UK surgeons help girl conquer rare condition

From the moment she was born, Audrey Armstrong defied every expectation.

Born in Morehead, Ky., at 41 and one-half weeks, Audrey immediately began having difficulties with bowel movements. Her parents brought her to the Neonatal Intensive Care Unit (NICU) at Kentucky Children's Hospital, where she was diagnosed with Hirschsprung's disease, a congenital condition characterized by missing nerves in the colon. 

The first of seven surgeries

"Hirschsprung's disease is a developmental disorder of the nerves located in the wall of the intestine," said Dr. John Draus, division chief of pediatric surgery at KCH. "In HD, there are no nerve cells in the wall of the affected intestine. Without nerve cells, the intestine does not move things through, and the affected area acts like a blockage. The baby can have a swollen belly, vomiting and inability to pass stools on their own. Rarely, the bowel can become so distended that it perforates. That is what happened to Audrey when she was a baby."

In the weeks that followed, Audrey's eating slowed, and she stopped gaining weight. X-rays revealed an obstruction in her bowel. At only 1 month old, Audrey received the first of what would ultimately be seven surgeries.

Following the first surgery, Audrey became very ill – she had a perforated bowel and needed emergency surgery.

"We were told she probably won't make it through surgery, and if she did, she might not make it through the night," said Audrey's mother, Morgan. Audrey's surgeon advised them to prepare for the worst.

Living one day at a time

Audrey did, in fact, make it through the night, but infections continued to spread through her body. She was on a ventilator and weighed less than her birth weight of seven pounds. Her surgical team couldn't risk transporting Audrey from the Pediatric Intensive Care Unit (PICU) to the operating room. Instead, they brought the operating room to her. Doctors and nurses filled the small room. The anesthesiologist asked if he could pray with Audrey's parents. The PICU was shut down so that Audrey's surgery could begin.

After a few hours, Audrey's parents were allowed to return to her room. 

"Life was measured one hour at a time," Morgan said.

What followed were seemingly endless rounds of infections, antibiotics and swelling. Two more surgeries took place in her tiny PICU room, followed by more infection. But slowly, Audrey responded to the treatments and grew stronger.

"Life began being lived one day at a time," said Morgan.

Surgery No. 6 was meant to be a five-hour procedure to close her abdomen up for good with a colostomy to divert the colon to an opening in the abdomen. What happened instead was a 10-hour surgery during which all of the remaining large intestine was removed and one lung partially collapsed.

"Audrey’s case was particularly complicated," said Draus. "She required seven operations and multiple hospitalizations. She was critically ill and almost died of sepsis. There were numerous KCH surgeons, physicians, nurses and other healthcare workers who participated in her care. It was a great team effort. We are thrilled that Audrey is doing so well at this time."

Turning the corner

But the complications were no match for Audrey's indomitable spirit. An ileostomy replaced the plan for a colostomy; her small intestine, rather than her colon, was rerouted to her abdomen. Respiratory therapy restored lung function. She had one final surgery at the age of 9 months to reverse the ileostomy, followed by a handful of hospital stays for dehydration and infection – common for patients with compromised digestive systems – and Audrey was on the road to recovery.

Today, Audrey is a bright and bubbly middle schooler with a passion for gymnastics and working with children. She has to maintain a strict diet and is curious about the trials she faced as a baby. Audrey loves spending time with her friends and volunteering in her church's youth ministry. Looking toward the future, Audrey wants more than anything to be a mother. And maybe a nurse.

"She will no doubt change this world for the better," said Morgan. "Her start to life was a struggle, though, and the challenges she has faced have shaped her into the lovely young woman we know today."