Hirschsprung Disease: Taitum's Story
Ariel Ellis had a normal pregnancy and delivery in a Springfield, Mo., hospital. But shortly after giving birth on Oct. 18, 2018, her newborn son’s health quickly declined.
“Taitum was jaundiced. Doctors put him under a bilirubin light right after he was born to see if he would improve,” Ariel said. “He wasn’t eating, hadn’t passed his first stool (meconium) and at one point, they had me feeding him with a syringe. I just felt like something was wrong.”
By Taitum’s second day of life, Ariel knew her mother’s intuition was right. He was vomiting a greenish-brown substance called bile and was moved to the hospital’s neonatal intensive care unit.
“The doctors in Springfield came to my room when Taitum was 3 days old and said they thought he needed to be transferred to a higher level of care,” Ariel said. “They asked if we wanted him to go to Kansas City or to St. Louis. We chose Kansas City.”
With Taitum’s condition continuing to go downhill, doctors ordered an immediate transfer to the Level IV Neonatal Intensive Care Unit (NICU) at Children’s Mercy Kansas City. The hospital’s Critical Care Transport Team flew to Springfield, transferring him safely to the NICU.
In the Children’s Mercy Level IV NICU, doctors went to work, trying to determine why Taitum wasn’t doing well. “I remember the attending doctor told me he had no idea what was wrong with Taitum, but they were going to figure it out,” Ariel said. And they did.
Diagnosing Hirschsprung disease
Taitum weighed 8 lbs., 2 oz., at birth, but he had not had a bowel movement in the first 48 hours of life and he had vomited bile, making doctors suspect he might have a rare condition called Hirschsprung disease.
Hirschsprung disease is a birth defect in which the end of a child's bowel, or colon, does not contain all the typical nerve cells, called ganglion cells. It also may affect the length of the bowel. The condition occurs in approximately one in 5,000 newborns and is more common in boys than girls.
The lack of nerve cells along the bowel in a child with Hirschsprung disease prevents food and waste from moving forward through the colon to the anus, causing problems passing stool (pooping), something Taitum was experiencing.
To confirm the diagnosis, Rebecca Rentea, MD, Director of the Children’s Mercy Comprehensive Colorectal Center, removed a small sample of tissue from the last part of Taitum’s colon for examination under a microscope.
“Hirschsprung disease is rarely detected prenatally,” Dr. Rentea said. “It is most commonly a surprise diagnosis after the child is born. That’s why the Comprehensive Colorectal Center at Children’s Mercy can be instrumental in diagnosing and treating these infants and children.”
As one of the few centers in the nation specializing in the complex and long-term care of this condition, Dr. Rentea said the center takes care of about 25 newly diagnosed kids like Taitum each year.
These families have the advantage of working with a highly specialized team of providers all focused on caring for children with Hirschsprung disease and other anorectal and pelvic floor disorders. Led by Dr. Rentea, they understand what is needed to successfully manage such a complex diagnosis.
“We have a dedicated team who specializes in treating this problem. Families want and need a team that is available for their child inpatient, outpatient and electronically at all times,” Dr. Rentea said. “I think it means a lot to families when they know the team their child is seeing cares about the same things they do. We can have a much more dynamic dialogue with these families.”
When Taitum’s biopsy confirmed he had Hirschsprung disease, Dr. Rentea recommended additional testing at 2 weeks old to see how severe the condition was.
“I remember Taitum’s case vividly,” Dr. Rentea said. “While the amount of Hirschsprung looked fairly typical, once we examined his colon closer, we found he was much sicker than his diagnostic imaging or pathology indicated.”
In fact, Taitum had developed enterocolitis, a dangerous inflammation of the digestive tract. To treat the problem, Dr. Rentea created an opening in his abdomen called an ostomy.
This is where the end of the bowel passes waste out through the ostomy and the waste is collected in an artificial external pouching system adhered to the skin. The ostomy gave Taitum’s bowel a chance to heal and recover.
