First SC Domino Transplant Leads to Donor and Recipient Reunion

“She was so still — we thought she was asleep.”

There was something wrong with Heraclio Perez’s daughter when she was born, but no one knew what. She spent the first year of her life listless and still, until a doctor in upstate South Carolina tested her for maple syrup urine disease (MSUD).

“We found the bright star,” said Perez. “And that’s when she started smiling again.”

With MSUD, Grace Perez-Davila lacked an important enzyme needed to break down certain amino acids, and a buildup of these protein building blocks in the body can be harmful. If left untreated, MSUD can lead to seizures, coma, severe brain damage and developmental delays in children.

MSUD is also known as branched-chain alpha-ketoacid dehydrogenase deficiency but is referred to as MSUD because it leaves a distinctive maple syrup smell in the urine of undiagnosed infants. Perez was able to manage Perez-Davila’s disease by limiting high-protein foods, which mean her diet consisted of a drinking a specialized formula over regular food.

But after a proper diagnosis and a strictly controlled diet, Perez-Davila began to smile for the first time in her life.

Perez-Davila and her father are close. Growing up without the ability to run like the other kids and having to work hard to stay healthy meant she was by his side all the time. He is her biggest fan and confidant. She loves spending every day after school at his Japanese restaurant in Central, SC, and describes him as her favorite person. “She is my Achilles heel,” says Perez, smiling over at her. “She says jump; I say how high. She’s my little girl, and she changed my life.”

Perez and his daughter’s physician, Neena Champagine, M.D., an MUSC Health expert in metabolic disorders at Greenwood Genetics Center in the upstate, wanted a more permanent solution for Perez-Davila, so they referred her to MUSC Health for a transplant evaluation.

Uniquely, Perez-Davila’s liver was perfectly healthy for someone without MSUD. While it didn’t have the enzyme she needed, people without MSUD have enough of the enzyme throughout their body that they can safely use a liver without them. So, after reviewing her case, Nagraj Kasi, M.D., a pediatric transplant hepatologist and medical director of the pediatric liver transplant program at MUSC Health, thought Perez-Davila would be a good candidate for a Domino liver transplant.

This method of allocating a liver like Perez-Davila’s to another patient has been used in the past to treat other metabolic disorders like MSUD to expand the liver donor pool but is being increasingly considered an option in recent years.

So when a liver became available in April 2017, just four months after Perez-Davila was placed on the transplant list, she became part of the first Domino transplant in the state of South Carolina. She donated her liver at the same time that a liver was donated to her. A new liver would provide her enough of the enzymes she was lacking to allow her to eat normally and live a more active and worry-free life. And by donating her liver, she was able to help someone else.

Satish Nadig, M.D., Ph.D., FACS, is the director of pediatric transplantation and living donor kidney transplantation and is the P.K. Baliga endowed chair for solid organ transplantation at MUSC Health. He performed Perez-Davila’s explant. The surgeries occurred simultaneously, so once Perez-Davila’s liver was removed, Nadig passed it along to his partner to perform the transplant into the organ’s recipient, Cindy Wingard, and then Perez-Davila received her new liver via cadaveric donation.

“We could feel the experience from the staff at MUSC as soon as we walked in the door on the day of the surgery,” said Perez. “They were very patient with us throughout it all.”

What’s unique about this type of operation, according to Nadig is the coordination. In addition to Perez-Davila’s blood type and size compatibility with her donor, she needed to be a match for Wingard. In addition, Perez-Davila and Wingard needed a hospital with the capacity and infrastructure for this kind of surgery. “Not only is MUSC the only transplant center in South Carolina, but we have the ability to do some of these out of the box types of things in order to help more patients,” said Nadig.

“The teamwork and coordination that goes into a surgery like this is incredible,” said Kasi. “And it would not have been possible without our collaboration with the Greenwood Genetics Center, in addition to the camaraderie of the entire team at MUSC.”

Domino transplant procedures are rare and so are living donor transplants. According to the United Network for Organ Sharing (UNOS), surgeons performed almost 40,000 organ transplants in 2019, but only 7,309 of those were living donor transplants. Liver transplants, both deceased and living, made up about 22% of transplants across the country in 2019.

And according to the Domino Liver Transplant Registry, only 79 Domino liver transplants were registered in 2017 across 10 centers.

By opting for a Domino liver transplant, MUSC Health surgeons were able to use a healthy liver from Perez-Davila rather than destroy it; they were able to widen the possibilities for organ donation to help Wingard.

Today, Perez-Davila is 15 years old and loves to run around with her nieces and nephews. She likes to paint her nails, and her favorite meal is teriyaki chicken from her father’s restaurant. She returns to MUSC regularly for checkups and follow-up tests.

 

Wingard and Perez-Davila met each other in the days following their surgery, along with Perez and Wingard’s husband Tally. But coinciding follow-up appointments provided the perfect opportunity for everyone to say hello and see each other again. Wingard and her husband drove down that morning from Columbia and Perez-Davila and Perez from Liberty for their appointments and a chance at a reunion, both sides very grateful for their transplants and for each other.

Now in her 70s, Wingard enjoys staying active. Tally jokes he can’t keep up with her these days. “She’s always going here and there,” he chuckles while swapping stories with Perez.

Wingard asks Perez-Davila about school. She is still learning virtually from home due to the COVID-19 pandemic but enjoys the extra time with her family.

“She’s a warrior,” Perez says of his daughter. “And we are so thankful that MUSC was able to put all of this together.” He grins over at Perez-Davila, and she smiles right back.