Article
Merce’s story: Navigating Hirschsprung's disease in the first weeks of life
When Lacey welcomed her firstborn son, Caiden, she felt grateful for a smooth pregnancy and delivery. At home, everything seemed typical at first – Caiden ate, slept and wet his diapers as expected. He even passed the initial dark, tarry stool, called meconium, that her doctors had prepared her for.
But after about a week at home, things suddenly changed when Caiden started vomiting.
“His vomit was green. And even though I was a first-time mom, I knew that wasn’t normal,” said Lacey.
Alarmed, she dialed 911. The EMTs directed the ambulance to take Caiden to Children’s HealthSM in Dallas, knowing they’d have all the expertise 8-pound Caiden might need.
In the Children’s Health Emergency Department, doctors performed a series of tests to check for life-threatening conditions that can cause a child to vomit bile. They did a spinal tap to rule out meningitis and an upper GI test to make sure his intestine wasn’t twisted. Then, the ER doctors consulted the surgery team who performed a rectal biopsy to check for a condition Lacey had never heard of: Hirschsprung's disease.
Just two days later, the specially trained pathologists at Children’s Health confirmed the absence of ganglion nerve cells in the lower part of the colon – the telltale marker of Hirschsprung’s disease.
These missing nerve cells meant Caiden’s intestines couldn’t squeeze and relax as they should. This was causing his stool to get stuck – leading to constipation, stool impaction in his colon and, as a result, belly swelling and vomiting.
A need for surgery at three-weeks old
Stephen Megison, M.D., Pediatric Surgeon at Children’s Health and Professor of Surgery at UT Southwestern, told Lacey her son would need one of two procedures to help him stool more easily and prevent life-threatening infection: either a temporary colostomy bag or more permanent pull-through surgery. He couldn’t be sure which one would be best for young Caiden until he took him back into the operating room. A colostomy bag would provide a way for stool to exit Caiden’s body through a small opening in his belly. Pull-through surgery would remove the part of the colon without nerve cells and reconnect the healthy part of his colon to his anus.
Surgery was scheduled just one week later, when Caiden was only three weeks old.
“It was so hard to give over my first baby – my little baby – to these doctors and not know what was going to come of it,” Lacey said.
This fear is something Dr. Megison never takes lightly.
I always draw pictures of what I’m going to do surgically for parents. But, more importantly, I tell them I’m going to care for their baby as if that child were my own.
Dr. Stephen Megison, M.D.
In Caiden’s case, Dr. Megison was able to perform a successful pull-through surgery. But even after surgery, most kids with Hirschsprung’s disease need extensive ongoing support because surgery is just the first step in treatment.
“Common problems with how the colon works, even after a successful pull-through surgery, require active treatment and ongoing monitoring," Dr. Megison explains. “Our Bowel Management Program, the only one of its kind in North Texas, is here to support kids for years to come.”
Receiving all the support he needed
Bowel management treatment – to help kids poop more completely, regularly and easily – is generally not one-size-fits all.
“Particularly with Hirschsprung’s disease, bowel management is not always straightforward," said Sharon Kluger, APRN, Nurse Practitioner in Pediatric Surgery at Children’s Health. “It requires a lot of minds working together to figure out what helps a kid.”
Thankfully, the team of specialists at the Colorectal and Pelvic Center and the Pediatric Neurogastroenterology and Motility Disorders Program are trained to help kids in many different ways.
Caiden’s multidisciplinary care team has used several treatments, including:
Irrigations, enemas and laxatives.
Dietary counseling from clinical nutritionists to help increase his fiber intake, make sure he’s getting all the nutrients he needs, and continuing to gain weight.
Botox injections to help his sphincter muscles to relax.
Pelvic floor therapy and anorectal biofeedback to help strengthen and coordinate Caiden’s pelvic muscles.
Medications to get rid of gastrointestinal bacteria and infections, including H. pylori.
Through it all, Caiden has continued to be a chatty, cheerful kid with a positive outlook.
“Biofeedback is actually kind of fun. It's a game where I have to use my muscles to make the flower bloom or make the dolphin jump out of the water,” he says.
Lacey is inspired by her son’s optimism, bravery and strength.
