Kara's Surgery

Kara will be having surgery on Tuesday, October 12th at the U of M Children's Hospital.

We won't know what time until the week before but if I understand it right, it will be right away in the morning and then will be under observation until late afternoon or so. She will be put under for the procedures to be done. They will be doing an egd and a sigmoidoscopy. They will be doing biopsies and this will hopefully give them a better idea of what we are dealing with.

GI Appt

We went down to the U of M yesterday for Kara's GI follow up appointment. Dr. S. was so good, once again. She came into the room and really seemed to remember us and specifically, Kara. I showed her my spreadsheet that I update as we progress or regress and she seemed impressed. She commented that I seem to have a really good handle on how to do this! (Thanks fellow FPIES Mommies!) After we briefly went through how the last month has been she told us that she wants to scope Kara. I'm so glad she was insistent on doing this because I was going to ask her if she would, anyway. It would be so nice to know what is going on in her insides after all these gastro-disasters lately.

Dr. S. was very interested in hearing about the pear fail and is thinking that Kara probably has some sort of sugar intolerance on top of the multiple food allergies. They will be doing biopsies to see if we can determine what exactly is going on with that as well.

She had to have some lab work done as well as a few other preop things done and she did so well. She is such a good patient when she's getting checked out! The lab draws are obviously horrible but I just remind myself that she's more mad than anything because they numb her arms with emla cream 20 minutes before the blood draw so she really can't feel a hting. I'm getting stronger about holding her down for these - both physically and mentally! It was a long day and we were all tired when we got home but I was satisfied with what we found out.

Potato Trial and GI

Our latest food trial is potato and we just finished day 2. She doesn't care for them too much so it's sort of a struggle to get her to eat them. I fried some up with corn oil tonight so I'm hoping she'll eat them a little better tomorrow.

As always, I'm debating what our next trial will be. I don't know if I'm ready to try a fruit, vegetable, a meat yet, or even wheat. After our pear fail I'm a little leery on another fruit but I would really like something to flavor her water a little bit, especially with our latest (almost) bout of dehydration. We really struggle to get her to drink her Neocate and even then, she'll only take it out of a bottle and sometimes even refuses that.

Tomorrow we go to the U of M for our follow up GI appointment. I'm really anxious for this trip, as usual. In the last month we've had two fails - soy and pear and two passes - banana and corn. At this rate we aren't getting anywhere very fast! We will find out tomorrow if Dr. S will scope Kara. with the recent pear fail and the week + of getting back to baseline, I'm really hoping that she will decide to do this. Poor Kara has reflux going on, not to mention her other icky tummy problems!

I have to pack for tomorrow and figure out some way to pack her food for the day - keeping it cold, warming it up, what to pack her, how to feed her. Going to a restaurant is out of the question, it's just not fair to Kara to eat in front of her like that. We'll probably be relying on Neocate tomorrow with Kix to tie her over and more or less entertain her.

I'll update about the GI as soon as I can. Wish us luck!

Week Update

This has been a busy week. I have spent the majority of it home with Kara, in my arms. I'm waiting for her poor little body to get back to baseline and just when I think she's getting there, something else happens.

I brought Kara back to the doctor on Thursday. Her temp went down but she was still so cranky, having icky diapers and her bottom (once again!!!) is raw. That trip was a waste of time, not to mention the $25.00 co-pay. We had to see a different doctor, as our doctor was completely booked for the day. I had faith that he would listen to us and do something to try and help us but I left there feeling extremely discouraged. He had NO CLUE what I was trying to tell him and the only advice I left with was "try Desitin." REALLY? DESITIN? She has a PRESCRIPTION for her bottom because it gets to bad. I use EVERYTHING on it. What about this awful tummy ache? What about labs drawn to see if everything is ok, check levels that might give us an answer as to how bad the inflammation is? (For the record, his answer to that was "I can run those labs but if they come back out of whack I wouldn't know what to do for it, anyway so we'll just let GI do what they want on Wednesday.") IN MY OPINION (but I'm no doctor so maybe I'm off!) RUN the labs anyway, we see GI on Wednesday and then we can be one step ahead when we get there. No such luck. He did look at the letter from our last GI visit and said "it says here her RAST and the scratch test showed no allergy to rice, oat, soy, and just a minimal reaction to dairy." I explained that all her FPIES reactions aren't IgE and he still didn't seem to get it. When he brought up her FPILES (that's right, PILES) I gave up. I did call and our nurse and let her know what happened once we got home. In the meantime we are trying to manage until Wednesday. Her doctor can see her on Tuesday if needed.

