Feeding Tube Awareness Week: Grateful for our Hardest Decision

For Feeding Tube Awareness Week this year I wanted to share something I wrote last year. I wrote it for a fellow FPIES mom friends website, Awareness Critters, to raise awareness to both rare diagnosis and tube feeding. 

I want to share how much having a feeding tube has positively impacted our son’s life.

The decision for our son to have a feeding tube has been one of the hardest decisions we had to make for any one of our children.  I want you to know, our choice to proceed was the best choice we made for the health of our fourth son who was born with a rare condition called Food Protein Induced Enterocolitis Syndrome (pronounced “F-PIES” for short).

Having FPIES doesn't mean he has to have a feeding tube, but he does.  Having FPIES does mean that he has a delayed food allergy condition. There are no tests for this type of food allergy and the only cure is to avoid foods that trigger this delayed allergic response.  Some babies/children are allergic to 1 or 2 foods, while others (like our son) can be allergic to multiple foods which can further limit the diversity of the diet. Making things more complex for him, foods like soy and corn; which our son is allergic to, are in/on so many foods (sprays and waxes on fruit, can linings, plastic materials, animal feed, preservative and additives, etc) which makes his variety of safe foods became even further limited.

FPIES is a rare and often misunderstood diagnosis and, seven years ago, when our son was first diagnosed, much less was known as we searched for best options to fit his needs – to provide the him nutrition to thrive and grow, nutrition that he wasn’t allergic to.  It was a constant balancing act that we were struggled with everyday.

Before he turned 3yrs, a feeding tube was discussed by his medical team for months as a means to help him thrive, and despite not currently having a safe formula to put in the tube, it was hoped that it could provide him with some consistency to escape his cycles he was trapped in due to his allergies and limited diet. The cycle of: not enough nutrition, trialing a food in hopes of finding nutrition, inflammation causing colitis, pain from the reaction; and malabsoprtion leading to anemia, low blood sugar and acidosis, vitamin deficiencies, and weight loss.  Keeping him further trapped in the cycle was not enough nutrition to make up for the malabsorption resulting in weight loss and further anemia, vitamin deficiency, and other unknown internal disruptions to his body.  And repeat with each food trial, each failed food, each accidental exposure or ingestion.  

A feeding tube should have been an easy decision. But, would it really help him?  We knew if it would not help his allergic condition (FPIES) but they assured us they were confident it would help him, somehow.   The final decision was left to us. 

No one would tell us this was the right decision.  We knew the odds were that it would help him; but having a tube doesn’t make allergies go away or less allergic to your allergens; so how could it help him? We were going through so many emotions….

We decided to proceed forward, a large part of the decision now was because he was currently on TPN (Total Parental Nutrition) via an IV line in his chest.   Trapped in that viscious cycle was a little boy who still had to enjoy eating and he was losing that battle, he had begun to refuse foods that provided nutrients he vitally needed; combined with villi damage from food trials left him needing TPN.  But, after 8 mo. on TPN and before committing him to a port for continued and long-term TPN, his medical team wanted to trial one more formula and we would do this through an NG Tube which would be his first tests of how his body would handle enteral nutrition.  We always remain hopeful that his body will accept the nutrition it needs but after a few weeks on this formula it became clear that even though he was not having a typical acute FPIES reaction to the formula; his body was not accepting it (initially by his outward demeanor, pallor, and sensory flares and then later confirmed by inflammatory markers in his blood and stool).  We still don't full undestand this reaction to the formula but by this time, we had already committed to the G-tube and moved away from TPN. What would we do now?  Had we made the wrong decision?

It was a difficult time, our little boy was hurting and he needed nutrition.  We decided to begin moving forward with a blended diet of his safe foods to start, with the hopes that his nourished body would begin to heal itself and, with nourishment we could slow down his food trials to the pace his body needed for recovery between trials and we could, hopefully, slowly, add more foods as we expected to do when he was first diagnosed.  He fell into a viscous cycle and now his tube could break him out of this!  We had hope! 

It has been a slow process but we have been able to add a few new foods -- to his diet and his blenderized feeds - every year since then, filling in his nutrition.  And, along the way, he thrived. 

Having a G-tube with safe nutrition allowed us to finally not have to worry as much about his daily caloric intakes, how we would coax him to eat enough, doing ‘dream feeds’, staying home to prepare his foods safely and fresh.  And it allowed us so much more.  For the first time in years, we were able to keep his blood sugars stable, he started to sleep through the night – not waking up hungry, and he gained weight and, in time, it helped with his severe anemia.  We relaxed around meal times, allowing him to eat his safe foods for pleasure.  We relaxed our schedule at home, not having to plan the day around when, where and how he would consume his few safe foods. This allowed us to do more things outside of the home and expose him to more of life and less of hospitals.  He has truly thrived.  We are truly grateful for this hardest decision.   

Nutrition Care for FPIES

This is very near and dear to my heart, nutrition in FPIES.   The FPIES Foundation is doing a webinar on October 25, 2016 for Nutrition Professionals.  To learn more and register, go here.

Dear Sarah, I just didn't know about FPIES. Food Allergy Awareness Week 2013: A Guest Post

I shared a post the other day on Food Allergy Awareness Week, and what it means to me to bring awareness to FPIES. Today, in the midst of Food Allergy Awareness Week, I am very touched by these beautifully written words, expressing so much of the frustration, anguish and most importantly, forgiveness we must give ourselves, as mothers, that we are doing the best we can everyday.  This is shared by a fellow FPIES mom, whom I have had the honor to 'met' via a Parent-to-Parent support group.  Jenn Booth has given me permission to share her letter to Sarah, here, on my blog. 

Sarah, I just didn't know about FPIES.

If I had known that you could look adorable on the outside, but be very sick on the inside, I would have known how much you needed me to help you.

If I had known that your newborn tears were from pain, I would have complained less about being up all night.

If I had known that feeding you baby food would put your life at risk, I wouldn't have spent all day trying to get you to eat it.

If I had known that you were desperately trying to tell me it hurt your body to eat noodles, I wouldn't have made you finish them before I got you out of your highchair.

If I had known that you had no other way to tell me that you were suffering, I wouldn't have punished you for hitting me.

If I would have known that you didn't just "get the flu" more than other kids, I would have questioned the doctors more.

If I would have known that your tantrums were a cry for help, I wouldn't have let people label you with a "behavior problem".

If I would have known how close we were to losing you, I wouldn't have wasted time brushing my teeth before taking you to the hospital.

If I would have known that the cookie I gave you at lunchtime could make you throw up 4 hours later, I wouldn't have gave it to you.

If I would have known that food allergies don't always show up on allergy test, I would have tried an elimination diet earlier.

If I would have known that anaphylaxis is not the only kind of life threatening allergy out there, I would have demanded faster treatment at the E.R.

If I would have known that your vomiting, bloody diarrhea, screaming, constipation, hair loss, pale color and passing out was from food, I would have eased your pain sooner.

