Friday, February 28, 2014

Rare Disease and Families

Today is Rare Disease Day and its purpose is to raise awareness about rare diseases and how they impact people’s lives.  FPIES is considered a rare disease and sadly there is little research being done to learn about it. Since this disease and many others are misunderstood and follow a constellation of symptoms that vary from child to child there seems to be a terrible trend happening with diseases like FPIES, EoE, Mast Cell and Mitochondrial Disease.  Parents who are saying no to “standard” treatments like an endoscopy, steroid use and a delayed vaccination schedule are being looked at as “medically endangering” their child. Sometimes state child protective services are being called in to investigate the health and wellbeing of the child. I am a veteran social worker, I carry the highest licensure possible in my state and I know how this works.  There is a need to protect children and families from abuse and neglect and there is certainly and illness that is real called Munchausen by proxy syndrome (MBPS) however it too is rare.  I can’t tell you how many FPIES parents are or have been investigated by CPS because their children are FTT (Failure to Thrive) or can only eat a handful of foods or who can only handle pureed foods at age 3 because of oral aversions or because they say no to medical tests that require sedation and those results may or may not impact current treatment planning. Parents are being accused of making up or exaggerating the symptoms or reactions of their child or children. Our beautiful boy has FPIES, our family knows exactly what it’s like to live it 24/7/365 and our doctors don’t.  They don’t see the neurological side effects our son has or the vomiting to a state of shock he has, the lethargy, allergy shiners, slap cheeks and the list goes on. Don’t get me wrong we have a wonderful team of specialists that Jack sees and they have been supportive and very helpful.  It appears at least for now we are in the minority and this really isn’t the case for many families dealing with FPIES and other rare diseases. 

Dealing with a child who has a rare disease is incredibly lonely and scary.  With FPIES, simple things bring fear and anxiety.  New food trialing with Jack leaves the whole family walking on egg shells.  Jack is doing beautifully now as far as eye contact and socializing.  He is playing well and interacting more with his twin brother.  He is seeking us out for comfort and his smile; it is luminous and melts our hearts. He is laughing and giggling like crazy! There is no diarrhea, no vomiting, no lethargy, allergy shiners or slap cheeks. He’s happy and content most of the time.  Can you blame us for not wanting to trial anymore foods for a LONG, LONG time? Not wanting to gamble and risk sickening our child by giving him say a banana or pork or broccoli? Yet the pressure is there to add more foods to his diet from his doctors.  Why are the doctor’s opinions more valid than ours as the parents? We know our baby; we know what happens when he fails a food and it’s horrendous.  What is the rush to add more food to his diet? He’s got oral aversions so we are desperately trying to work on increasing texture too. Does it really matter if he eats peaches before he’s two? 

We are thrilled with our GI and Allergist and they partner with us but I still feel the pressure to increase the number of foods in his diet. I am not sure how much of this is real or imagined. I do know that when we visit them in a few weeks we may or may not have a new food pass from the last visit we had.  Jack’s stomach flu thwarted our pear trial.  We hope to pick back up the beginning of next week again.  Doctors and parents need to work together and listen to one another. It’s a partnership and one side is not more dominant than the other however the final decision always goes to the parents. This is about the best interest of the child, it’s not about ego or protocol, and it’s a case by case basis, what works for Jack may or may not work for the next patient.  I hope whatever is happening with rare diseases, doctors, parents and CPS ends soon.  These investigations are causing undue trauma, stress and anxiety. Do doctors need to be mindful of MBPS, yes of course but the number of reports to CPS is disproportionately high as far as I can tell in families where rare diseases exist. CPS needs to focus their time and attention where it is most needed, with kids and families who are dealing with sexual abuse, physical, mental abuse, domestic violence and neglect (among many other things.) For the most part (because nothing is ever 100%) families living with rare diseases are just trying to do the right thing with no road map, and no framework to go by to keep their child safe, healthy and pain free.

Friday, February 21, 2014

The Stomach Bug

On Friday February 14, 2014 our baby boy Sean began with diarrhea and few short hours later he was vomiting. 24 hours later it hit Jack with a vengeance, then 5 hours after Jack I was hit and 15 hours after me Grammy was down. The babysitter got it too. Can I tell you I haven’t been this sick in almost 20 years?! It was horrendous.  I have no idea how my wife was able to dodge this bullet, but she did. 