Yesterday Kara finally started to be feeling a little better but her squealing, screaming and screeching continues. She is not happy to please and in the meantime we are creating a monster. This afternoon we couldn't please her no matter what we did. She was finally content, and when we discovered what she was in to, we decided to just let her be because she was happy, our house was quiet. She pulled all of the DVDs off the shelf, one by one. There must be 100 of them, in a pile on the floor. She had a blast. What do you do in situations like that??

Yesterday we had some friends over. Brenna forgot the rules and brought her sippy upstairs with juice in it. Kara went up to see what the big kids were doing, found Brenna's sippy and sucked it down. It was white grape juice. She's had grapes before and prior to the pear fail I would have been pretty sure they would be safe but since then, what's safe?? She's so crabby but has been, she has icky diapers, but has been, so who knows. Then this morning i discovered her walking across the room eating one of those pink/white coated animal crackers with sprinkles on them. Brenna talked me into buying them at the grocery store and must have put some in a baggie yesterday when her friends were over. Kara got a hold of that baggie, and thought it was gold! I didn't even look at the ingredients. I know she ate one of them, I couldn't get to her in time, and I'm sure they contain milk, soy and who knows what else. I'm worried at what's to come as the day goes on because of the extreme crabbiness today has already brought.

In the meantime, I have gotten two get aways the last two nights and they are healing! Thursday night we had a fire department auxiliary meeting and a few of the girls went out afterwards. It felt so good to be away but we talked about Kara quite a bit. It's funny how we deal with this every day and don't think of it's general difficulty a lot anymore, it's become a (frustrating) way of life. Talking about her and answering questions about her really got me thinking and emotional about how much this sucks. It's tough but it's a way of life. There's no other way for her/for us. In order to figure out what she can and can't tolerate, we need to go through these challenges and unfortunately, deal with FPIES reactions if it's something she can't handle. Last night, I was able to get out and have appetizers, a margarita and go to a movie with a long time girlfriend. We haven't hung out one on one in a long time and it was so nice to get away again! Shannon, next time it needs to be longer, or lets even make it a weekend! :) Brian gets his getaways but I have a hard time leaving Kara too often so it really felt good. Tonight I may make it a 3rd night and get out on a date with my hubby if Kara isn't too fussy. I think some Grandma and Grandpa time is definitely the cure for that fussiness if she's ok otherwise! :)

Poor Baby!

I didn't post much about the pears fail yesterday because I wasn't entirely sure what was going on, other than a fail. What we have is a very sick baby on our hands and I don't want to fail another food, ever again. I understand that the process of finding foods that Kara can eat is going to more than likely involve more fails but hopefully this one is fresh out of my mind before that happens again.

All weekend I kept commenting on how Kara wasn't herself and that she had the pear fail. Her diapers were bad, her coloring was bad. Her complexion had a sort of gray-ish tinge to it. Sure she was running around and playing but she just wasn't quite right.

I left for a few hours yesterday afternoon and then came home to a fussy, warm baby. I took her temp and it was 102. I reluctantly gave her motrin finally because she was so cranky and we needed to get that fever down. That resulted in a sick baby running in circles around the room. I hate what it does to her! It takes her fever down but she's still sick, has no balance, falls and gets hurt. She refused her bottle last night and I pretty much knew what was to come. I woke up at 4:00 this morning panicking because she hadn't been up yet. I ran up to check on her and she was burning up. I took her temp, again, 102. She popped up so I got her up, gave her more motrin and tried a bottle again, with no luck. We sat in the chair for an hour as she whimpered on my lap, then I put her back to bed.