If I would have known the juice I was mixing your medication into was the reason you were constipated, bleeding rectally, malnourished, vomiting, and bloated in first place, I would wouldn't have force fed it to you.

If I would have known that pediatricians, children's hospitals, specialist, and feeding clinics could all be wrong, I would have sought help elsewhere.

If I would have known that there were other kids like you out there, I would have tracked down their families and compared stories sooner.

If I would have known that the scopes weren't going to show much because the prep for the scope is to stop all FOOD days before the test, I wouldn't have put you through it.

If I would have known that not all kids with FPIES outgrow it, I would have prepared better for the long haul.

If I would have known that some kids have NO safe foods, I would have been less freaked out about only having 27 safe foods.

If I would have known that not all ingredients are listed on labels, since you can never be sure how each ingredient is derived, I would have called food companies earlier.

If I would have known, if the doctors would have known, if anyone I rambled on to would have known, it wouldn't have taken 6 years to diagnose you.

If I would have known that at 7 years old you would be in so much pain and feel so "not normal" that I would overhear you pray to go to heaven early, I would have explained sooner that there is no such thing as normal and reassured you that it will get better someday.

If I would have known how embarrassed you are about a medical need to wear pull-ups at 8 years old, I wouldn't have waited to tell you that I was a bed wetter until 5th grade.

If I would have known how isolating, scary, confusing, frustrating, and financially debilitating it is for families living with fpies, I would have reached out to give and receive help sooner.

If I would have known how many well-meaning people would offer you treats, I would have stopped them before you realized you were missing out.

If I would have known how many good people we have in our community, I would have asked for help sooner.

If I would have known there was a place we could get information, support and understanding, I would have contacted thefpiesfoundation.org sooner.

If I would have known you were suffering from food multiple food allergies, I would have found you help sooner, saw you smile more, and kept you safer. Because I love you, because you are my child, because we are in this together, we will be okay.

Love,
mommy

Please spread the word. It is Food Allergy Awareness Week and these kids and their families don't need to suffer. This is a rare type of reaction to food, and most of the medical community are not aware of it, or don't clearly understand all the symptoms.
If you ever asked how you could help or if you know and love Sarah, please repost this, like this or whatever....after all, I did just tell you that I peed my bed until fifth grade. :)

Allow time to process, and other tips to thrive...

April is "Caregiver Mental Health Month" at Complex Child E-Magazine.  A fellow FPIES mom (Cradlerocking Mama) wrote a great article about her Tips to Thrive for Parents of children with special needs.  I also recently wrote an article for their tube feeding awareness segments in February (Overcoming The Small Percentiles: Our Super Tubie).  It is a great e-magazine where parents submit real-life experience and insight articles to share among other families that have children with complex diagnosis.   FPIES is complex just from the shear nature of it, no test can define it, no test to find out what foods your child is allergic to, and then there are differences in how acute and chronic FPIES presents.

Insights and experiences on different and new ways to cope with this rare, and often times complex, allergy are shared graciously among the other parents going through this with us. There seems there is always something new to learn, even if you are expected to be a 'seasoned veteran". I remember the seasoned veterans on the babycenter boards when I was first logging in, how much more sure of themselves they seemed.  They had learned to trust their instincts, go with their gut, follow their child's lead, learned life outside of the restrictions that FPIES can initially put on your family/lifestyle.  Now, armed with the skills and tools needed, they were on to thriving with the new knowledge gained and new perspectives, but graciously continued to come back to the groups to share their insights and experiences, and even their continued frustration of some new challenge that was needing to be overcome.  I remember well something a fellow mom had said one day that stuck so well to where I was at the time....give yourself time to process. Allow time to process through the new stages, the new information, the changes that may need to be made in your home or lifestyle.  Not in a negative way, but in the 'give yourself time to process' and find your way to thrive scope. Give yourself time.  That is what I would add to CradleRocking mama's list.  When faced with a new diagnosis of any kind, or any stage in the journey, it is ok to give yourself time to process; allow yourself that.

Remember being new to FPIES?

Do you remember being new to FPIES?  I do. Our son wasn't diagnosed until he was just shy of his 1st birthday, and those months prior were some of the longest, most stressful and anxiety filled months we have endured.  Little Man is now 3.5yrs.old, FPIES is no longer a new diagnosis. We are versed in the changes that we needed to make to keep him safe, to learn about his allergies, to trial foods, and get support, to help friends and family understand and support us by respecting our sons needs. FPIES is still a big part of our Little Man's daily life as although his list of safe foods is growing,  his list of avoiding foods is still long enough.  

Recently, Little Man and our family got to meet another FPIES family who were in town receiving some further consults on their son's complex FPIES.  Their son has no known foods he can tolerate at this point. He survives, and thrives on an elemental formula for his sole nutrition. He is 3.5yrs. old, just a few months younger than Little Man. They were instant friends. They both noticed right away that they shared 'bobbles' (they both said bottle in the same cute way!).  And although it wasn't a big part of our meeting, they did both recognize that they shared "food makes my tummy hurt"'; which may not seem like a 'happy' thing to share- it is their reality, so having others to share that with is important for them.  

Little man has met other little friends with FPIES, but Little Michael had not. Little Michael had only recently even received the FPIES diagnosis (just before he turned 3yrs). His family had been managing his allergies under "multiple food protein intolerances" and since he was thriving on elemental formula since 4mo.old, and no one knew why food caused the symptoms they did- they were alone.  They were alone in trialing foods, alone in keeping him safe, alone in trying to have others understand, and explain to their growing son why he couldn't have food, they tried foods here and there- hoping and praying each time that they could find something that didn't cause excessive and irrational screaming, violent vomiting and days of diarrhea....not to mention how some foods also caused edema-like swelling and hospital stays. 

So, what would you say to you if you could go back to those first months of the diagnosis? What things helped you understand the most about FPIES?  What helped you cope? What helped you with food trials? What helped you help family and friends understand the magnitude of this allergy? What things helped you feel less alone?  

 These two boys share so much, and they knew it. 

Feeding Tube Awareness Week 2013

I recently wrote this article (also below) Complex Child E-Magazine; Overcoming the Small Percentiles: Our SuperTubie.

Along with writing the article, I made this YouTube video to illustrate the story. I've been a little behind on updating this blog so this article will certainly catch up the 'highlight reel', I hope to still fill in the details with my journal entries at some point. But, in the meantime I wanted to honor Feeding Tube Awareness Week 2013, and Complex Child called for those wishing to share their experiences, to write their story. I wanted to share our story since it it is not within any "norm" - with starting on TPN, going to an NGT, and now a blenderized diet with a G-Tube (due to not tolerating any commercial formula's). Little Man's FPIES is complex, although we have yet to figure out exactly why- but we just keep moving forward in any ways that we can. Overcoming the obstacles in front of us as they arise.