By Sunday evening I was feeling “OK” and Sean seemed a bit better, Grammy was down for the count. Jack was lethargic, still vomiting, very fussy and not producing enough wet diapers.  We spoke with the nurse on call at the pediatrician’s office and at 5:00 pm Momma and Jack went off to the local ER. This is the first time Jackson and Sean have had a severe illness. I am a stay at home mom and because of Jack’s FPIES we have worked very hard to keep them healthy and away from major illnesses.  Heather and I were ready to engage our special emergency treatment plan for Jack.  This includes a letter from his allergist Dr. Wayne Schreffler at Mass General Hospital and a Corn Allergy Emergency Medical Instruction Template given to me by a friend who works in the medical community who also has food sensitivity reactions to corn and other foods.

Once again I will say, almost everything has CORN! GAH! It’s so incredibly frustrating. Jack could not have the numbing agent when he had his IV places because it has 3 ingredients in it with CORN. We were not interested in finding out while Jack is puking his guts out if he’s going to have a skin reaction or break out in hives etc., to an injection filled with corn derivatives.  Heather presented all of the documentation in the triage area of the ER and she cannot count how many times she had to remind everyone that he could not have anything with corn.  “Oh, we will just give him Zofran now under his tongue to stop the vomiting.” “No! He cannot have ANY oral medications that are not compounded specifically for him.” “We will start him on Pedialyte.” “NO, he cannot have regular Pedialyte, we have to make it at home with his safe CORN FREE sugar and salt.” “We will give him an IV with saline and dextrose.” “NNNNNNNNNNNNNOOOOOOOOOOOOOOOO! HE CANNOT TOLERATE ANYTHING WITH CORN IN IT AT ALL! NONE! He can’t eat it, drink it, take it in his veins, have a suppository with it or have an injection of it under his skin. NO CORN, ZERO! It gets very frustrating and you cannot let your guard down for one second.  After an IV bag of corn free saline Jack comes home and sleeps from 11:30 PM to 5:00 AM.  He wakes in a good mood and is playing a bit. He gets fussier as the day goes on and he can’t seem to settle for a nap but he does a huge, great poop so we think he’s on the mend. I speak with his GI, Dr. Qian Yuan at MGH and let him know what’s happening with Jack.

9:00 PM Jack begins to vomit uncontrollably, I am holding him and I can hear it coming again.  I throw the towel on the floor and turn him towards it while he is retching from his toes. My heart is breaking. It’s a ton of throw up and I look down and there are brown mucous chunks – blood. I check his diaper and it’s dry. I page Dr. Yuan, he says get to the ER. I load him in the car and we go. When we get there I pull out all of Jack’s paperwork again. He gets the big red band that says allergic to corn and acetaminophen. I go through the FPIES story again and again – the receptionist, triage nurse, nurse, ER doc – no one has ever heard of FPIES. One again I refuse any oral meds, suppositories, everything but a simple saline IV.  The nurse we have is amazing. She scans and copies Jack’s forms and reads all of them. She tells me her daughter has colitis and can’t have dairy or soy.  She tells Jack exactly what she is doing and when it’s going to hurt. She’s very gentle and professional.  Jack is already traumatized by his last ER trip and he moans and cries.  Once the corn free Zofran is administered in his IV, he falls asleep. The ER doc informs me he is going to contact the in-house pediatrician, she arrives about 30 minutes later.  I begin my spiel about FPIES and she knows about it!!! She was a resident at MGH and knows Jack’s GI!!! She gets him on the phone immediately and they begin the transport discussion, she feels Jack’s needs will be better served on the pedi GI floor at Mass General.  I am exhausted and still recovering from this bug too and at this point as it’s about 1:00 AM.  Heather agrees to come to the hospital and ride with Jack in the ambulance. She arrives and they load into the ambulance at 3:00 am.  I get home, shower and talk to Heather. I fall asleep at 5:30 am and am up with Sean at 7:30 am. I talk to Heather and then make Jack’s safe pedialyte and head into Mass General.

Jack is miserable. He hates being in the hospital and he cries anytime someone medical looking comes into the room. He’s hungry, exhausted and just wants to be held or lay down in the baby jail crib (mostly just held.) I hold him while Heather sleeps.  The doctor arrives and says they feel Jack doesn’t have a major GI bleed.  It was most likely a small tear in the lower part of the esophagus that was already weak from the excess acid he already has due to his FPIES.   The vomiting over all those hours and days was just too much.  He’s starting to take watered down formula.  He is going to stay another night to make sure all is going to stay down.  His labs have come back again, normal. We are relieved it seems to be a minor issue but we are in one of the best hospitals in the country on a GI unit and they still suggest options for Jack that contain corn. They want to give him oral Nexium when we leave (he’s had it in his IV,) guess what? Yeah, it’s made with corn.

