Kennedy Stevenson – AdoHcy Deficiency – Living-Donor Liver Transplant Patient Story

Kennedy Patient Story

Every mother knows that her own child is special – one in a billion, and different from others in every way. But Donya McCoy didn't know that her daughter, Kennedy Stevenson, really was one in a billion.

At age three, Kennedy's doctors diagnosed her with a rare metabolic disease. It's so rare that only eight other people in the world have ever had it.

S-adenosylhomocysteine hydrolase (AdoHcy) deficiency is a genetic enzyme disorder that affects the brain, muscles, and liver.

The disease results from changes in the gene that codes for the enzyme AdoHcy hydrolase.

Solving the Mystery

When Kennedy was eight months old, she had a seizure. This prompted Donya to take her daughter to the nearby Clinic for Special Children in Lancaster, Pa.

The Clinic diagnoses and treats children with genetic disorders. This is where she met Kevin Strauss, MD, the Clinic's medical director and a leader in diagnosing and treating rare diseases.

Dr. Strauss and his colleagues found that Kennedy was not growing as she should.

Her head was abnormally small (microcephaly). She also had global developmental delays, muscle problems, liver disease, and factor VII deficiency.

The late S. Harvey Mudd, MD, a world-renowned metabolic expert, was part of the team. And it was Dr. Mudd that pinpointed Kennedy's condition as a disorder involving her AdoHcy hydrolase enzyme.

Her best bet to better health would be a liver transplant.

The Path to Pittsburgh for a Liver Transplant

Donya and Kennedy made their way to Pittsburgh, where they met with transplant team at the Hillman Center for Pediatric Transplant at UPMC Children's Hospital of Pittsburgh.

The care team placed Kennedy on a restricted diet. 

Even in the early stages of her journey, she proved she was brave and willing to do what she needed to do.

"We never imagined something like this would touch our lives," said Donya. "But Kennedy has done so well. She took it all in stride."

Doctors explained that the enzyme deficiency made it impossible for Kennedy to process protein in an efficient way.

This caused a build-up of toxins in her body, which led to degenerative nerve problems.

A liver transplant could correct the problem.

Because of how the organ donor list works, Kennedy wouldn't have a very good chance at securing a deceased-liver donor.

The system that allocates organs uses an impartial formula that doesn't take into account extremely rare diseases like AdoHcy deficiency. According to the data, Kennedy's liver was healthy.

Living-donor liver transplant looked like the best possible route.

A Procedure With Promise

UPMC Children's Hospital of Pittsburgh has done more pediatric living-donor liver transplants than any other center in the U.S.

And UPMC's survival rates are far higher than the national average.

Since 1997, Children's has performed:

  • More than 180 living-donor liver transplants.
  • More living-donor liver transplants for kids with metabolic disease than any other hospital, including adult centers.

Unlike deceased-donor liver transplants, a healthy living person can give part of their liver to the person who needs it. The donor's liver then regrows, and the donated portion grows in the recipient's body

The procedure is complex and demanding, but outcomes are favorable. The challenge lies in finding a suitable donor.

Turning to Social Media to Find a Liver Donor

The bleak assessment didn't stop Donya and Kennedy from staying positive. They're fortunate to have an extensive support system from their large, blended family.

But a complicating factor was that Kennedy's living-liver donor could not be a relative because of the hereditary nature of her disease.

Donya knew that if she were to find an unrelated liver donor, she would have to reach out to her friends.

Facebook is a great way to stay in touch, share photos, and show off your home improvement projects. But it's not every day that somebody turns to the social media outlet for help with a life-or-death situation.

"Okay, it is...the request of a lifetime," Donya posted to her 800 or so friends in July 2014. "Is there anyone out there who has O+ blood? And would be willing to donate 25% of their liver to save Kennedy's life? Trust me, I know it's a lot to ask. But as a mother, I have to exhaust every option possible."

"This was the biggest network of people I knew, and I had to do it. When I clicked that button, I was nervous and overwhelmed," said Donya. "This isn't something you'd ever think would happen."

But soon after her post went up, she got a message from Mike Thompson, a former classmate from Bethlehem Catholic.

When Mike's daughter was in the NICU, Donya had reached out to him — and now he wanted to help Kennedy.

"I'm a firefighter, so I help people every day. But to help somebody like this is just a once-in-a-lifetime opportunity," said Mike. "It's just the right thing to do, and I'm glad Donya let me help."

The Spirit of Giving

After Mike's evaluation to see if he was compatible for Kennedy, everyone headed to Pittsburgh for the transplant surgery. The large group included family members of both the donor and recipient.

Though the trip took 10 hours, Donya said Kennedy was ready.

During the surgery, both families spent time getting to know each other even better than before.

Today, Donya says they're just one big family.

Kennedy calls her liver donor, "uncle Mike," and he calls her, "KJ."

Mike said he wasn't nervous. Everybody else was more nervous than he was.

"I knew I was in good hands the entire time. I've heard so many good things about UPMC," he said. "My only real worry was that the transplant would get rejected. That was my only fear."

His family supported him in his decision, and he knew in his heart that he was doing what he needed to do. He says his only disappointment is that he can't do anything like it again.

"I'm not even allowed to donate a kidney, so I guess I can just donate blood," he said.. "If something like this ever happened in my own family, I'd just hope for somebody to step up. My wish is that anybody who wants to do it will just give it a try. If you're lucky, you'll find somebody you can help."

Today, Kennedy and Mike are doing well.

"Physically, she's great," said Donya. "She's eating protein and developing so well. She seems to be catching up, and the doctors are amazed at her brain development. She emulates her big sister, and they're just so close. She is such a happy, playful, loving child. You'd never know she's gone through anything serious."

Her blended family, selfless friend, and — most of all — strong daughter have shown her how love can overcome hardship.

"Kennedy's been through more in her four years than most of us have been through in our whole lives. She is super brave, but not hardened at all," said Donya. "She's just so sweet and happy. She is my hero."