Our son is our Super Tubie! Even though he is only 3.5 years old, he has been on intravenous Total Parental Nutrition (TPN), a Nasogastric Tube (NG), and now Gastrostomy Tube (G tube). Samuel has a delayed food allergy of his intestines called Food Protein Induced Enterocolitis Syndrome (FPIES), and since even before his diagnosis at age one he was overcoming obstacles of small percentiles. From the small percentiles he had fallen to on the growth chart...to the small percentage of children so allergic to corn that he suffered chronic FPIES to even hypoallergenic formulas...to the small percent (according to studies) that cross react to multiple foods (dairy/soy and grains)...to the labs that fell to small percents causing iron and Vitamin C deficiencies necessitating short and long term TPN...and to his latest obstacle overcome--going from reacting to formula and severe aversion to his own G tube, to accepting it, and thriving with the help of a blenderized diet.

IV Nutrition (TPN)

Due to his allergy to the corn syrup in all formulas, the decision was made at 18 months to trial soy. This caused inflammation and enteropathy with blunted intestinal villi (villi are the finger-like projections in your intestines that absorb nutrients). Intestinal villi take additional calories to rebuild, and do not break down and absorb nutrients well when they are blunted. The enteropathy could not be turned around without the extra nutrition and calories that formula (that he was allergic to) provides and thus led to his first course of TPN. An upper-arm PICC line was surgically inserted and we lived for five weeks in the hospital while he received TPN for 20 hours a day. Just past his second birthday, eight months later, the combination of no nutritionally complete formula, and repeated food trial fails causing more intestinal damage and further malabsorption, we found ourselves in another emergent situation of needing TPN. Weighing the risks of central lines and infections, and then deciding that his need for the nutrients to bypass his intestines outweighed all of that was not an easy decision. However, he was very sick following his latest trial, and we had to proceed with the TPN again. We didn’t know how long he would need to be on it since he needed replacement of his nutrients, regrowth of damaged intestinal villi and gut rest. Samuel remained on TPN for eight months while he continued to have his limited diet of a few safe foods. After months of gut rest, and through more food trials and fails, TPN provided his body and brain with the needed nutrients. It also taught us how much his immune and gastrointestinal systems functioned and was literally a lifesaver, even through a life-threatening line infection and sepsis. After a second line infection scare that ended up being an FPIES triggered reaction, and due to repeated intestinal damage and noticeable regressive behaviors, we needed to make the choice to convert to an IV port to continue TPN or challenge a formula in his gut. It was decided to challenge his gut once more with another formula, a corn-free formula with hydrolyzed milk protein, Alimentum RTF, in hopes that we could finally find something to supplement his tiny menu. This is when we moved to the NG tube.

Temporary NG

The NG tube was a temporary stop in his feeding tube journey, only four weeks. It was placed to challenge the Alimentum RTF because he refused to drink it, rather starving himself than drinking it. The NG was very hard on him, flaring his sensory issues significantly, but he kept it for the four weeks while we made the very difficult decision to have a G tube placed surgically. All his medical team encouraged us that this was the right decision, but that it was ultimately our decision. No one knew whether he would start taking enough by mouth within the next six months so that he would not need the tube. We feared it would take longer than that, and did not want him to have the NG tube for six months, only to end up with the G tube anyway. The NG seemed more difficult to maintain, and besides flaring his sensory issues, it was limiting his play. A G tube would become part of him and he could play and be a three-year-old during the day. Plus, he would have the back up for feeding that he needed throughout the day and night.

Deciding on the G Tube

I am a Registered Dietetic Technician. I know the benefits a G tube can provide. My head told me the G tube was the right decision; it made all the clinical sense for a child with these food challenges. However, I am more importantly Samuel’s mom, and my heart wasn’t following my head. I am very aware that so many parents whose children need G tubes do not even get to make a decision, so why was this such a difficult decision for us? Sam could drink from his bottle, but the nutrients in his hemp milk formula were not complete to pull him out of reactions that damaged his villi and caused further malabsorption. Our son can drink and chew, but doesn’t know how to eat to fill himself. Due to his limited diet and pain with eating, he also has a multitude of texture challenges and food aversions to overcome. And that’s when we find enough safe foods to even practice on. He does have a few safe foods, but his allergy triggers outnumber them by far. He does not have a healthy relationship with food. Samuel was no longer failure to thrive but only because we fought it so hard. He is developmentally on track; however, his quality of life is diminished because he needs to eat every one to two hours all day and night and suffers low blood sugar in between. As he gets older and his sensory issues progressed, he would only take bottles at home, warmed to a certain temperature. Feeding him was becoming more and more challenging, and not just for nutrients, but purely enough calories. We knew the G tube would provide him that back up he needed, to provide the calories (and hopefully missing nutrients) he needed in a day. It would allow him to grow, play, and thrive outside of counting calories, and it would increase his quality of life. We were also encouraged being told that 90% of the families that make this decision later state that it was the best decision they made for the care and quality of life of their child.

FPIES Flares

Little did we know then that Samuel would again fall into those small percentiles. The first eight months after tube placement were rough for Samuel, and for us as a family. We once again went into crisis mode functioning as a family, where all the focus was on getting Sam through his symptoms each day. We just weren’t sure what was going on. We saw signs of trouble while he was on the NG tube, but chalked it up to sensory issues and not the tube itself, or blamed reactions to the lubricant they used to replace it time and time again. He had an endoscopy done during the G tube surgery that showed chronic gastritis and duodenitis--inflammation, again. But, what was this from? The lubricant? An increase in stomach acid from the NG? Was it the formula that we thought he had “passed” because he hadn’t had an “FPIES vomit?” It would take us a few more weeks to figure out that it was the formula, and it was making him very sick, inside and out. He was becoming averse to having us even touch his tube. We switched to another formula (ProViMin) in hopes it was the carbohydrate source and not the hydrolyzed milk protein in the Alimentum RTF that he was reacting to. His inflammation diminished in some places and flared in others. This formula wasn’t going to fit him either.