This hospital stay highlighted a few things for Heather and I. The first is that Jack is medically fragile when it comes to FPIES and getting a GI virus or bacteria. It hit him incredibly hard.  He got much sicker and it was a lot quicker than any of us. It also highlighted how little the BEST medical professionals in the country know and understand about FPIES.  There is so much education, advocacy and research that needs to be done. Lastly once again Jack is a tough, tenacious little boy.  He defiantly suffered some medical trauma that we will work through together. Jack inspires us to be better and do better. We will not fall victim to FPIES, we will rise up in pure defiance and do everything we can for our son and to make every medical professional we encounter, FPIES WISE.

Tuesday, February 11, 2014


The word tenacious as defined by The Merriam-Webster Dictionary,

te·na·cious adjective \tə-ˈnā-shəs\

: not easily stopped or pulled apart : firm or strong

: continuing for a long time

: very determined to do something

A tenacious spirit or tenacity is a quality I see in every child I am connected to with an FPIES diagnosis. Of course I see this in my own son, but there are so many impressive kiddos in this community. Almost all of Jack’s FPIES buddies are far away from us.  Actually, he’s never met any of his buddies in person and I haven’t met their parents in person either, yet we are a fierce and tight knit community. There are so many examples of tenacity I could share, but here I will highlight only a small few.

S is a 4 year old boy who lives in the Midwest. He likes to cross country ski with his family.  This week he was out on the trails and need a snack, so right there in the snow he had a liquid bolus feed through his feeding tub and then off he went on the trails. FPIES doesn’t stop S from living and enjoying time with his family, he rocks on!

M is a 5 year old boy who lives here in New England, (we hope to meet soon in person) he likes to hit the slopes.  He’s been trialing some foods and struggling with nutrition recently.  Has this stopped him from skiing with his dad? Nope! Even when he’s exhausted and falling down he wants to keep going, Mom and Dad have to pull him off the slopes.  M is a free spirit and one of the happiest kiddos out there, he rocks on!

W is 2.5 and lives out West.  He’s really struggling right now with a systemic infection. He has no safe foods and his formula is questionable. The medication he needs for his infection makes him react; it’s a very difficult situation.  He was feeling very crummy the last few days and the family was going on an adventure to the Dinosaur “park”.  Mom decided they were going to go to give W a break from the usual.  He loved being there and didn’t want to leave! Mom also held him or “wore” him the entire time. W also participates in extensive therapies weekly to help with his overall development, mom reports even on his hardest reaction days; W is participating in therapy and making strides. He rocks on!

S lives in the Northeast and is 9, she’s been losing what were safe foods recently due to an inflamed gut. This is very hard for a young school girl, who just wants to eat normally like all of her peers. S is incredibly creative and her mother recently posted a video of S in an elaborate pretend play scenario. S was cute, dressed for the part and quick witted. By the way, she made this video all by herself.  She rocks on!

FPIES isn’t rainbows and butterflies, but man do we have kids who teach us daily what it means to live, love and be in the present moment.  Behind every tenacious child with FPIES is a parent, grandparent or caregiver with just as much strength, tenacity and courage. Together we rock on!

Saturday, February 8, 2014

Trauma and FPIES

I read a wonderful short article in Sojourner on-line this week written by Catherine Woodiwiss titled A New Normal: Then Things I’ve Learned About Trauma (
The very first thing she discusses is how a traumatic experience or long term trauma permanently changes us. Trauma changes the fabric of our being.  It affects how you interpret the world.  Living with FPIES is a traumatic experience for the entire family.  While Jackson deals with pain, vomiting, diarrhea, constipation and mucous filled stool, limited food choices, texture aversions, no ability to commutate with words his pain and discomfort (the list goes on) during post exposure, my wife Heather and I have our own traumatic experiences too. (My mother does as well as we live with her and so does Sean, he doesn't like when his brother is very sad, he often will cry in distress when Jack does.) We watch and hear our child in various levels of discomfort and there are times where there is little that will comfort him.  We have anxiety responses to foods.  Just thinking about a new food trial is scary.  How do you choose? We don’t want to give him something that will make him sick and hurt and yet that is what we have to do in order to know if he can have the food.  It’s incredibly counterintuitive. I’m literally afraid of pears right now as they will be his next trial. Today we tried broccoli.  The poor guy was so overwhelmed by the texture and taste of a minuscule amount he gaged and vomited. (Not an FPIES reaction.) We have shelved broccoli. The doctors gave us a list of food families and we are to pick from the same families he has passes in with a few more options outside those as well, dairy is off the table until he is over 3, eggs until about 2.  The highest FPIES trigger foods are dairy, rice, soy, barley, peas, green beans, sweet potatoes, squash and all poultry.  This is not an exhaustive list and honestly in my experience someone can be FPIES to any food on the planet. Jack can have peas, squash, sweet potato and chicken on that list.  He failed green beans horribly, just like the corn. Barley, rice, dairy and soy are not in the cards right now. Just think for a moment what it feels like being afraid to give your baby food? Not just a certain food like eggs or peanuts but almost ALL foods.

