In honor of National FPIES Awareness Day on May 4th
The moment those double pink lines revealed themselves we were overcome with joy. Expectations, dreams, and excitement surrounded us. Being the third child we would bring into our family gave me confidence. Having previous experience took some of the nerves away. I already knew we would breastfeed, co-sleep, baby-wear, and follow the baby led weaning method for foods. No need to buy bottles. No need to buy special equipment to prepare purees. We had a plan. We had a proven system. The hard part of trial and error testing parenting was over. What could possibly go wrong?
I will admit, I didn’t research much of anything; why would I? I was a pro! The pregnancy was bumpy as I suffered from Hyperemesis Gravidarum (HG), a severe form of morning sickness. This left me bedridden and caused me to lose weight when I should have been gaining. Medications and Powerade were my saving graces. Later in the pregnancy, I was diagnosed with Gestational Diabetes (GD). Unfortunately, because of my HG I was unable to control my GD with diet changes alone which led to more frequent monitoring, non-stress tests, ultrasounds…and more medications. Despite some pre-labor scares and modified bedrest, I counted down to meeting my precious boy and thanked God that he was healthy and we were going to be okay. It would all be over soon and the perfectly messy journey of being his mom would begin.
When induction day arrived I was equally excited to meet our new addition as I was to end this sickness hell I had been living and finally EAT! When his blood sugar levels came back normal and he seemed to take to breastfeeding easily it seemed like smooth sailing from there. He was happy and content; he actually let us put him down to sleep! He was PERFECTION at 7 lbs 14 oz and 21.5 inches!
On our second night in the hospital, he started making an odd gurgling sound in his sleep. We asked nurses and doctors but were assured that these were “normal newborn” sounds. So, we eagerly brought our little guy home and he was greeted by his adoring sisters. Life was beautiful and we were incredibly blessed.
The next day at his pediatrician appointment we found out he had jaundice and a Billie Blanket was ordered for him to use at home. Our little glow worm took it in stride and after a brief scare of his levels not dropping as expected, he made progress and no longer needed the blanket. Now we could finally move on and enjoy our perfect, healthy baby boy….right?
Over the next few months, he seemed to have trouble adjusting to nursing. He would choke and gag frequently. Reflux symptoms worsened with projectile vomiting, arching, screaming and frequent unexplained low fevers. Again, we were told this was all normal. Reflux was normal and since he was gaining weight no medication was necessary. Low fevers were okay, his immune system was just developing.
At his two month appointment, our pediatrician noticed retractions in his breathing but again, since he wasn’t struggling no one was concerned. It was chalked up to immaturity in his airway tissue and he would outgrow it.
A few weeks later, our little man turned blue. Yes, BLUE. He didn’t stop breathing, but around his lips turned a hue of bluish purple. Scary right? Off to the doctor, we went! All the while wondering and anticipating what this could be. A chest x-ray was ordered and a pulmonology referral was given for the next day. We were terrified. His choking episodes continued during feedings and in his sleep, he was turning blue, having retractions and puking and no one seemed to know why. We feared the worst.
The next day our pulmonologist was on point! His chest x-ray was clear! She heard nothing wrong with his lungs and low and behold….she suspected that all of his issues were caused by REFLUX! Who knew?! She was able to pull the pediatric Gastroenterologist (GI) into our appointment and together they developed a plan to start reflux medication, order a sleep study and send us immediately to see a feeding specialist. This is also where my husband’s never ending battle with the insurance companies began.
After a whirlwind appointment, we drove down the road to meet with the feeding team who recommended feeding therapy and a swallow study. We were incredibly blessed to get in with so many specialists so quickly. This is NEVER the way these appointments work. We were even more blessed to be able to have his swallow study and sleep study completed within the next two weeks. After waiting for results and feeling awful for putting our precious baby through test after test, everything came back normal! Although this was a huge relief, we were still terrified. What was going on with our baby?!
A month later, with all of his symptoms continuing despite meds and therapies his pediatrician referred us to cardiology. Let the Google spiral commence. We again were blessed with a very quick appointment and a fantastic pediatric cardiologist who performed an echocardiogram (ultrasound of the heart) at our appointment and again…everything was NORMAL! So why was our precious new baby having such bizarre symptoms?
With everyone stumped, we went back to GI. A 24 hour GI probe study was ordered and we waited. Our little man would have a probe placed down his nose and into his stomach (similar to a feeding tube). This probe was attached to a monitor and we were to input everything into that monitor for the next 24 hours: feedings, positioning, vomiting, and blue spells. Low and behold, his blue spells happened with every acid spike! His GI ordered an increase in meds but seemed disconnected from our little Os-ling’s care.
At his 4 month check-up, we saw that his weight gain was slowing but he had been through so much! We were advised to start solid food, starting with oatmeal cereal. At our follow-up GI appointment, his GI actually FORGOT about all of the testings he had done already. Talk about being frustrated! It was at this point that we made an appointment with a different GI for a second opinion. While we waited for this next appointment we continued feeding him a variety of foods and documenting his bizarre symptoms.
On our family vacation a week later things got even more bizarre. In the middle of the night, our boy woke up vomiting. The only way to describe this episode was like a scene from The Exorcist. Vomit EVERYWHERE. Vomit every 10 minutes. I’m talking the bed, the floor, our clothes, blankets…EVERYTHING was covered. We panicked and called the pediatrician who told us it was probably a stomach bug and to offer small amounts of breast milk at a time and monitor for dehydration. He vomited until it was all stomach bile but eventually kept some fluids down and an ER trip was avoided. Then he slept. He slept most of the next day and only woke up when he had bouts of horrific diarrhea. When we returned home a few days later this scene replayed again and through some miracle, we realized that both nights, he had eaten oatmeal. But oatmeal couldn’t cause that? Could it?