Stoma Troubles

We decided to take a bold move and stop the formula as well as stop using the tube altogether. The tube site or stoma needed to heal. We had trouble with it from the beginning, and he just needed it to heal. He needed to heal. He had woken up from surgery with a fever and quickly developed bile leaking out of his tube. We were granted a few extra days in the hospital to assure it wasn’t some sort of infection. It quickly went from bad to worse and the granulation tissue began to grow from the constant seeping bile/stomach acid. His shirt would rub and it would bleed. He was in so much pain. We tried creams and then repeated silver nitrate treatments (to burn off the granulation tissue), and each time the surgeon’s office tried to assure us that the burning did not hurt, and each time taking more people to hold him down because he was fighting this so badly. We later learned that granulation tissue does hurt, that rubbing against it hurts, and that silver nitrate removal can hurt some sensitive people. It was enough; this was “make it or break it” time with this tube. We had a love-hate relationship with this tube, which was quickly becoming more one-sided. I knew he could benefit from it if we could get it to heal, and find nutrients to put in it. I was determined to make this work for him, knowing the possibilities of the benefits. Remember, Samuel can drink and swallow, but his hemp milk formula and tiny menu were not nutritionally complete. We could never advance his diet quickly enough to avoid nutritional shortcomings. Stopping the formula he was reacting to, along with stopping using his tube was a big gamble that we would end up back on TPN too quickly. It was not an easy decision but we decided to let it be, through the summer, to see if it would heal. We didn’t do a lot that summer--my schedule revolved around his bottle and caloric needs 24/7. It was very important to stay on top of the calories he could safely have to keep him above the line and give that tube a chance to heal. Within days of stopping formula that was causing reactions and intestinal inflammation, the granulation tissue disappeared on its own and has never returned! The aversion to having his tube touched, to even HUG me or have his stomach touching anything didn’t go away as easily. We let him guide us. We took time away from even talking about it--taking a bath cleaned it well enough for us to leave it be--and hope for the best. My instincts were telling me that if we pushed him into using it, he would reject it altogether. Before stopping using it, he had gotten so hateful of it, even trying to pull it out himself! It hurt him so badly that he couldn’t even hug me. That didn’t seem right to me at all and was one of my last straws. I knew it shouldn’t be causing this much pain. People live with them every day and can hug others without pain.

The Blenderized Diet

What was my goal with a healed tube but no formula to put in it? Use the tube for a blenderized diet. This fall, once I felt Samuel was healed and beginning to accept his tube (our Mini Buddy helped with that!), we had home health care services come out to empower us to help him. Within a few visits and the nurse’s help and guidance, we were slowly able to get him more comfortable with his tube again. He accepted that it was part of him, and that it was there to help him grow big and strong. I began to use his G tube for his hemp milk and safe foods, blended by my Vitamix blender. Day by day, he lets us use it more and we are able to get his needed calories and more of his nutrients in daily. His menu is growing and we’re adding in the new foods as they pass to his blenderized bolus feeds to assure his nutrition is consistent and optimal for further growth and development. We are also able to keep him hydrated during/after reactions and keep him stable through the day without the ups and downs of inconsistent bottles. He sleeps better and longer at night. His quality of life, in spite of his chronic illness, is maximized. He has overcome so much in his three and a half years. He is our Super Tubie!

For when I am weak, I am strong....

"But He gives a greater grace. Therefore itsays, "God is opposed to the proud, but gives grace to the humble." James 4:6. 

I'm not a big 'scripture person", meaning I don't necessarily memorize or quote scripture but of course am familiar with passages and gospel stories and am reminded of them in so many area's of life, in the everyday.  God uses whatever means He needs to to speak to us- sometimes in the voice of our children, in the eyes of a small child, in the cry of a wounded soul, in the wake of tragedy are the times we are listening most for His voice-but He is always there.  I learned this as a small child, something my dear grandmother taught through her example. And it was impressionable on me. It was a gift-directly given from her but indirectly given from God....He knew I would need this gift.   

I came across this post today, "When you give up, and you break, you've made it.",from A Holy Experience on the heels of my last post trying to express what it feels like to be filled with graces, the graces to sustain, the graces to see the works before me, the graces to appreciate the painful days and even more so the graces to be so grateful and humble for the so.very.many.blessings.  

These words from the (above) post...."But here comes the upside, the so unbelievably bright side: when you are just done, and broken, and tired, you’ve made it.

You are now about to experience the most profound, amazing, life-altering, freedom and grace that will set you so free you are going to fly.

I mean it.

I mean it.

When you are broken enough and tired enough and angry enough that you just can’t mold yourself, fix yourself, do better, be better, when you are just done, grace is lavished on you like nothing you’ve ever experienced."

Wow. Wow. Read more, trust me.  Let the words wash over you.   

The title of the post isn't what grabbed my attention though.  I don't feel like I've given up- or that I am ready to.....but that doesn't mean I don't think about it when I am weak, or broken, or learning. 

What grabbed my attention in this post,was the first words...‘"I don’t want to be a servant –I want to be a Caroline.” So says the three year old wonder-child who humbles me as a parent and makes me think deeper about life."

My 3yr.old humbles me as a parent, too.  Last week, I was working on an article, and decided to also do a slideshow to illustrate along with the article (I love pictures!) for Feeding Tube Awareness Week 2013.  Little Man was watching the video with me, as I edited it.  We came to this picture:

And, he said- "mom, what's this" (pointing to the heart).  I said, "it's a heart".  He said, "no, this" (pointed to the same place). I said, "it's your feeding tube but inside is the heart, do you see it?".   He said, "no, that's not a heart- that is a kiss".  

Almost a year ago, he had that tube placed. Although I would say it was love- a symbol of love, of tough love at that time, but of love; I would not have said it was a kiss.  Today, through a multitude of graces, I know it is.  A kiss from an angel. A bridge to saving his life, to restoring health and quality of life in our little man. A way to get nutrients, medications, hydration in without constant chronic inflammatory reactions.

What strikes me from this post (above) is the first lines and how my own 3yr.old said those very words to me just yesterday. "I am not a supertubie, I am Sam.....I have a tube, see- right here (lifting his shirt and pointing to his gastric tube).  My tube is super. I am Sam".

Feeding Tube Awareness Foundation put a lot of thought into the Tubie logo, and it has amazing symbolism needed to bring compassion, understanding, empathy and most importantly, awareness to medically necessary feeding tubes. The word tubie in the logo was coined (and the logo trademarked by Feeding Tube Awareness Foundation) to bring the "human" to the tube (in the words from  Feeding Tube Awareness Founder for this awareness article on Complex Child EMagazine) "...logo embodies what we are trying to do.  Re-position tube feeding from something that is scary, icky and complicated to something that is friendly, approachable and beneficial--when it is medically necessary...... is why we chose the term "tubie" over something more clinical like "enteral feeding."  Words matter in communications..... The emotional connection is important, too.  It needs to be personalized.  The heart is pure tubie love."  

Beautiful. I thank Feeding Tube Awareness for this logo, this symbol of love.   


But in this instance, in a time when my mind is struggling with this chronic illness and the up and down effects on the quality of life of our family; it was simple words from a 3yr.old that reminded me of the importance of not naming the illness of a person.  The illness is not a person, the person has an illness.  The illness doesn't define the person, the person defines the illness.   Sam is a child with a severe food allergy of the gastrointestinal system, he has FPIES.  He is a child with FPIES.  He is not an FPIES child.  Can you hear the difference?   Medical Sociology 101: Do not define a person by their chronic illness.   Do not let a chronic illness define a person.   The medical field relies on the definitions and naming of an illness is to allow understanding of common symptoms, and to help define the illness by grouping symptoms.   However, in so doing, we risk taking out the individualism.  That very individualism that is needed in a complex subset of symptoms that ties together a syndrome called FPIES. 