“You’d never know it by looking at him, but Caiden has been through so much. He’s missed school, had accidents, endured intense pain and constipation, irrigations and enemas at home, had multiple biopsies and endoscopies – and been hospitalized for enterocolitis,” said Lacey. “But the team has always been there to help us or to try a new approach.”
Getting his ACE button
In 2022, when Caiden was 8, his care team started discussing an ACE button with him and his family.
An ACE button is a small port or opening in the belly button that connects to a child’s colon. It helps a child flush out their colon more easily and can give them more independence.
“Having Dr. Megison give Caiden an ACE button has helped make his intestines a lot happier,” said Lacey. “And having Sharon be our point person to help us find the best balance of saline and glycerin to use for his daily button flushes has been so helpful.”
Unlike an enema, an ACE button also gives older kids like Caiden the independence and the privacy to do flushes on their own.
“I can play with toys or talk to friends on my phone while I do my flushes now. And even though some people might be surprised, having my button doesn’t stop me from playing baseball or even swimming,” says Caiden.
Long-term, life-changing relationships
Kluger is quick to tell people she’s “passionate about poop.” She’s received a lot of extra training in colorectal care and regularly attends conferences to stay abreast of the latest research and treatments.
When I’m able to help families like Caiden’s with something that seems so simple, you really can change their life. And they're quite thankful for it.
Sharon Kluger, APRN, Nurse Practitioner
Lacey is definitely thankful for Sharon Kluger and Dr. Megison too.
“Dr. Megison has always been the doctor we love having and seeing. He remembers us. He’s so easy to talk to – and he’s so informative,” Lacey said.
Caiden and his family also appreciate the added expertise of Alejandro Llanos Chea, M.D., Pediatric Gastroenterologist at Children’s Health and Associate Professor at UT Southwestern Medical Center and part of the Pediatric Neurogastroenterology and Motility Disorders Program, who joined his care team in 2024.
Dr. Llanos used an advanced test called colonic manometry to do a detailed study of Caiden's nerve functions in his large intestine. This helped him tailor Caiden's treatment and ACE flushes to his exact needs, which has helped him have fewer accidents, have more energy and gain weight.
“We have the full array of manometry tests available here, which many pediatric centers don’t have access to,” said Llanos. “Many GI doctors focus on the ‘hardware’ of the GI tract – inflammation, allergy, infection, obstruction. But, as a motility specialist, I can focus on the ‘software’ of the GI tract – the nerves that need to work so we can stool properly.”
Embracing life and growing their family
Caiden is now a sixth grader who loves dinosaurs, baseball and trains. “My friends call me ‘Train man’ because I love all things trains,” said Caiden.
Caiden’s mom further explained that her son loves riding trains, learning about them and playing with them. And Caiden still has the Thomas Trackmaster set that Child Life gave him in the hospital in his bedroom at home.
When asked what he wants to be when he grows up, Caiden gave a wiser-than-his-years answer: “I don’t know. It kind of depends on how life goes.”
Having lived with a child with Hirschsprung’s for 11 years, Lacey offers wisdom and encouragement for other families in similar shoes:
Advocate for your child. Speak up when things don’t feel right.
Build independence. Teach kids to care for themselves as they grow.
Seek community. Join support groups – locally or online – to connect with others, especially because Hirschsprung’s disease can be invisible from the outside.
Ask questions.
I always have a lot of questions. And they always answer them – and respect my wishes.
Caiden, patient
When Caiden was younger, life was very stressful and overwhelming for Lacey.
“But once we got on the other side of things, I thought, ‘OK, I could have another child,” she said. “And now Caiden has a two-year-old brother – and another baby brother on the way.”
Learn more
The General Pediatric Surgery Program at Children's Health provides expert surgical care tailored to each child's individual needs, from newborns to young adults. Each year, we help hundreds of kids who have complex bowel and pelvic conditions through our multidisciplinary Colorectal and Pelvic Center, our Bowel Management Program, and our Pediatric Neurogastroenterology and Motility Disorders Program. Our goal is always to provide the most comprehensive support we can as soon as possible to help maximize a child’s quality of life for years to come.
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