Lila Lake — Maple Syrup Urine Disease

Lila Lake | UPMC Children's Hospital of Pittsburgh

The Challenge: A Rare Metabolic Disease

At five days old, Lila Lake was losing weight. She had no appetite. And although she slept a lot, she was very agitated when she was awake.

By the time she was 10 days old, doctors diagnosed her with maple syrup urine disease (MSUD).

MSUD — a metabolic disorder — can lead to brain swelling, brain or nerve damage, or even death.

After spending a month in the hospital, Lila was doing well — responding to treatment and a strict new diet. But her mother, Chelsea, was still worried.

“As good as we could care for her, at some point, she would get sick from the maple syrup urine disease. And it could lead to brain damage,” Chelsea said. “It was hard to live with that fear every day. Kids are going to get sick because that’s how life works.”

Chelsea joined a support group on Facebook for parents of kids with MSUD. In the group, she learned that a liver transplant might be an option for Lila, now about a year old.

A member of the support group discussed their child's transplant experience at UPMC, and Chelsea decided to make an appointment.

The Path to Living-Donor Liver Transplant at UPMC

Lila and Chelsea met with the transplant experts at UPMC Children’s Hospital of Pittsburgh. After a thorough assessment, they added Lila to the liver transplant waiting list.

Chelsea learned about living-donor liver transplant at that first appointment, but she wasn’t sure if it was right for them. So, they chose to wait for a deceased donor transplant.

Months passed without a deceased donor, and Lila’s condition grew worse.

She returned to Pittsburgh two separate times for a potential transplant, but neither of them worked out.

“As Lila got older, the need for a transplant became more urgent. We knew that living-donor liver transplant offered us a chance to receive a transplant sooner,” Chelsea said.

To help her find a living donor, Chelsea turned to the UPMC Living Donor Champion Program. Her first step was making a call to the Living Donor Champion Ambassador.

The Ambassador taught her the ins and outs of finding a living donor. Soon after their first phone call, Chelsea posted Lila’s story to Facebook

After another transplant mom in the Pittsburgh area shared the post, dozens of people replied interested in being Lila’s living donor.

The Solution: A Man Honors a Family Member by Saving a Child

Lila’s eventual donor, Wayne Bell, began his living donation journey a year earlier when someone close to him needed a liver transplant. Without a second thought, he traveled from Arizona to Pittsburgh to learn if he could be his loved one's living donor.

Unfortunately, Wayne wasn't a match. And sadly, his loved one passed away later that year.

Now, more than ever, Wayne felt sure about being a living donor.

“If I couldn’t help her, I wanted to help someone else in her honor,” said Wayne.

Wayne's doctor at UPMC asked if he would be willing to donate a piece of his liver to a little girl. The doctor explained that she was having trouble finding a match.

Wayne said yes.

The Result: A Normal Life for Lila

Without ever meeting, Lila and Wayne had a successful liver transplant on April 4, 2019. A few weeks after surgery — before Wayne went home to Arizona — they met for the first time outside of UPMC Children’s Hospital.

As Chelsea wheeled Lila out to meet him, Wayne couldn’t help but wonder if the toddler really understood who she was meeting.

“When Lila saw me, the first thing she did was pluck a flower from the ground and hand it to me. That was incredible. Now, no matter how bad a day I have, I think of that little girl, and life isn’t so bad,” Wayne said.

For her part, Lila’s recovery went well.

She and her family had to stay close to Pittsburgh for eight weeks so that the transplant team could monitor her recovery. But after that, she went back home to South Carolina in better health than ever.

“I could just tell that she was thriving,” Chelsea said. “She seems so much more aware.”

Almost a year later, both Lila and Wayne have fully healed and are doing well.

“I feel like a million bucks,” Wayne said. “I’d do it again in a heartbeat.”

Lila still comes back to UPMC Children’s for regular checkups.

“She loves going back to Pittsburgh. When she plays at home, she sometimes likes to pretend she’s having a checkup at the hospital,” Chelsea said. “We had the best experience in Pittsburgh. Every single person who was part of her care was amazing.”

Lila and Wayne’s treatment and results may not be representative of all similar cases.

Learn More About Living-Donor Liver Transplant