It’s a special year for eight-year-old Brielle, of Chicago. She’ll begin third grade at a new school and, later this month, walk down the aisle wearing a sequined rose gold dress in her mother’s wedding.
Most remarkably, though, in August, Brielle received an organ transplant she’d been waiting for her entire life.
Brielle was born with necrotizing enterocolitis, a serious intestinal illness in babies. Her mom, Jackie, remembers when her firstborn couldn’t eat, and doctors told her much of the tiny girl’s intestine was failing.
Brielle endured two surgeries before she was three months old at Ann & Robert H. Lurie Children’s Hospital of Chicago. Surgeons removed part of her intestine, leaving her with short bowel syndrome, a condition that can make it difficult for a child to eat and digest food properly. Brielle depended on total parenteral nutrition, or TPN, in which she received fluid and nutrition through a vein to nourish her body.
“It was such a scary time for her father and me. To see all those tubes on her and her stomach open. It was very hard for us,” Jackie said.
After the second surgery, Jackie learned that Brielle’s name would be added to a waitlist to receive a bowel transplant, which would offer the best shot at curing her condition.
In the meantime, Brielle, who her mom calls a “social butterfly,” could attend school while receiving TPN at home with a nursing assistant and careful monitoring from Jackie, along with regular visits to Lurie Children’s.
Brielle grew up accustomed to wearing a backpack that carried her nutrition bag, and managing a central line and gastrostomy tube, or g-tube, that helped her grow and stay as healthy as possible. Over the years, she took ballet classes and participated in gym at school with few problems. Still, while waiting for a transplant, frequent hospital stays were necessary because of central line problems and other setbacks common among children with Brielle’s condition.
“There were difficult days, for sure,” Jackie said.
Then, in August, a routine appointment visit to Lurie Children’s turned extraordinary.
Valeria Cohran, MD, her longtime doctor and Medical Director of Intestinal Rehabilitation and Transplantation, and Riccardo Superina, MD, Head of Transplant Surgery and Co-Director of the Siragusa Transplantation Center at Lurie Children’s, were meeting with her when Dr. Superina stepped out to take a call from the organ procurement coordinator. When he returned he told Dr. Cohran the good news. She looked at Brielle and asked, “How would you like to get a new organ tomorrow?!”
“Brielle started crying — she was so excited,” Jackie recalled. “I was just in shock.” Finally, after 8 years, a healthy small bowel was available for Brielle’s transplant.
Bowel transplants are increasingly rare among children because of advancements in treatments available for some conditions. In 2018, there were only 37 intestinal transplants performed on those between ages zero and 17 years, according to the United Network for Organ Sharing (UNOS), a nonprofit that manages the country’s organ transplant system with the federal government.
Still, intestinal transplants remain one of the best options for certain intestinal failure patients who are unable to stop using TPN, like Brielle. Waitlist time largely depends on the recipient’s age, size and geography, among other variables.
Jackie and Brielle went home for a quick pizza party with family and friends to celebrate their news before returning to Lurie Children’s for transplant surgery the next day.
Dr. Riccardo Superina performed the all-day procedure. Nurses called Jackie, Brielle’s father Bruce and other relatives to keep them aware of updates throughout the long process.
“It was a long day. It was nerve-wracking,” Jackie said.
Still, Dr. Superina’s demeanor and expertise helped keep her calm and reminded her she was in the right place for her daughter’s treatment, Jackie said.
“I looked him up before she got the transplant; people travel from all over the world to see him,” she said. “I’m so thankful for him and for Dr. Cohran, who is amazing. She’s on top of everything and is genuinely passionate. I can feel it when I’m talking to her. You’re in the best hands at Lurie Children’s.”
Brielle remains in Lurie Children’s over the next few weeks as an inpatient while she heals, spending a lot of time painting and perfecting her slime-making technique.
Like all transplant recipients, she is on many medications, including some that suppress her immune system, while she waits to go home. Risk of rejection of the transplanted organ is always possible, but Brielle will be monitored by Lurie Children’s physicians for many years going forward to ensure the best outcome.
She is expected to be weaned off her TPN support and eventually no longer need a central line or g-tube. Her progress since surgery has been quick, her providers say, and was evident as she danced and munched on noodles recently in her hospital room.
Tears sprang into Jackie’s eyes as she reflected on what’s ahead for her family.
“We’ve been waiting eight years for this,” she said. “We’re looking for brighter days and greater days.”