the setback this infection has caused but there is a peace of heart knowing that everything happens for a reason. With that Faith, I have been able to get through this difficult time of the infection, sepsis, hospital stay; but I admit I still have had lingering difficulties with not knowing or understanding the plan, the next steps. I’m a planner- so FPIES often has me turned upside down. I’m learning the difference between planning for the future while trusting in my Faith to intervene in my plans as needed.
I will admit that I feel better today though, knowing the infection is clear and a plan (re-plan) is forming. He will get IV iron this week as his hemoglobin is dipping low again (low 7’s now, normal is 11); and then next week he’ll have a baseline endoscopy before food trials the following week.
All in His plans…trust….
Sunday, October 23, 2011
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?
Saturday, October 15, 2011
They have identified the bacteria, normal gut bacteria: pantoea agglomerans (previously known generally as enterobacter). This is a gut flora bacteria, belongs in the gut- not in the blood stream. It has set up home in one of the lumen’s of his PICC line. The antibiotics have eradicated it from his system, but it remains a threat in the line. Only further time, waiting for culture tests, will tell if we will be able to save the line (although looks optimistic that we will). We do cultures every day and as soon as those cultures do not grow any bacteria, they will be able to send us home on IV antibiotics.
The other complication is that his hemoglobin is dropping again, now down to 7 (normal is 11, we have to consider transfusion at 7 and if he gets any lower- we will be facing a transfusion). They haven't given him any IV iron this week yet but honestly the last 3-4 IV irons have not done ANYTHING to raise his hemoglobin....this still remains a mystery to me and seems there surely must be something else going on with how his body produces hemoglobin and how the reactions affect that.
Another kink is that the next day, he threw up this morning. Daddy (who takes the overnight shift) said he had a great night- slept through the night, and when waking up - before he had anything to eat, he threw up. Could be the antibiotics messing with his gut flora causing it; could be that he painted the day before (a dot marker type painting) and he painted his hands and then later licked his hand.....12hrs.later he threw up- classic for him (and has happened before); we had to just wait and see.
He never did have any further throwing up or tummy pain; so we concluded it was the paint. Bizarre but FPIES. He continues to do well, and we are dismissed from the hospital to continue IV antibiotics at home; having identified the bug and the antibiotic to treat it. We are happy to go home.
Wednesday, October 12, 2011
He had stabilized after the episode I was there for, and they finished his vancomyocin antibiotic; we then did a quick site care/dressing change (due once a week) and then I headed home- trading shifts with his daddy. The vanco takes ~1hr.so that finished up and they started the TPN. Within minutes monitors were going off, and he was decompensating quickly- this time worse than the previous one. I'm still fuzzy on the details (and maybe that is better) but they quickly transferred him to the ICU and got him stabilized. Little man’s daddy called me ~11:30pm to let me know he had been transferred and was now stable. They would be getting chest and abd.xray's to be sure nothing else isn't creeping in. He had a few more rough hours, he was stable from the shock episode but his monitors kept going off- he was in pain…from being full of stool! Gave us a little insight into how painful it is for him! They were able to give him a suppository that “took” within minutes and he was able to relax.
We stayed in the PICU for the rest of the day, with close monitoring- he continued to have blood pressure problems and a low grade fever throughout the day but by evening, he was dramatically improved.
Little Man has a double lumen PICC line- which means there are two lines that converge to the catheter that goes into his vein. One of the lines has always been "sticky", we suspect this line is where the infection set hold. Still unclear of how he got the infection but infections are the highest risk with PICC lines. We do feel we caught it early though; as early as we could have. The infectious disease doctors are optimistic that we will be able to preserve the line, that the organism that is infecting it is an easier to treat one and that the antibiotics we've been giving him are working.
Still not sure of the plan from here, but of course that remains on my mind- how long we'll have to wait for the food trials, how much the antibiotics are messing up his system, how long he will be on antibiotics, how long he will be in the hospital, if having an infection means we have to stay in the hospital or can we continue his TPN at home?
Monday, October 10, 2011
Little Man is rarely ever sick, besides his FPIES, but could this be a viral illness? Should we be calling his doctors? Taking him to th ER? The home health nurse confirms that the degree of his fever is concerning and that he is still shivering/shaking and burning up- she said to give him a few more minutes and check his fever again, if it was still elevated- to take him in to the ER. I checked and it was 103.7. I don't know what is going on, but I do know sepsis is a number one concern with a PICC line and I have no desire to wait around to see if he is going to get better or worse at home....I head to the ER.
Once at the ER, we are taken back immediately and the assessments begin immediately. His temp is down to 99, we assume the tylenol has kicked in and remain concerned over his symptoms due to his PICC line so blood tests are ordered and an IV is started right away as well as IV antibiotics (thankfully via his PICC). The risk of sepsis is high and there is no time to waste. The blood will need to be cultured to see if it grows any bacteria, but in the meantime, he will be treated for sepsis.
We are admitted for further observation while we wait for more tests. Little did we realize we are also admitted for further tests. We were up most of the night- getting blood draws (had to have one not from his line), they also got a urine sample (NOT fun!) to check for UTI as well. We finally got to sleep around 4am; and were up by 6:30am. In there he was also started on another antibiotic- they were doing two kinds to cover as many "bugs" as potentially could be an infection in his line. Unfortunately (of course), little man is one of a rare few that reacts to Vanco with something called "red man's syndrome". I swear he looked like an umpa loompa! He was getting so red and SO itchy. The RN promptly stopped the infusion of Vancomyocin and the redness quickly disappeared. We waited for IV benedryl to continue giving this antibiotic. He tolerated it better after that but still had some redness and itching.
The next morning, he was doing well and even though we didn't have answers yet, we were optimistic maybe we were "just" dealing with the GI bug. That afternoon he was getting some IV antibiotics so we watched a movie while he stayed hooked up (the IV vancomyocin was infusing for an hour every 6hrs.). Once that was done, we unhooked and went to play- he was running and playing for a little over an hour. Then he was ready to sit down and relax with a bottle. I was finishing my dinner and I noticed he was started to look really tired, and sunken eyes. He leaned over and started moaning- as if he was achy. He then started shaking, the chills consuming his body. I called for the nurse so I could get her to take his temp before I gave him some ibuprofen- he was clearly uncomfortable.
Before the nurse could come in, the Resident was coming to tell me that the scope we had previously scheduled, had been postponed. She quickly noticed his poor tone; falling asleep in my lap....she tried to get him to open his eyes and he couldn't even do that. We took a quick temp - he was clammy, his temp matched as low. Other nurses were coming in to help and getting blood pressures and oxygen saturation's, as well as heart rate and respiratory rates. He was all over the board. He is now having full body bright purple mottling, and his hands and feet are BLUE. His O2 was 84! The next temperature reading was 39degrees C, now he's burning up- from cold and clammy to burning up in 5minutes! I held him close and comforted him....I just wasn't sure how bad this was going to get. But that was it, his body started to relax a bit more, his breathing evened out better and his blood pressure stabilized. We were able to give him some ibuprofen and hook him back up to his IV fluids as well as antibiotics. The senior resident came in to confirm with this reaction and the cultures that were growing; we're dealing with sepsis.
Is this as bad as it will get? Will he have more of these episodes? He is fighting his way through this....