My son, Quinn, was born with the most severe form of Hirschsprung disease. He has come far in his three years of age – in fact, he’s able to go eight hours without any of his three assistive nutrition modalities. This might not seem like a lot for those who are used to getting nutrition by mouth, but it’s a huge milestone for us. Here’s how the intestinal rehabilitation team – and in particular his dietitians – helped us get there.
Feeding Issues Following Birth
My pregnancy offered no clues as to what we would discover immediately following Quinn’s birth.
Our first indication that something was wrong happened when I tried to breastfeed him. He pulled away to vomit a substance that was as green as grass. Rotation of the intestines was ruled out as a cause, so he was rushed to Cincinnati Children’s for further evaluation.
Radiologic tests showed that Quinn has Hirschsprung disease, which occurs when some of the nerve cells, called ganglion, in the intestine do not form properly. When the ganglion cells are missing, food will not move through the intestines in that section. It can look different in each kid – some may only have a short segment of the intestine with missing ganglion cells – some much longer.
Quinn’s doctors then discovered during an 8 ½-hour surgery that he had the most severe form, called intestinal aganglionosis. Ganglion cells were missing in all of his colon and most of his small intestine as well. Quinn is left with 30 cm of his small intestine, jejunum only. An ostomy was also placed for the removal of stool.
Because he’s missing so much of his small intestine, he has short bowel syndrome. Problems that can result from this are malabsorption, malnutrition and dehydration.
Getting Help from Dietitians
At this point, we were overwhelmed and thinking the worst for Quinn. But a dietitian in the NICU helped ease our minds. When she said that she helps babies without stomachs get the nutrition they need, our hope was restored.
Quinn was started on TPN, or total parenteral nutrition (giving nutrients and fluids by IV that bypass the gastrointestinal tract), and lipids (fatty acids). A month after his initial surgery, he had a g-tube (tube inserted on the outside of the abdomen that connects directly to the stomach) and central line placed.
The dietitian walked us through all of his new eating modalities, and explained to us that every kid is different. Another child with the exact same diagnosis can have a completely different course of treatment. She said that we would take it day by day and see what he could tolerate, ensuring that he meets all of his nutritional needs.
He started out slowly – getting 1 ml an hour of breastmilk through his g-tube and 5 mls breastmilk three times a day in a bottle. The dietitian also encouraged me to feed him breastmilk, which I think was the key in his ability to eat by mouth later on.
Goal Setting with Our Dietitians
When Quinn first came home from the hospital, he didn’t have any time off from his continuous feedings. This meant that he was constantly attached his TPN, g-tube and central line. We longed for Quinn to have time away from his feeds – to have some semblance of normalcy.
So we made this a goal with our dietitians. In order for him to get a “window” of time free from continuous nutrition, he had to start gaining weight and maintaining blood sugar levels without it. These markers signal that his intestines are adapting by absorbing nutrients.
Around six months of age he started eating solids. This helped with his weight gain. He loves to eat and is always excited to try something new. We have to stay away from sugar so we started with meats and veggies slowly and carefully to make sure he tolerated the new foods and it did not cause too much stool. Too much stooling can lead to dehydration. Kids like Quinn can become dehydrated extremely fast, which can mean a trip to the hospital.
Quinn’s First “Window”
His very first window happened when he was four months old. He worked his way up to two hours and we felt our first taste of freedom. These windows allowed Quinn to meet all of his milestones at appropriate times. Developmentally speaking, Quinn has always been on track.
He continued to gain weight and we were slowly able to build up to a three-hour window. Then four. Then five. He continued to do so well that after one year of working with his dietitians, we’ve made it up to eight hours without him needing assistive nutrition modalities.
His current routine is:
- TPN starting at 7 pm – 11 am. (16 hours straight)
- G-tube starting at 7 pm – 7 am (12 hours straight)
- Smoflipids over 12 hours daily
A Longer Window and More Normalcy
So he’s essentially now tube-free from 11 a.m. until 7 p.m. What does this window of time mean to Quinn and our family? Everything. He gets to be a regular kid without anything attached to him for eight hours. He runs and plays tag with his brother. Rides a tricycle. And importantly, eats regular meals. We can run errands and go to the zoo and park without stares and Quinn can be independent. It’s awesome!
I’m amazed at the progress he’s been able to make and we’re so grateful for the hours of “normalcy.” Ultimately, I’d love for him to work his way up to 12 hours a day. That way he could essentially be tube-free in the daytime and only utilize assistive nutrition in the nighttime. It’s a new milestone that we’ll work towards. In the meantime, we’re making the best of the tube-free hours we have and continually hope and pray for future medical advancements.
For more information about our Intestinal Rehabilitation Program, please call 513-636-6155 or email ir@cchmc.org.
Your sons story is so similar to my daughters it was almost as if I had written it. Our daughter was also born in 2014 and was diagnosed with Pseudo Obstruction. She has ganglion cells but they are abnormal. I completely understand how long and difficult your road has been. I am so glad to hear how well your son is doing and I hope that you see continued progress. Best of luck to all of you. Thanks for sharing your story.