We were advised to stop oatmeal and start rice instead. Cue the awful mucus diarrhea. I know. This isn’t a topic anyone wants to hear about but seriously, it’s crucial. I began to realize after stopping rice that although his mucus situation improved it never really went away. Soon we realized that banana and sweet potato caused GI upset as well. Even worse was the realization that even with no solids in his diet, he would have bouts of awful GI upset from breast milk. Had he ever had diapers without mucus? Surely he couldn’t be allergic to all of these foods! And there was no way he was allergic to breast milk!
The following week we met with his new GI. It was at that moment that things changed. He was diagnosed as failure to thrive and I remember feeling like the ground dropped from beneath my feet. We had done everything we were advised to. We jumped through hoops, ran from doctor to doctor, we did everything. How could he be that small? Was he really that sickly? She advised cutting soy, dairy, and egg from my diet and seeing if that helped him. She agreed the constant mucus was a concern as well as his continued reflux despite meds. She felt strongly though that reaction to all of those foods was highly unlikely because she had never seen anything like it in her career.
As I researched his symptoms I stumbled upon the FPIES Foundation website. Suddenly all of the pieces fell together. His symptoms could all be explained by Food Protein Induced Enterocolitis Syndrome, a rare form of food allergy that affects the GI system with a delayed response hours after ingestion. He could very likely be reacting to all of those foods. He could also very likely be reacting through my breastmilk and even to my breastmilk protein which was causing the severe reflux, leading to the feeding difficulties, blue spells, retractions and failure to thrive.
A week later we saw the allergist and she confirmed his diagnosis, admitting that she had never actually worked with a child with this diagnosis. She completed allergy testing which all came back negative, confirming the FPIES diagnosis. You see, the challenging part of FPIES is that there is no testing. Typical allergy testing doesn’t show what will trigger an FPIES reaction. The only way to know if your child will react is to trial foods. She advised that I start an elimination diet to figure out what I was eating that was triggering his symptoms or that we make the switch to a specialized formula. It was at this moment that I made the difficult decision to stop breastfeeding. Breastfeeding had always been so important to me. I nursed my daughter until she was almost two and I donated over 1,000 ounces of milk to babies in need. Now, I was making the conscious decision to eliminate this liquid gold from my son’s life. I just couldn’t bear to watch him suffer any longer. I couldn’t bear the thought of playing Russian roulette with his GI system with everything I ate as well as his food trials.
We were very lucky that he tolerated the formula well and that his insurance covered the costs after significant battles. I found an incredible support group online and researched until I drove myself crazy. Luckily we were able to find specialty care of an FPIES guru in Boston, not too far from where we live. His diagnosis was confirmed and we were given a plan and instructions moving forward. We have Zofran for home use to help stop vomiting from a reaction. We have a letter to give to ER doctors if we should need IV fluids to prevent shock. And best of all, we have our GI doctors cell number. He is the biggest blessing on this journey.
We are now on 8 months of managing his FPIES care. He has proven to be one of the rare kids who has more than 3 triggers. We have to trial EVERYTHING. EVERY additive. EVERY spice. EVERY possible ingredient or item he could ingest. He currently has 14 safe foods (including formula, salt, sugar, and baking soda) and twelve triggers. Everything he eats is made by me (with the exception of 3 limited ingredient snacks I can purchase). Every brand is researched for cross contamination. Each week is a new trial: documenting, examining, waiting, watching, prepping. He struggles with feeding aversions because of his experiences with food. We can’t share family meals as we planned. He is 15 months and still on formula. He is tiny and his weight is always a concern but we continue to push through…one trial at a time.
Yes, it could be worse. He could have a fatal condition. He could have multiple severe anaphylactic allergies that require split-second responses. We know this. We are grateful that it is not “worse”. But it is important to understand that strain and stress this diagnosis does put on our family, our marriage, our finances, our life. Telling a parent it could be worse is not helpful. His condition is life-threatening. Although his allergic responses are GI related and happen a few hours after ingestion they can be so severe that they lead to hypovolemic shock. Every time we feed him a new food or start a trial we hope for the best but are prepared for the worst.
The silver lining? It is likely that he will outgrow his triggers in a few years. Re-trialing will likely be done in a hospital setting with an IV placed just in case, but there is likely an end in sight. We know this is great news; but it in no way diminishes the tedious, severe and serious nature of his current situation and our family’s journey.
There is little we can control with the hand he was given. There are more diagnoses and medical conditions on the table for him. It will still be a long bumpy journey with specialists and testing. So, I seek to take control where I can. I raise awareness. I write. I share his story; our story. If you are a family seeking a diagnosis for bizarre symptoms, keep looking. Keep searching. Find a medical team who supports you and is willing to walk beside you in the quest for answers. Seek a medical team who believes in the importance of quality of life even if the symptoms don’t seem “serious enough”. Surround yourself with like-minded people: friends, family, a support group.
In honor of National FPIES Awareness Day on May 4th, we have decided to share every tedious detail of his journey. Our hope is to not only raise awareness for his rare disease but to also provide encouragement and support for all families seeking answers and support for their children.
I welcome you to follow our little man’s journey on Facebook at Living La Vida Lucca. https://www.facebook.com/Living-La-Vida-Lucca-2369903016563479/?ref=bookmarks
Follow the FPIES Foundation. https://www.facebook.com/FPIESFoundation/
Change your profile picture to show your support for FPIES.
Wear teal on May 4th.
Knowledge is power and together maybe we can make a difference.
