Clayton Niemesch — Carbamoyl Phosphate Synthetas

The Challenge

Clayton Niemesch was born with a life-threatening condition known as carbamoyl phosphate synthetase I deficiency (CPS-1 deficiency), a rare urea cycle disorder that weakens the body’s ability to metabolize ammonia, a breakdown product of protein.

Just two days after being born, his parents, Stacie and Matt Niemesch, were on their way back to the hospital with Clayton because he was having trouble eating.

“His ammonia level was so high that the doctors told us they were not sure if he was going to make it,” said Stacie.

After doctors began to stabilize Clayton’s ammonia level, Stacie and Matt, who live in Ohio, wanted to know what their options were for Clayton’s survival. They were presented with two potential options. Clayton could take medication and follow a limited protein intake diet to manage the condition, but they would still need to monitor and watch for increased ammonia levels for the rest of his life. Or, he could receive a liver transplant. If not transplanted, Clayton would never have enough protein intake to grow properly.

The Path to UPMC

Matt and Stacie decided that a liver transplant would be the best option for Clayton. They were referred to UPMC Children’s Hospital of Pittsburgh by their physician.

“As terrifying as it is to have an infant receive a liver transplant, it seemed like a better alternative than to be worried for the rest of our lives, including his,” said Stacie.

Clayton was placed on the organ transplant waiting list in October 2020. Stacie and Matt then began the search for a living donor who could save Clayton’s life.

When both Stacie and Matt went to be evaluated to become Clayton’s donor, they were advised not donate because they both carried the gene that has mutated in Clayton.

After creating a Facebook page and asking close family to become potential donors for Clayton, Stacie and Matt were running out of options. However, when Stacie’s sister-in-law, Patti Marton, caught word that her nephew was in desperate need of a liver transplant, she did not hesitate to schedule an evaluation to see if she was a match.

“It was all a learning process for me because I never even thought about living donation before,” said Patti.

“Stacie helped educate me about what needed to be done and walked me through the process, and I ended up being a match for Clayton.”

After traveling from Ocala, FL to UPMC Children's Hospital with her husband, Tim, Patti was ready to embark on her transplant journey.

“After we talked about it, Tim was very supportive and wanted the best for Clayton,” said Patti.

With certain COVID-19 protocols and restrictions in place regarding newborns, Patti only met Clayton for the first time just one day before surgery.

The Result: Family Is Forever

On Jan. 26, 2021, both Patti and Clayton underwent successful liver transplants and are both doing well with their recoveries.

“Clayton is on some oxygen at home but is doing good,” said Stacie.

Clayton will need to take anti-rejection drugs for the remainder of his life, however, he will be able to begin a normal diet when fully recovered and the worry about his ammonia breakdown will no longer be an issue.

Patti’s recovery has been great as she continues to get much needed rest.

“I am doing well, and each day is getting better and better,” said Patti.

Not everyone is aware of the living-donor transplant option and educating the people around you about this alternative procedure can potentially save the life of someone close to you. Early living donor transplant can also help prevent medical complications that occur with CPS-1 and other metabolic conditions.

“Some people don’t even know about the option of a living-donor liver transplant, so I encourage everyone to at least educate themselves on the topic,” said Patti.

“Because who knows, you might end up needing that information one day.”

Learn more about organ donation.