Patient Stories | Levi and Hirschsprung disease

Teamwork Helps Levi Enjoy a 'Normal' Life Despite Hirschsprung Diagnosis

Most babies have a bowel movement within their first 24 hours of life. At 36 hours old, Levi King still hadn’t had one.

“Right after he was born, he was eating really well, but around that 36-hour mark, he vomited up lime-green poop,” said Lindsey King, Levi’s mom.

Immediately, Levi was transported to a nearby children’s hospital in central Ohio, where he stayed for five days. The hospital performed a rectal biopsy, but Levi started going to the bathroom on his own, so they sent him home. Then, 48 hours later, Levi began vomiting stool again.

“We instantly took him back to the emergency department,” Lindsey said. “That’s when we learned the biopsy results showed that Levi had Hirschsprung disease.”

Family Seeks Second Opinion at Cincinnati Children's

Hirschsprung disease is a rare colorectal condition, affecting roughly 1 in 5,000 children who don’t have correctly formed nerve cells, called ganglion cells, in their colon. Normally, these cells help move gas and stool through the body.

Because they’re missing these cells to help their bodies pass waste, children with Hirschsprung disease are prone to bacterial infections called enterocolitis, which can make them sick with symptoms like diarrhea, fever, vomiting and nausea. These infections can lead to frequent hospital stays, including time in the intensive care unit (ICU).

Levi was diagnosed with total colonic Hirschsprung disease, which means he was born without any correctly formed ganglion cells in his colon. It’s especially rare, causing more severe symptoms than Hirschsprung’s other forms. Because Levi’s entire colon was affected, he needed several surgeries when he was a baby to give him a stoma (an opening in his abdomen that allowed waste to pass through) and a colostomy bag to collect stool.

“We weren’t making great progress with Levi,” Lindsey said. “It was a lot of two steps forward and one step back. We knew it was going to be hard, but he was constantly getting sick. It felt like every six weeks we were going to the hospital for five- to six-day stays because of enterocolitis and dehydration.”

For this reason, Lindsey requested a second opinion, and a consultation was scheduled at Cincinnati Children’s right around Levi’s first birthday. But before Levi could come in for his first appointment with Aaron Garrison, MD, a surgeon in the Colorectal Center, the two ended up meeting in the hospital’s emergency department, where Lindsey had taken Levi for his most recent bout of enterocolitis.

“We were told that Dr. Garrison was in surgery at the moment, so I didn’t expect to see him,” Lindsey recalled. “I was just explaining to them that we were in the process of getting established with Dr. Garrison. But as soon as he got out of surgery, he came to the emergency room to meet us. Just to say hi.”

Levi Benefits from Team Effort

Once Levi was his patient, Dr. Garrison quickly enlisted the help of Catalina Arce-Clachar, MD, a gastroenterologist in the Division of Gastroenterology, Hepatology and Nutrition, as well as a team of nurses, dietitians and physical therapists. This collaborative effort would help get Levi where he needed to be from a growth and nutrition standpoint.

First, Dr. Arce-Clachar began monitoring Levi’s nutrition, stool output, electrolytes and sodium levels, and she prescribed the supplements he needed.

“I got involved with Levi to help co-manage his Hirschsprung disease,” Dr. Arce-Clachar said. “The team approach we have for kids like Levi is so important. Good communication among our team and the Colorectal team really helps facilitate better outcomes for our patients. My nurse, Brittany Taylor, RN, and our dietitian, Kathryn Hitchcock, RD, played a key role in this communication with Levi's parents. And we all just really love Levi.”

One of Levi’s nurses within the Colorectal Center, Megan Rapp, RN, will never forget the first time she met him—during an appointment she’ll forever refer to as the “pear irrigation.” Lindsey had called that morning because Levi was suffering from a particularly bad case of enterocolitis.

“She brought him in, and we taught her how to do rectal irrigations at home so they wouldn’t have to come to the hospital as much,” Rapp said, referring to the method commonly used for flushing out the bowel. “We also ended up irrigating quite a few large pieces of undigested pears out of his stoma at that appointment. Surprisingly, it didn’t seem to bother him much.”

Bonding over Superheroes, Making Levi Smile

Just a few months later, Levi was doing much better. He was starting to eat and, most importantly, starting to gain weight.

The following year, Levi was ready for a pull-through procedure—a surgery to take out the diseased portion of the bowel and pull down and connect the rest of the bowel to the anus to allow waste to pass. Dr. Garrison performed the surgery in September 2021.

Levi was 2 years old at the time of the surgery, a challenging age for the pull-through procedure because of the common constipation issues toddlers typically encounter when they start potty training. Plus, toddlers who have the surgery often struggle with diaper rash because they’ve had a stoma for so long. The surgery also commonly requires follow-up treatments, like Botox injections and antibiotic prescriptions. These treatments help the anal sphincter relax and make it easier for kids to go to the bathroom on their own.

“Levi was telling everyone that Dr. Garrison helped him so he could poop on the potty,” Lindsey said. “But there was a period when he was mad at Dr. Garrison because of the rash he got after his surgery. He was giving him a lot of side-eye for a while.”

To help get back in his patient’s good graces, Dr. Garrison went out of his way to drive to the Burnet Campus to pick up a surgical hat featuring Spider-Man—Levi’s favorite superhero—before driving to the Liberty Campus for their appointment.

“Levi couldn’t help but smile,” Lindsey said, laughing.

“Sometimes negotiating with a 2-year-old is challenging, so you have to do the things you have to do,” Dr. Garrison joked.

From Complex Patient to Regular Kid

Today, Levi is living the life of a typical 5-year-old boy. He’s gaining weight, is fully potty trained and has no restrictions aside from avoiding sugar, which can make him sick.

“He’s constantly moving, constantly talking,” Lindsey said. “He truly believes he’s part-ninja, and he wants to be a handyman when he grows up so he can help people and fix things. He also always finds the good in everything. Even if it’s something sad or upsetting, he can find the positive in it. He’s a special kid.”

Much of Levi’s success is due to the support of his family, Rapp said. “Levi’s mom, in particular, is always on top of it and such a great advocate for him. She’s always willing to do anything and everything we suggest. She’s very much a member of Levi’s care team. And now, today, going to the bathroom is just another part of Levi’s life. It doesn’t dominate it.”

Successful outcomes for complex patients like Levi often come down to teamwork among the family and the providers, Dr. Garrison said.

“That’s the thing about Levi,” he said. “Total colonic Hirschsprung is a pretty significant condition. But if you have the right teamwork and the right circumstances, these kids can thrive. And now, with Levi for example, you can’t even tell the difference between him and his two older brothers.”

According to Lindsey, Levi is looking forward to joining his brothers on hunting trips with their dad, Josh. He’s also excited to start kindergarten this fall.

“Levi’s case is a good example for families in that tough period in the first year or two of life, where sometimes things aren’t so smooth,” Dr. Garrison said. “But if you keep hope alive and keep working on making progress, these kids can have normal lives. Patients like Levi make it fun when things go well, and they keep you going when things aren’t always as smooth.”

(Published April 2023)