Advocating for Bridget: Coping with Hirschsprung’s disease

Hirschsprung's disease

On June 20, Bridget Landry celebrated a very special day with a hearty steak dinner. But it wasn’t her birthday. Instead, the 10-year-old and her family were marking the sixth anniversary of the day her ostomy bag was removed. As she enjoyed her meal, her parents, Carl and Laura, marveled at just how far their daughter had come. “For her first birthday, she couldn’t even take a bite of cake,” remembers Carl.

Bridget’s first few months of life were similar to those of most infants: She had a typical birth, nursed and met all her milestones. Yet at six months, her parents began to notice something was amiss. She wouldn’t eat solid food, wasn’t having bowel movements and her belly looked distended. Her pediatrician suggested that she might just be constipated. “But in our heart of hearts, we knew something wasn’t right,” says Laura.

treatment for Hirschsprung's disease

A frustrating diagnosis

By the time she was a year old, Bridget’s doctors in Maine had diagnosed her with Hirschsprung’s disease, a disorder in which some of the intestinal nerve cells don’t develop properly, causing them to interfere with the movement of food and stool in the intestines. Although the majority of children with Hirschsprung’s develop obstructions and other symptoms within the first six months of life, others don’t show signs of the disease for months or years.

Her local surgeons treated her condition with a standard approach called a pull-through procedure that involved the removal of part of her large intestine. Still, her symptoms persisted and the pull-through failed. She remained in the hospital for almost three weeks. When she returned home, she had a central line, received nutrition through a nasogastric (NG) tube and passed stool through an artificial opening in her abdomen called a stoma — but she wasn’t getting any better. After a few months, her physicians referred her to the Motility and Functional Gastrointestinal Disorders Center at Boston Children’s Hospital.

motility disorder

“He treated us like human beings”

After running a series of motility tests, the Center’s director, Dr. Samuel Nurko, determined that Bridget’s stoma was too tight and that, despite the pull-through surgery, her colon still wasn’t functioning properly. It was complex, but the Landrys appreciated Dr. Nurko’s down-to-earth approach. “He’s probably the top GI specialist in the world, but he didn’t use medical jargon with us,” says Carl. “He could have buried us with medical terms but he didn’t. He treated us like human beings.”

Based on the test findings, his colleague, Dr. Tom Jaksic, surgical director of the Center for Advanced Intestinal Rehabilitation, performed a series of operations over the next few years: He revised Bridget’s stoma, removed the central line, and performed a reconnection surgery that ultimately allowed her to stop using the ostomy bag.

The procedures were successful, but her recovery was long — and hasn’t always been easy. At first, Bridget struggled to achieve regular bowel function and developed a colon infection. But her parents are convinced she’s in the right hands. “No matter what the problem, someone at Boston Children’s has been there to help with a solution,” says Carl. “Every speed bump we hit, Drs. Nurko and Jaksic had an answer.”

Hirschsprung's disease treatment

“You have to be an advocate”

 Today, Bridget is an active child who enjoys playing soccer, basketball, softball, and traveling with her parents and older sister, Brooke. She still sees Dr. Nurko regularly — and their relationship has grown with her. Where doctor and patient once discussed Elmo and his pals, they now cheer on the New England Patriots and Rob Gronkowski.

What hasn’t changed, say her parents, is their bond. Dealing with a motility disorder can be embarrassing and uncomfortable for kids, but Dr. Nurko makes it a little easier, says Carl. “She trusts him to do what he needs to do to help her.”

The Landrys hope that sharing their story will help other parents and kids with chronic illness get the care they need, too. “Early on, doctors were telling us that Bridget just needed more fiber, but we knew that was wrong,” says Laura. “You have to be an advocate for your child’s health, because no one else is going to do that for you.”

Learn about the Motility and Functional Gastrointestinal Disorders Center.