Acidic Bile Vomit

The other day, I came across this picture....from January, a year ago.  I had washed it 2-3 times by the time I took this picture....and then threw it away.   The pillow looked dirty when it was clean.  I took the picture before I threw it away.  Why take the picture?  Because I am still in disbelief of how this vomit stains EVERYTHING! Before the FPIES diagnosis, or knowledge of anything FPIES- I threw away shirts with these same stains on them- both Little Man's and mine. I would throw them in the wash IMMEDIATELY -we always had a full load by the time the vomiting was done, all the towels it took to soak it up, the bed sheets, the clothes we were wearing, the washcloths used to clean up...and they would always stain.  The stains remain on the mattresses, the pillow themselves, as well as blankets and sheets we are not throwing away, and the some spots on our carpet....yellow vivid stains. A reminder when I change sheets of these long nights....

Bile vomit is very, very acidic. It stains everything. This pillow....this night...the vomit was scary. It made me think of all the times he was alone in his crib, not even waking up to vomit (the allergist who diagnosed him said this was indicative of shock- where they don't even...can't even, wake up to vomit).  This night, the story this pillow tells....a night in January when little man was (unknowingly) reacting to sunflower lecithin that had recently been added to his previously safe Living Harvest Tempt  Unflavored/Unsweetened Hemp milk.  It was 3 nights of green vomit around midnight before we figured out it was the hemp milk (that was previously safe when it did not contain sunflower lecithin).  This night, this last night before we discovered it was the sunflower lecithin  I had just laid my head down on the pillow next to him (hubby or I took turns sleeping with him while he was on TPN- IV nutrition, as his IV line was a central line, we didn't want him twisting and turning in the night and getting tangled in his lines).  I had just laid my head down and I heard this gushing sound...like the sound of the toilet overflowing. I jumped and looked towards the bathroom- expecting to see one of my other boys running out of the bathroom- trying to find help for an overflowing toilet.  Except as I sat up, I realized it was Little Man because he the gushing happened again- he wasn't even awake, barely lifting his head off the pillow to vomit this green substance.  I scooped him up and had him in the bathroom a few seconds later (right across the hall from his room) to finish emptying the contents of his stomach in the sink.   I just can't forget that sound....the gushing of vomit, and looking over at him to see his eyes not even open and more vomiting coming....FPIES sucks.

Why share this?   Why share this scary story?  Because this is the reality of an FPIES child, of an FPIES family.   The amount of vomiting that little man has experienced is not normal, not ok, not healthy.   The amount of vomit we have had to clean up, his brothers have had to watch him experience, we have had to help him through....all more than one child should have to endure for sure.  But all the stark reality of these children and families. Maybe this will change one day, maybe more will be known about the mechanisms of this allergic response and future generations will not have to go through so much....it begins with this, sharing our stories (yes, even the 'scary' ones), it starts with awareness of what FPIES is....

Which way are we going?

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

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

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

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

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

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

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

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

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

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

Sharing our story....

It was a decision to make our lives visible, our FPIES experiences known.  It was my first observation in this FPIES community, that there were more blogs and support groups than there was medical pages.  Sure, there were those handful of FPIES papers- mainly from Mt.Sinai where much of the FPIES research has come from over the years since Dr.Powell first coined this syndrome that had been noted by other doctors as a severe inflammation of the entire intestinal tract, causing vomiting, diarrhea, dehydration, often lethargy and sometimes bloody stools and shock symptoms.  There were a few moms, many of them also active in the support group on babycenter, that had started blogs - to share these everyday experiences, how they learned and their tools for thriving through this rare allergy.  In June, shortly after our formal diagnosis, I began this blog outlining our journey.  The good, the bad, the ugly. I quickly learned that as I was learning, this blog was becoming more about what I was learning- my inner working thoughts of how I was tying what I was reading in journal articles to my own son.   So, I started a 2nd blog- My FPIES File Cabinet, a resource page of sorts.  A place to keep those "research" posts filed, while continuing to chronicle the experiences we were having with our little man and his FPIES.  Since then, I have also joined with other moms and founded The FPIES Foundation.   I am passionate about sharing our story, sharing my personal insights seen through my clinical nutrition goggles; but also about sharing resources, tools, experiences, and insights - not only from experienced parents but experienced medical professionals.

From June of 2010 to today, August 29, 2012; I have logged 385 posts (and yet I'm still months behind) and have had over 35,000 page views (wish I would've figured out how to put that little page ticker on my footer at the beginning); it's been humbling to see how many have followed and shared in our story.  Sharing our story has become an important part of this ride, as others have done before and beside me- it helps.

"When you stand and share your story in an empowering way, your story will heal you and your story will heal somebody else"  Iyanla Vanzant.

With the busy-ness of 4 boys and summer, the complexities of FPIES, and my work with The FPIES Foundation, I have been a little more absent from updates, but do promise they are coming.  I've been keeping a journal as we go through this muddy mess we've been in with little man's FPIES as he approaches and passes 3yrs.old and still stuck, still adding new triggers and not outgrowing old ones...the updates are coming!   Overall, he is doing well, growing and gaining weight and meeting developmental milestones, and continuing to teach us about patience and appreciation for God's simple gifts in life.

In the meantime, I would be honored if you would check out the new website for The FPIES Foundation.   It is a 'labor of love', a resource packed with tools for new families and struggling families, a place to share stories and find support - a network of caring.  A place to come home.   We are not alone.

Lazy Days of Summer

Summer has kept us busy, just having all 4 boys at home is busy enough!  We are well into the lazy days of summer and enjoying just spending time at home. 

First day of school is Sept.4th, our Kindergartner has Meet The Teacher and then starts on Sept.6th- Kindergarten this year for him!  BIG step! He's excited, we're excited, he needs this in his routine, he needs the challenges and the routine base.  My mommy guilt cries for how his childhood has been molded by his brothers chronic health needs.  I know it has benefited him as well- he is empathetic and independent (sometimes TOO independent!) in ways his brothers weren't at this age but the 1:1 time I got with the two older boys just hasn't been there for him.   This is where I need to remember that everything that has been given to us, including Sam's challenging days, are God's Will and repeat to myself..."Thy will be done" many times a day; because I do simply trust in that -- even if my human nature takes over and my guilt or impatience creep in, trying to understand what role I need to take....which parts does He want me to do? Which parts do I leave to Him?  Which parts do I ask for help with?  These are my daily struggles....where my Faith meets my human nature.  

One morning last week, I woke up (as usual) to the sound of Little Man crying "Mommy, I need you"- he doesn't transition well and waking up from a nap or night is one of those times when he needs help with the transitioning; so if I don't wake before him- I am woken to crying out for me.  This one particular morning, I woke up still half in a dream...tried to pull myself out but it was "sticking" hard....I had been dreaming about Our Lady of Perpetual Help and I couldn't "shake" it...it just kept repeating in my head.  If you are familiar with the image of Our Lady of Perpetual Help (or look it up), you will see that it is the image of Our Lady holding the baby Jesus.  I have the image, in a frame....it was my Grandmothers.  