During Taitum’s NICU stay, Ariel used the private lactation room to pump her breast milk, storing it in the Milk Room next to the NICU for Taitum. “Breast milk has a lot of good bacteria in it that’s important to maintaining a healthy gut,” Ariel said. “I really appreciated that the hospital supported breastfeeding.”
After three weeks at Children’s Mercy, Taitum’s condition improved. He was released to return home but traveled back to the Comprehensive Colorectal Center for follow-up visits.
A couple of months later, Taitum had a second procedure called a pull-through surgery, the primary treatment for Hirschsprung disease. During the surgery, Dr. Rentea removed the portion of Taitum’s colon without any nerve cells, then connected the healthy bowel to his anus, allowing stool to pass.
Secret to Success
According to Dr. Rentea, Ariel’s involvement in Taitum’s care from day one has been the secret to his successful outcome. “Hirschsprung disease is a life-long problem. How a child does depends on how much disease they have, how well they heal from their surgery, how they do with toilet training, and the type of ongoing supportive care they receive from family and providers.
“Ariel was extremely engaged in this process right from the start and has been with our multidisciplinary team from the beginning,” Dr. Rentea added. “She understood Hirschsprung is not a one-time diagnosis. Her willingness to communicate, work with us and learn how to care for Taitum has made it possible for him to stay home and stay healthy.”
That included learning how to perform rectal irrigation to help Taitum get rid of waste and keep his colon healthy, if waste is not passing on its own. Irrigations are done by inserting a tube into the child’s anal opening and flushing the colon out slowly. This can get rid of the waste and gas that can make the child sick and provides immediate relief. Doing an irrigation can sometimes prevent hospitalizations or getting sick.
“Rectal irrigation can be a scary process for parents, but it’s essential,” Dr. Rentea said. “I compare it to CPR for the bowel. If you don’t do it when it is needed, the child can get very sick. We strive to have every family feel empowered to understand what can make their child well and what to look for.”
Growing and Going …
Ariel admitted Taitum had a rough first year of life, but since then, he’s been thriving, growing and developing like other toddlers his age.
Taitum attends a medically licensed day care in Springfield, where he’s walking, running and talking. “He’s a typical 2-year-old, getting into everything,” Ariel admits.
He loves to play outside with his big brother, Teagan, and one of his favorite foods is broccoli! “Taitum loves his veggies,” Ariel said.
“Taitum should have a normal, great life, as long as we stay on top of any possible issues,” Dr. Rentea added. Potty training is one of those.
“We’re in the midst of the potty-training process with Taitum,” Ariel said. “Potty training is a challenge under normal circumstances, but it’s especially so with Hirschsprung on top of it.”
Looking back, Ariel is thankful Taitum was transferred to Children’s Mercy just three days after he was born. “Dr. Rentea diagnosed Taitum with Hirschsprung when he was only 5 days old.
“Throughout this process, whenever she would sit down to talk with us about Taitum, she explained things in terms we could understand and let us ask any questions we had. She’s very down to earth,” Ariel said.
Because Taitum lives more than 100 miles from Children’s Mercy, those visits also have included telehealth to facilitate virtual appointments. “Even if we need an image, we can obtain that locally and still have our appointment by telehealth,” Dr. Rentea explained. “We’ve tried to support our families virtually, especially with the pandemic.”
“We’ve been able to build relationships with the nurses and medical staff. They’re always there to answer our questions,” Ariel added. “We’re very thankful Dr. Rentea was able to figure out what was going on with Taitum so quickly. I would highly recommend Children’s Mercy to anyone who is dealing with any type of complex medical need. Dr. Rentea and her team are awesome!”
Hypersensation of the gastrointestinal tract: Amarissa’s story
At 4 ½, Amarissa Hampton has struggled with painful constipation since birth. Finally, she was referred to the Gastrointestinal team at Children’s Mercy, and after a lengthy hospitalization, she’s doing better than ever. Now she’s enjoying her favorite foods, like ice cream and pizza, she has lots of energy, and she’s thriving for the first time in her young life.
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Director, Comprehensive Colorectal Center; Associate Program Director, Pediatric Surgery Fellowship; Associate Professor of Surgery, University of Missouri-Kansas City School of Medicine