I got up this morning and made her an appointment with her family doctor and also called the allergist to catch him up on everything that's been going on in the last few weeks. We made an appointment in 2 weeks to see him again, after her GI follow up. I'm not sure he has any real idea what to do but wants to be updated, anyway. In the meantime our doctor is doing some looking around for an allergist that can maybe help us out better.

While at her appointment we ruled out possible causes of the fever. Ears are good, lungs are clear, throat looks good, and then started looking in her chart at her past fevers. She's had a lot of them but we never really had a definite answer to why she had them. Looking back, they run hand in hand with diarrhea so this is opening up the picture a little more. She's more than likely been having small reactions all along that we couldn't pin point (until the elimination diet) and tends to run a fever right along with them.

Kara's weight is down. She had her 15 month well child check a week and a half ago and we were so excited because she finally gained weight. She was 21 pounds, 6 ounces. Today she was 20 pounds, 12 ounces. This is on the same baby scale, with only a dry diaper on, both times. This is discouraging. She hasn't thrown up, I explained to the doctor but has been really "urpy" and as I said that, Kara showed her what I meant. We are watching closely. She's not dehydrated yet but close. We are doing unflavored pedialite but she won't touch it. She took a total of 14 ounces of Neocate today and a few bites of kix, some banana and some corn meal cereal. I put her to bed with a 100.6 temp and I'm hoping for some improvement in the morning, otherwise I'm afraid we're going to have to go in for IV fluids.

We need a few more days of just corn products and banana to get back to baseline before we can trial anything else. With her refusing all but a few bites of food I'm hoping we don't run into food aversion problems again. Hopefully she's back to herself soon, it's been a tough couple of days!

Pears Fail

We trialed pears the last few days after much consideration over what our next food was going to be. I decided on pears because it is one of the few foods that most FPIES kids can actually have.

We started pears last Wednesday around suppertime. I gave her a sippy cup of about 1/4 pear juice, 3/4 water. That night she was up a good part of the night really fussy, just laying on us, obviously uncomfortable. On Thursday she had a total of 5 bad diapers. Hello, diaper rash, it's been a whole two days. I can't say we've missed you!

I did some researching of this and thought maybe it was because of the juice, maybe it was the syrup in the pears. I had some fresh pears but when we finally went to trial pear, they weren't so fresh so I settled on what I had. It was on Friday that I steamed some up. This breaks down the proteins a little more so hopefully lessening the chances of her reacting.

It was evident by Saturday morning that we were failing so I called it quits. I'm bummed about this and not sure what to trial next. I really thought this was going to be a pass food but it certainly explains a lot of past behavior and symptoms in Kara before we started the elimination diet and food challenges!

FPIES Article

I came across this article tonight and thought it was an easier explanation of FPIES to understant.

http://http//www.pediatricallergyindy.com/2010/02/17/fpies-food-protein-induced-enterocolitis-syndrome/


This is by Dr Frederick Leickly from Riley Hospital for Children in Indianpolis.

FPIES- Food Protein-Induced Enterocolitis Syndrome
Last week I had the pleasure of meeting a young lady with infantile FPIES. Both of her parents were with her for the evaluation. The family alerted me to a contribution to the literature written by allergists for a condition that may not be commonly seen by an allergist. What I learned from that encounter has broadened my perspectives. FPIES or Food Protein-Induced Enterocolitis Syndrome is a clinical condition rarely seen in the allergy clinic. Thankfully it is a condition that is very uncommon. Based on how these children present, I would think that FPIES would be most often seen by our colleagues in pediatric gastroenterology. FPIES would not have been something that we could diagnose by a skin prick test (SPT) or by specific IgE in the blood. This is an immune reaction that is cell- mediated, not antibody mediated. IgE is not involved with the reaction. This cell-mediated reaction is more akin to how contact dermatitis or poison ivy affects susceptible people.

This young lady’s mother had with her an article that escaped my attention. The article was written by known experts in the field of Allergy (the lead author was Anna Nowak-Wegrzyn with Hugh Sampson, Robert Wood, and Scott Sicherer as contributing authors). The paper was a nice review of FPIES and a study of 14 special children. I think that any allergist who sees young children should review this paper. These young children can present with signs that are possibly consistent with anaphylaxis.