The prayer is about needing help and remembering to ask Our Lady, the mother of Jesus, for this help- as she will advocate to Jesus for us.  So, of course we continue to pray to Jesus for assistance but we also ask his Mother to take our cares and needs to Jesus for us by praying to Mary.   We honor Jesus in this way, respecting his Mother.  The prayer and image are here: http://www.ourcatholicprayers.com/novena-to-our-lady-of-perpetual-help.html

It is again a reminder for me to ask for help, even if I don't know what I'm asking for (that is my downfall, I have a hard time asking for help and even harder time if I don't know exactly what I need).   

Maybe that is why I haven't been able to write an update....I have a hard time discussing (especially with those unfamiliar with the day in and day out of this invisible illness) because there are so many 'hidden' challenges- things that we as a family or Sam goes through that isn't "normal" but isn't something anyone can help with either.  His body wont' accept food as nourishment and yet he needs nourishment to live and thrive...that's the bottom line of this viscous roller coaster ride we are on.  And making the right choices for that balance is my constant, constant thoughts.  Food that makes him sick vs. avoiding foods with nutrients his body needs. Teeter-totter, tilt-o-whirl, roller coaster ride. 

Bottle please, mommy

He ASKED for a bottle! Actually 3 times yesterday!  What music to my ears!  He asked for a bottle! (this is normal for him except in the last few weeks).   So, maybe we are turning a corner and he is healing or whatever it was that was the catalyst for this recent spiral is moving out of the picture?...

The other thing I changed was that I increased the hemp in his formula "recipe"- taking the recipe from the 18cal/oz to 21cal/oz (at least) - and he is feeling SO much better!  I did have to change the amount of ProViMin that goes in to the recipe (and haven't calculated out that yet), but this seems to be the break he needed in this spiral....he needs the calories more than the nutrients (so his body tells us) - and I had it the other way around.   The 'recipe" is too thick with either too much hemp or too much ProViMin (powder).   I will have to do some calculations over the next few days to see where he is now at with nutrients but I am just so relieved he is coming out of this "funk".   He has a few spots in the day where he is edgy but nothing compared to how he has been over the past 2-3 weeks.  Another situation where you kid yourself on how bad it is, just finding ways to get through it, until you're over the hump and looking back realize how hard it was - which means how "off" he was.   

I still am unsure what the catalyst was- whether it was the heat and then not getting enough to eat or if it was the work in the basement (mud and taping,etc) and the subsequent corn exposure it gave him.   His allergy is a gut allergy and we avoid corn ingestion like the plague.  In fact, he hasn't had a corn ingestion reaction (full vomit) since a multivitamin in Jan. 2011.  However, he's had corn contamination symptoms, and he's had an inhaled corn reaction (vomit).  This concerns me that his corn allergy is worsening, or that he has more than FPIES going on (which we have suspected all along anyway) - not that other kids don't experience inhalant FPIES symptoms as well but it is more commonly heard of with EGID (eosinophlic gastrointestinal disorders) - which are very close in nature to FPIES.  In fact, I've often wondered if he doesn't have some form of an EGID that has gone undetected because of how difficult/specific it can be to diagnosis - especially when we keep his diet so tightly controlled.  I don't know but inhalant allergy symptoms sure do bring things to another level.  And I've been noticing a pattern of this in the past - days when hubby mows the lawn and little man wants to ride on the tractor- he inhales all those fumes (yep, ethanol = corn), times when I've used a different laundry detergent and it has a perfume smell, he gets very pale/sheet white on these days...the smell alone - with all the corn, is making him sick.   

I asked this on one of the corn free forum's I follow and was actually a little surprised by the answer- how many people had actual experiences with house remodeling....and ER visits because of how severe it set off their corn allergy (people on these forums have varied degrees and forms of corn allergy from your typical allergy of hives/wheezing/etc to GI distress to neurological, emotional/mental effects,etc).  I follow these groups because it is at least a starting point to helping me learn and keeping our son safer.  And I am so very grateful for them. 

The dietitian let me know today that she was low on her numbers for his calories/ounce in his formula recipe- wow, that is a relief!!  Although it is still only 20cal/oz, it is better than the 18 she had mistakenly calculated it at.  She thought something must have been off so she went back and recalculated it.   She has a program for calculating it but the Hemp seeds aren't in that program- so she has to add those in by hand - and she was doing a full analysis of EVERY.SINGLE.NUTRIENT.   I'm glad she double checked, it makes me feel a little less sad about how low he went for those days - either because of the heat or because he was feeling poorly from the potential corn-y exposures....or (and likely) a combination of both.   

Whatever it was, we seem to be over the hump - and I know the prayers that went up were pivotal with this.  God has been trying to teach me this....that the answers to my prayers don't always come from my whisper to His ear but instead from my whisper to the hands (and knees) of family and friends....showing us that we are never alone....no matter how isolated we feel when no one knows how to help when he is sick.  

We are blessed. 

That Mommy Instinct once again...

Before little man started going downhill again, my mind was going towards next steps.  I still wasn't feeling quite like ProViMin was a sure bet just yet- until we saw some follow up labs and weight checks,etc. but did want to start preparing for some food trials- to build his diet, using the tube if possible -- for the trials and to get re-acclimated to the tube to utilize it to get his caloric needs in without obsessing about every ounce of formula and having to choose eating over daily activities.  

We couldn't stay with our heads in the sand for forever, it was time to move forward.   So, I had made phone calls to get a follow up with the dietitian, and a follow up with the GI.  I also called home health to get them on the radar that we would want some assistance with getting Sam used to his tube again.  A week went by and no one called us back (no one from the GI office).  Hubby made the follow up phone call- to see if we could get some attention.  By now, things were starting to fall and the trials were getting further from my mind....

We finally got the follow up appointments scheduled.  But now, we have new things to discuss with everyone because I'm noticing he is not picking up on his eating- even though the heat has broke.  I did a 3day "snapshot" check of how many ounces he was taking and it was around 28-32....I wasn't positive how many calories/oz his formula was (with ProViMin mixed into hemp milk) and was thinking it was around 20cal/oz....this is not enough calories.   Maybe this is why he is so tired?  But then, why doesn't he eat (drink) more?  I am offering a bottle every time I turn around it seems- and realistically, it's every 1.5-2hrs, and he accepting them every 2-3hrs. Something just is not right.   It appears (from his symptoms) that his anemia is coming back (low appetite, low energy, and disturbed sleep), but why? 

We went to the Dietitian appointment this week, and learned his formula is more like 18cal/oz.  I almost cried.  He is hungry, starving himself...but why?  Why is he starving himself?? The other time he self limited was when the food hurt him.  More specifically, the formula.  He did it with Elecare- starved himself into a hospital admission when he was 7mo.old; he did it with Neocate before we switched to Hemp milk, he was doing it with Alimentum and now again....is it the ProViMin or?? Either his appetite is really off because something is "disconnected" (his anemia or his carnitine or maybe even his thyroid?) or he is reacting to the ProViMin so he is self limiting- taking only when he is so hungry he can't not take it.   When you need ~1500 calories/day and instead you barely eat half that- what would cause that???  