We no longer know what life is like without FPIES. This is our new normal. What used to be thoughtless activities are now front and center and frankly exhausting.  Leaving the home with the boys is a painstaking process.  I pack snacks for Sean and foods that are safe for Jack (mostly still baby food purees), formula and bottles.  We haven’t been on a playdate since the summer because we don’t want Jack to get sick.  He cannot have antibiotics like most kids.  Jack cannot tolerate the corn fillers in them, ALL of them.  Having antibiotics compounded is often counterproductive because it can take days to get them. Our best option is a shot of an antibiotic called Rocephin.  Hopefully our pedi will have it in stock when we need it, you always run the risk of it not being there for him on a day he might need it. Jack can’t use finger paints, crayons or play with play-doh, you guessed it: they are made with corn. I blow bubbles with him and then wash his hands immediately. We are planning to take a trip to Michigan in June for our niece’s graduation party, this is a terrifying thought. First of all, flying with 20 month old twins, enough said. But how are we going to keep him safe in someone else’s home? Can we get all his safe foods in Michigan? I have to research all this before we go. Our family is wonderful but they don’t live with this daily like we do.  It’s quite an adjustment and I know they are up for the challenge because they love Jack. I’m guessing that many of you reading this who don’t live with FPIES are maybe feeling stressed, overwhelmed by the thought of all this? You should be! It’s incredibly intense!
Where is the good news in all of this? Heather and I have found strength we never knew possible. We move with courage every day. We have the joy that is Jackson Harris and the joy that is Sean Timothy.  Jack has FPIES, we can’t change it but we sure as hell can try to heal him of it.  We work with a great medical team and are seeking information on holistic and biomed approaches to treating and healing FPIES. I believe the cure to FPIES is where western medicine meets eastern medicine and there isn’t one answer that is right for all. Living with FPIES isn’t easy or fun but it’s a gift. It brings new learning with it daily.  It brings all your “crap” to the top.  Any old wounds or traumas you haven’t addressed – HELLO. It taxes your marriage more than just having children does. Heather and I are learning to work more closely as a team. Some of your friends think you have fallen off the face of the earth or that you are “too over protective “of your children. I assure you, if you’d seen and lived with what we have, you’d know this wasn’t the case. Our dear friends have risen to the top as they always do. My FPIES girls always have my back. (Heather isn’t able to research and go on the boards because she works 40-50 hours a week.) These are only some of the gifts of the trauma that is FPIES. So there you have it:  the good, the bad and the ugly, the wisdom, strength and courage that comes with living with FPIES and the most courageous and wise of us all in this experience is and always will be our son, Jack.

Monday, February 3, 2014

It takes a village!

UPDATE: Sadly Nutricia is only going back to an older version of Neocate Jr. for Jamus. While this is wonderful for his family, it's incredibly short sighted. There are many more families who need this version and even older. This is their official statement.

 I am thrilled to say that through the hard work and dedication of many, The FPIES Foundation announced today that Nutricia is re-starting production of the old label Neocate Elemental Formula in the next 90 days. They will continue to make the new version and also smaller batches or the old for those families who must have it for their children's health and main nutrition source. Erin F. & Robyn have been warriors in the process. Jamus, Robyn's son has been at the center of this challenge. The FPIES community and the EoE community are beyond thrilled and will sleep well tonight knowing that their child(ren)'s only food source is not going to disappear. If your child needs the old label Neocate please contact the nutrition specialists at Nutricia directly.  It takes a village! Thank you to all who supported this endeavor. Now on to Fighting For Mack with the makers of Elecare.

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