The article was published in the journal Pediatrics in 2003. It is a review of 14 children who presented over a five year period at the Mount Sinai Pediatric Allergy and Immunology Clinic (New York, NY) and to the Allergy Clinic at Johns Hopkins Children’s Center (Baltimore, MD). The reactions that these children experience include severe diarrhea and vomiting which can lead to dehydration and shock. This is a clinical diagnosis; there are no specific laboratory tests that make the diagnosis. A food challenge can confirm the diagnosis.

Milk and soy have been the most commonly implicated foods causing FPIES. This article shows that other foods specifically solid foods have been shown to be associated with this syndrome; rice, oat, barley, peas, string beans, squash, sweet potato, chicken, and turkey. These children underwent food challenges to show the cause-effect relationship between the exposure and the symptoms. There were many combinations of foods causing the problem; cow’s milk alone, soy milk alone, both cow and soy milk, a single solid food, and more than one grain. The group was compared to children who were only milk/soy sensitive.

The profile of the Solid Food FPIES population was as follows;

■Age at onset of the reaction: 5.5 months (range 3-7 months)
■Age at resolution: 24 months (range 14-44 months)
The Milk/Soy FPIES profile was the following;

■Age at onset of the reaction: 1.0 months (range 2 days to 12 months)
■Age at resolution: 28 months (range 14-21 y)

This was the first published study of FPIES triggered by solid food. Oat was the most common food causing solid-food FPIES. The study also showed that breast-feeding may have a protective role in preventing/delaying the development of FPIES. The diagnosis of solid-food FPIES was not made until after two reactions. It was also noted that these reactions were severe. The delay in diagnosis was attributed to a number of possible factors; low incidence of the disorder, a presentation that looks like septic shock, and the belief that solid foods such as grains, vegetables, and poultry are of low allergenic potential. It was also noted that the time course of the reaction may delay making the correct diagnosis. The daily feeding of milk – cows and soy, leads to chronic problems. The re-introduction of the milk causes symptoms two hours after the exposure. As mentioned previously another problem is the lack of any test (other than avoidance and a food challenge) to confirm the diagnosis.

Another point that was made was that almost half of the children in this series had multiple food sensitivities. Children who were already on a casein hydrolysate formula had a median of four solid-foods that they were sensitive to.

No infant developed FPIES with exclusive breast feeding in this series. The authors pointed out that they were unaware of any reports of FPIES during breast feeding with absolutely no direct oral feeding of an offending food. No infant developed FPIES to milk/soy after age 1 years and the oldest child who had the solid-food FPIES was 7 months old. There were no ‘predictors’ of which child with milk/soy FPIES would go on to develop solid-food FPIES.

The Bottom Line-

The reaction of vomiting/diarrhea possibly leading to shock can be consistent with an IgE-mediated reaction and these are perhaps more common than FPIES. Such a reaction would lead to an allergy evaluation which will be negative if the diagnosis is FPIES. However, the infant is still at risk for a severe reaction with re-exposure.

Board certified allergists are credentialed in the care of allergic conditions in both pediatrics and internal medicine. Some of us went into allergy after completing training in pediatrics and others were trained in internal medicine. FPIES would not have been a clinical entity seen during internal medicine training. It may have been seen/talked about for a pediatric oriented allergist. FPIES favors infants. My point to all this is that although very rare, we need to keep this type of presentation in mind when seeing young infants with scary episodes of vomiting leading to shock with solid-food exposure. Their evaluation will show no evidence of allergic sensitization. We can help by teasing out the history of exposures and clinical course. We can offer recommendations for avoidance of the common foods that have triggered solid food-induced FPIES. This profile of young infants reacting in such a violent way needs to be considered in the evaluation especially if they have had issues with cow’s milk or soy milk.

This young lady made an impression on me. Her story was very scary. She caused me to go back to the literature and review what is known about her presentation.

Fred Leickly