He has a lab re-check in a week and a half but we decide we need to request to move it up, he is symptomatic and another week and a half he may need to be admitted to the hospital if we don't figure out how to help him.  

The lab draw was yesterday.....and it is concerning.   His anemia is back (why??), his protein stores are low (why??) and his Vit.D levels are low.  Well, an easy answer would be that he isn't getting enough but with the ProViMin being a powder - even though he's only taking in 30oz/day- he still gets the better portion of his nutrients from the ProViMin....certainly enough not to cause this.  Our Dietitian recommended we check a few other nutritional labs as well and they are within normal ranges so why would some be low and some be fine if he wasn't getting enough nutrition?  The answer seems to be that he isn't absorbing the nutrients, the iron is the same nutrient he malabsorbs when he has inflammation;  that the inflammation is in that place in his small intestine. And with protein stores being low, despite getting adequate protein intakes....he is malabosorbing.  But, why?  What is causing it?  Is it the accidental corn exposures?  Is it the inflammation from Alimentum not healing?  Is it from the ProViMin?  We don't know....we are hoping the GI will have some idea's but worried she may not.  

The pediatrician's recommendation is that he is clearly not getting enough nutrition on his current plan, and that he needs to be back on the Alimentum since that provided the nutrition he needs.   Quite frankly, this was poor advice.  For one, the ProViMin does meet all his needs.   The Dietitian has charted this, that he needs about a cup of ProViMin/day to meet 100% of every nutrient.   He is only getting 1/2cup right now (at best) but still getting a good amount of most of his nutrients.  The numbers don't add up....he should read the Dietitian's note so that he can give informed recommendations, to help us make informed and the best-interest-for-our son decisions.  The Alimentum took over 50oz/day to meet his nutrient requirements-- and he wasn't drinking that much...and yet he was malabsorbing even more.  He was not tolerating the Alimentum, and his body was rejecting it as quickly as it was taking it in....causing more damage to his stores than if he wasn't taking it at all!   How do we know this?  His behavior, his mood, his appetite, his sleep, his crying, his pale skin, his blue around his lips, his labs, his dramatic decline in iron stores and hemoglobin, his terrible-terrible looking and painful, leaking tube, his no weight gain, his volume-ness stools, and the last telling sign was the analysis of those stools, which revealed a high alpha1 antitripsin level and a high calprotection - indicating malabsorption of proteins and significant inflammation and enteropathy.   

His color improved on the ProViMin, his mood, his behavior.  His diapers have not.  My suspicion is that the Alimentum caused significant villi damage and that the ProViMin was working- when I was able to get enough in him, but with the heat and his decline in intakes, he started falling behind....and now we're seeing the (quick) effects of that.  

Either that, or he's reacting to the ProViMin- something subtle...  

So, the plan?  We need to push the ProViMin and see if things get worse or better.   He won't drink more so we will need to use the tube- it's make it or break it time with this tube.  We had our home health nurse come by on Thursday to see if he would allow her to check on his tube and even use it - and he did!!  It took a bit of coaxing but it ultimately went smoother than any of us anticipated!  We are encouraged that since he now doesn't have constant pain with it, that he can start using it again.   We will plan to give him whatever feeds he needs above his formula intakes, we hope to be able to do this at home but worry that the GI will want him admitted.   When he first came home from the hospital after having his Gtube surgery, he had the extension hooked up 24/7- there is a "button" in his stomach wall, that gets opened and an extension- the tube- hooked up to it for 'feeds'.   We took that extension off because it was pulling (and possibly preventing healing) and the tape was causing a reaction to his skin (painful).  Getting him hooked up for feeds since has been a challenge.  So, we may try this method again for a few days/week to see if we can get him back on track....or it will make it clear that ProViMin is a problem. 

Is it just the heat?

Little Man had been doing well, then the last week of June brought really, really hot days....and we tried to stay cool; but it wasn't enough.  His appetite tanked, and it became my full time job (not that it isn't already) to push more and more with the bottles.  I was worried that he wasn't even taking enough to stay hydrated, much less for his nutritional needs.   And I prayed for a break in the heat and we kept the air on and kept him inside and tried to keep our activities low key.   And I wondered why he was responding so negatively to the heat? He would sweat within 2 minutes of being outside, and 15minutes would have him drenched in sweat!?! 

We enjoyed his brothers 5th birthday party- a backyard BBQ- and he was doing primarily well that day....it was the days that followed that he started to decline.    Was it the heat?  Was it being too busy all week preparing for the party and having church school and going, going?  Or, did he get a corn exposure?   

Something isn't right because he clearly started going downhill that week....maybe he didn't recover from the IV iron (the dextran that is corn), maybe his body isn't recovering from the Alimentum inflammation?, maybe it's something else in his diet, maybe it is the ProViMin,...

I'm getting worried....one of the last times we spoke to our GI she relayed that she was "getting frustrated" that we don't understand why his gut doesn't function properly; and that she wanted to have him admitted (somewhere, not necessarily here) that scares me.   It scares me to have him admitted here because they always set him back, it seems no one even tries to understand his FPIES and the mechanisms that happen in his body. And getting admitted someplace else?   Where?  Where could we travel, be away from the boys, spend time away from my family, and work, and put him through more and more tests and get actual real help and answers?   Where??  Where would we go that we don't get the overwhelmed door shut in our face, travel how far to say there is nothing else to do, nothing else to help him....

So, with all of this weighing heavily on my mind, and little man clearly not getting better- even with the break in the heat, his appetite was not picking back up and his energy was really low.  His sleep was getting more out of whack- not waking in his sleep in pain, but unable to fall asleep or sleep very long. Something is off, what is it?   

The G-Tube....the other 10%?

What are we doing with the G-tube?   Does he still have it? Why, if we're not using it?   Why, if he doesn't need it?  

And, then my own guilt questions:  did we make the wrong choice for him?  He's now drinking the formula, does he need the tube at all?   We put him through so much and the benefits have been so few from the tube- do we stop now and take our losses or push things and hope we can turn it around?  

When making the decision for the tube, what we were looking at is a child that struggles with weight gain, struggles with foods, gets his nutrition from a formula- that he will only drink from a bottle (at a certain temperature with minimal distractions), that he would not drink the supplemental formula (at that time) that he needed to not have TPN, that we could utilize it to do food trials in a child that struggles with textures and new foods that might make him sick, that we could increase his (and the families) quality of life by taking the stress and worry off of how much he drinks from the bottle or nibbles from his few safe foods and allowing us to bolus feed him if/when he doesn't consume enough calories or nutrients from safe foods during the day to get him through this illness thriving.  

We made the decision to go ahead with the Gtube, to get rid of the NG tube so he could still get the formula.   The formula that caused enteropathy in his intestines, the formula that he then started drinking in his bottles, the formula that we now have put on the reaction list, and switched to another formula that he does seem to be tolerating and is also drinking in his bottles.  

