Hirschsprung's Disease: Dariel's Story
Patient Story Highlights
- Dariel was born prematurely and spent his first month of life in the neonatal intensive care unit.
- He was diagnosed with Hirschsprung's disease, a birth defect affecting the colon's ability to move stool through the bowels.
- The affected part of Dariel's colon has been removed, and his bowel function has improved dramatically thanks to Johns Hopkins All Children's Hospital.
A familiar song fills the air. As the electronic beat permeates the house, 3-year-old Dariel’s leg starts to twitch. He drops what he’s doing and instinctively starts to dance.
“Anything to do with music, he loves it,” gushes his mom, Yaritza.
Right hand, left hand. Palms up, palms down. Dariel cheerfully shows off his Macarena moves to his baby sister, Yonielyz, who seems unfazed.
As the toddler eagerly repeats the dance steps, it’s hard to imagine he has overcome challenges harder than getting a 1-year-old to pay attention.
Dariel’s struggles began shortly after birth in his native town of Luquillo, Puerto Rico. Born prematurely at 35 weeks, Dariel spent his first month of life in the neonatal intensive care unit (NICU).
“He was having a lot of trouble at first,” his mom recounts. “He was throwing up and his meconium didn’t discharge.”
Meconium is a newborn’s first stool. When Dariel didn’t have a bowel movement within 48 hours of birth, his medical team ran tests. He was diagnosed with a birth defect called Hirschsprung’s disease.
Hirschsprung's disease occurs when a baby is born without special nerve cells in the muscle layers of the intestine. Without these nerve cells, called ganglion cells, the muscles in the bowels are unable to move stool through the colon.
Hirschsprung’s disease affects one in every 5,000 live births and is generally more common in boys than girls. Treatment involves surgery to bypass or remove the affected part of the colon.
Dariel was just 1 month old when he had his first operation.
During the procedure, called a diverting ostomy, the intestine is biopsied to identify where there are normal ganglion cells and the surgeon diverts the intestine by creating an opening in the child's abdomen called a stoma.
A special bag is placed over the stoma to collect stool that usually passes through the colon and out of the body.
“Everything was fine after surgery,” Yaritza says. “He was a happy boy.”
Shortly after Dariel turned 1 year old, his doctor recommended a second surgery to remove the abnormal intestine and close the stoma. But Mother Nature had other plans.
On the day of Dariel’s operation, Hurricane Maria hit Puerto Rico with a vengeance, destroying the island, his coastal town and forever changing the lives of Yaritza and her son.
“Some people lost everything,” recounts Yaritza. “But I had my baby, my family, my life.”
Unfortunately, the storm’s extensive damage meant limited medical resources for Dariel.
“The doctor said, ‘If you can go to the U.S., it’s going to be better for him,’” Yaritza says. “So I called my sister in Florida and she said, ‘Let’s do it.’”
The family packed up everything and moved to Florida’s east coast.
Dariel’s condition remained stable until he was 2. Then, at a family gathering in Tampa, he couldn’t hold down food. He was bloated and vomiting uncontrollably.
“I thought he was going to die,” Yaritza sobs. “He looked at me with sad eyes and said, ‘Mommy, I feel pain.’”
Yaritza knew her son was in trouble. She needed to act fast.
Yaritza’s sister, a nurse, knew just where Dariel would get the best care. They rushed him to the Emergency Center at Johns Hopkins All Children’s Hospital.
“Our life changed the day we took him to All Children’s,” Yaritza says warmly.
“When I met him, his abdomen was distended and he looked very uncomfortable,” says pediatric surgeon Nicole Chandler, M.D. “X-rays showed he had a blockage. When that happens, we worry about the health of the intestine. He looked like he was heading down that road of developing a perforation.”
Dariel has long-segment Hirschsprung’s disease, a rarer and more serious version of the disease affecting a much larger portion of the intestine.
“The length of his intestine with absence of ganglion nerves goes pretty far up into the small intestine,” Chandler explains.
Dariel was taken to surgery immediately. After all they had been through, Yaritza felt confident that her son was in good hands.
“Dr. Chandler is a wonderful doctor,” Yaritza says. “She came and talked with me before surgery and explained things to me straight.”
“We see an average of one case per month here at Johns Hopkins All Children’s Hospital, so Hirschsprung’s disease is something we treat pretty frequently,” Chandler explains.
During hospital visits, Dariel remained upbeat and always greeted his care team with a hug, a smile and, of course, a few Macarena moves.
“The nurses called him Bruno Mars,” Yaritza giggles. “They would come dance with him.”
At age 3, Dariel has had five surgeries in his short life. Four of them performed at Johns Hopkins All Children’s Hospital. The affected part of Dariel’s colon is gone and his bowel function has improved dramatically.
Today, the little performer is doing well.
“Three years I’ve been waiting for this day,” Yaritza laughs. “When he had the (ostomy) bag, we were afraid to go out. Now that it’s gone, we go to the park, the beach and parties. I can tell he feels more comfortable.”
Chandler credits diligence by Dariel’s family with much of his success.
“Dariel’s mom puts a lot of energy and attention into making her son successful,” Chandler says. “Surgery can only take you so far. Other things like paying attention to diet, exercise and hygiene are important to take you further and be successful.”
After a few sidesteps, Yaritza and Dariel are happy and finally settling into the rhythm of their new lives.
Nicole Chandler, MD
The Division of Pediatric Surgery at Johns Hopkins All Children's Hospital, based in St. Petersburg, Florida, provides a wide range of services including advanced miniature access and minimally invasive surgery, neonatal surgery, congenital diaphragmatic hernia surgery, chest wall deformity surgery, ambulatory wound services and oncology surgery.