We were encouraged by all his team that this was the right decision but that it was ultimately our decision (that we could just continue the NG tube for ~6mo and hope that in that 6mo.,we could move his diet up enough to not need any formula or tube).  We did not want him to have the NG tube for 6mo- this was very hard on him because he couldn't play with his brothers.  A Gtube would become part of him and he could play and be a 2/3yr.old during the day;and have the "back up" for feeding that he needed.    We were encouraged that "90% of the families that make this decision, 6mo. after the tube is placed relate that it was the best decision they made for the care and quality of life for their child". 

So what about those other 10%?.  Right now, we're that other 10%....but I don't want to stay in that 10%- so how do we change that?   Well, he's drinking the formula so that reason for the tube isn't a priority anymore- although it would be an increase of his quality of life to give him bolus feeds of this formula so he is less dependent on the bottles and increased flexibility of our schedule and his playing (the job of being 3).  He needs a lot of the formula, at this point, to restore his nutrient levels and if he has any future food fails- he will need increased amounts of it then too as villi damage (small intestine) is part of his FPIES reactions. The tube would also help keep him hydrated should he have a vomit/diarrhea FPIES reaction (if we pushed a food that far). 

The Alimentum RTF caused problems before the Gtube was even inserted.   He woke up from surgery with a cough and fever, green pussy drainage from the tube from day one and granulation tissue very early on.    The green, yellow, and brown drainage never let up.....until we backed off on the Alimentum.   The tube was getting too painful for him to allow us to use it, and the burning off of the silver nitrate caused a strong aversion sense to having the tube.   So, we backed off of using it....this is what helped us see that the that symptoms were correlated with the Alimentum- but we still weren't sure what from.  The longer breaks we took, the more clear that became.  But now, he was already so averse to the tube....

If we can get home health to help us help him be comfortable with USING his tube again and then we can use it for his bolus/supplemental feeds and for food trials as we expand his menu and catch him up to where his menu should be by now- it will make it worth it...I guess I'm giving conditions of MY coping with this decision made for him....that might not be wise, as none of this may work out at all but I have to at least try. 

Murphy's Law

It seems like whenever I saw something, it happens.  I was dragging my feet (or burying my head in the sand) to make phone calls to the care team.  Any mom of a chronically ill child will relate that it is a lot of work to coordinate everything, and sometimes you just wish someone could help with that.   In the past, I've had a misconstrued idea of what that entailed for a chronic illness....my perception was that preventive care was the goal.  That, with known chronic issues surrounding a health illness, it would be beneficial to stay on top of problems that repeatedly come up, to steer care in the direction of health.   I'm serious, even if that sounds like a sarcastic tone (those reading this that know better)- I honestly had an expectation far from the one we experience with little man's chronic health needs.

So, our complex care nurse called yesterday morning to let me know that our pediatrician looked at little mans labs and was very pleased with how they looked that indicated he responded well to the IV iron.  She then relayed that "he would like to see him back on the Alimentum as it seemed to provide the necessary nutrition while working on additional foods by mouth".  I literally chuckled outloud...I had to catch myself and back up because I didn't mean to sound disrespectful and had to remind myself that the Pediatrician had told us he would be on on vacation this week- he is checking these labs from home out of his concern for our little man and follow up....and he must've just forgotten that we are not on the Alimentum, that that was causing enteropathy and caused this dramatic decrease in iron and hemoglobin.   So, I told her that yes- we would continue the ProViMin but he would not be getting the Alimentum.   I go on to tell her my thoughts on where he is at right now and where our plans are for him in upcoming weeks, who I need to call or coordinate discussions with....saying, I am also very pleased that his labs look so good- that his body responded so well to the IV iron and that he must be tolerating the ProViMin well too because he has never responded this well to the IV iron before (ProViMin helping him stay ahead of the line).  I am very encouraged by that.  This is also because we have been pushing the bottles- so that he gets as much of the ProViMin that he can during the day, which means less eating, even of his safe foods- because he isn't as hungry because I've just had him take a bottle (every 1.5-2hrs pretty much around the clock).  The ProViMin is a powder and it is mixed with his hemp milk and a few weeks ago, I asked the dietitian what the iron content in it was -- he gets over 2-4x the iron his body needs (if he didn't have decreased stores, she wasn't positive what his needs would be to replenish stores and deferred that discussion to our GI), but also that it provides well over the protein that he needs in a day as well...so we are hitting those numbers well (which is good because of the enteropathy, he was dumping proteins in his stool and his stores were low).   So, we've been pushing the ProViMin to get these (iron and protein) levels restored, as long as he tolerates it; and to allow his gut the chance to heal from the Alimentum so we can do food trials again soon.   We need a follow up with the dietitian to find out where he is at now that I have the recipe of the ProViMin and hemp milk and how much he takes in a day all worked out and to help decide a list of foods for the upcoming trails. 

I also discussed how he hasn't been sleeping well at night and having some moodiness during the day that we aren't sure what it is from but doesn't seem to be from foods that we can corelate so I was curious if the genticist thought if it was from his low carnitine levels again.  Carnintine is an amino acid found in meats and dairy - which he does not get; it is supplied in formula's but there is none in hemp milk....also none in TPN (which is when we first discovered he was carnintine deficient).  Carnitine helps keep blood sugars stable and so with a low carnitine- maybe his body isn't regulating his blood sugars as well.  The role of carnitine and the effects of the deficiency is much more complex than that but that is what we saw most from Sam being affected by being deficient.   

We also need a follow up with the GI doctor- to discuss further this enteropathy and how he is doing now, and to get a letter of medical necessity for the ProViMin so we can see if Insurance will help cover the costs; and discuss next steps regarding foods trials and the tube.  

I also need to call the home health agency we work with and find out if they can come out for some regular visits to see if we can get Sam acclimated to his tube again - utilizing nursing support initially and then adjusting to us using it and him being comfortable with that.  Even if we just do 1-2feeds/day and we do that to give him the missing calories or nutrients in his day - so we can have less focus on a bottle every 2hrs, and more focus on allowing him to EAT (safe foods and trying new things).   And to do this- we would use his tube to give him a "feed" with the nurse but to also trial some foods- to expand his choices on his menu (and then later work on getting him to eat these foods that we determine as safe).  

He also needs a weight check and his 3yr.well child visit.  

I relay all of this to our complex care nurse.   She says, ok and goodbye.   

One message, one internal note cc:d to all his providers for her to send to almost all of his care team would save me multiple phone calls and get everyone on the same page at the same time, coordinate everyone's insights and recommendations and would be the ideal thing for him in this patient-centered care environment we are supposed to be belong too.  But, no- she's only there to pass along the message from the Pediatrician.  I know it's all my job, he's my son- I just can't help but wish we had more help from time to time; so I could be more mom and less nurse. 

Well, at least I ran through my own mental list now...so I can get started some phone calls.....head out of sand- here we go....