Sister helps Wisconsin woman cure chronic disease with gift of liver transplant

MADISON, Wis. — Jaime Marshall’s first bite of steak was a revelation. At 47 years old, she had never savored a sirloin with onions and mushrooms due to a rare inherited condition.

“From new foods to new experiences, I don’t take anything for granted,” she said. “I am fortunate compared to other patients with this disease who suffered serious physical disabilities.”

Marshall was born with maple syrup urine disease, or MSUD, a rare, inherited metabolic disorder that prevents the body from breaking down three key amino acids found in protein-containing foods. These amino acids build up in the blood, become toxic and cause severe health problems, according to Dr. Jessica Musto, transplant hepatologist, UW Health.

“Without the proper diagnosis and treatment, the toxins can damage organs very quickly and cause brain damage,” said Musto, who is also an assistant professor of medicine at the University of Wisconsin School of Medicine and Public Health. “Once diagnosed, patients need lifelong monitoring to ensure they are getting the proper nutrition.”

As a newborn, Marshall spent 13 days in the hospital while her parents and doctors struggled to figure out why she was not thriving. It wasn’t until a diaper change that a nurse noticed the disease’s hallmark — urine that smelled sweet, like maple syrup. The nurse started making some phone calls.

“Babies weren’t tested for this when I was born, so I am grateful that nurse was there that day and made the connection,” she said.

The state of Wisconsin began testing babies for MSUD in 1978, the year after Marshall was born. The Wisconsin State Laboratory of Hygiene on the UW-Madison campus screens newborns for rare and potentially serious disorders using blood samples taken from the heel. In 1978, there were 10 disorders on the newborn screening panel. Today, lab technicians can test for 48 disorders.

As a baby, Marshall was given a special formula that contained essential nutrients without the specific amino acids that would cause toxins to build up in her blood. Marshall continued drinking that formula as she grew up and had to stick to a strict low-protein diet without meat, eggs or dairy products. Her childhood was filled with regular hospital visits, blood draws and routine checks of her liver function. Marshall’s older sister, Vikki Ness, said she admired her sister for enduring all the tests and hospital stays with strength and patience.

“Jaime didn’t have it easy, but she took it in stride,” Ness said. “I admired her for staying positive during challenging times.”

Marshall learned to manage her condition into adulthood thanks to the patience and guidance of her mother Christine Ness, who made sure her daughter followed the dietary requirements and took the medications she needed to stay healthy.

As she raised her own two children, Marshall visited her doctor regularly to monitor her liver. While the disease doesn't directly impact the liver, the extra calories patients must consume lead to a higher risk of developing conditions such as diabetes, high blood pressure and fatty liver disease, according to Musto.

“The disease can cause fat deposits to form on the liver and we have to continually monitor it,” she said.

In August 2023, Marshall’s health took an unexpected turn. She began experiencing brain fog and forgetfulness, common symptoms of encephalopathy, a general term for a brain disorder or disease, and she was repeatedly admitted to the hospital. She also showed signs of worsening damage from fatty liver disease and was referred to Musto for additional treatment.

The team at University Hospital first tried to stabilize Marshall with medication and fluids, but her condition did not improve. The team assessed her Model for End-Stage Liver Disease, or MELD, score. This is a standardized scoring system for liver disease severity based on laboratory tests, according to Musto.

“Jaime’s MELD score was a 15 and at that point, we know a liver transplant can be beneficial,” Musto said.

Marshall’s mother encouraged her daughter to begin the evaluation process for a liver transplant. There are two options: Receive a liver from a deceased donor, or receive a portion of a liver from a living donor since the organ can regenerate. The UW Health Transplant Center is the only transplant center in Wisconsin that performs living liver transplant surgeries in adults, according to Musto.

Marshall began talking to her family about getting listed for a transplant and that it might take months or even years to find a donor.

“I was on the phone with Vikki explaining my options and she asked what she could do, and I said, ‘Would you want to be a donor?’ and that got the ball rolling,” Marshall said.

Ness immediately wanted to explore living donation but had plenty of questions.

“I didn’t even know my own blood type,” she said.

Ness began the evaluation to become a living liver donor in November 2023 and quickly learned she was blood type compatible with her sister. In addition to checking blood type, the process involves a detailed medical history, multiple scans, blood and urine tests and physical and mental evaluations that take place over several days, according to Melissa Schafer, senior transplant coordinator, UW Health.

“It is crucial that living donors know what they are getting themselves into,” she said. “We must make sure they are in good physical shape and prepared emotionally. Our team is committed to making sure they are well educated about the process.”

In this case, the team at the UW Health Transplant Center also tested Ness to make sure she wasn't carrying the same genetic condition as her sister.

“It is a major surgery and we needed to make sure we weren’t putting Vikki’s health at risk to help Jaime,” Schafer said.

Due to insurance requirements related to living donation, Ness needed to wait for Marshall to be added to the transplant wait list before she could complete the entire donor evaluation. While the sisters waited, they suffered an unexpected loss: Their mother died in January 2024. She had been the biggest advocate for a living donation between her two daughters.

“I don’t think enough good things can be said about the wonderful kids she raised and how hard she worked to keep them both healthy,” Musto said.

The sisters found out in May that Ness was cleared to donate to Marshall, and they were able to schedule the surgery to allow Ness’ daughter to come home to assist with their recovery. On May 28, Ness and Marshall were wheeled into side-by-side operating rooms at University Hospital.

“I said a silent prayer and asked God to keep us both safe,” Marshall said.

Ness had her own silent hope as she was placed under anesthesia.

“I just knew I was going to save my sister,” she said.

Dr. David Al-Adra, transplant surgeon, UW Health, and assistant professor of surgery, UW School of Medicine and Public Health, led the surgical team that removed 70% of Ness’ liver. This allowed Dr. David Aufhauser, transplant surgeon, UW Health, and assistant professor of surgery, UW School of Medicine and Public Health, to transplant the organ into Marshall’s body.

In the first few hours of her hospital stay after surgery, the care team determined that Marshall’s MSUD was cured. Marshall was free of the disease that had ruled her life for more than 40 years.

“I am thrilled to live my life free from this chronic illness,” she said.

While Marshall remained in the hospital for nearly three weeks following her transplant, Ness returned home after a week. Both sisters needed to take it slow; their bodies were re-growing nearly an entire organ. The liver typically grows back to its full size within six weeks of the operation, according to Schafer.

“Vikki was on a mission to help her sister and our entire team was honored to give that gift to both women,” she said.

The sisters credit the team at UW Health for supporting them through the complicated, stressful yet successful transplant process.

“We are incredibly blessed to have such a wonderful team care for us through the entire donation and transplant process,” Ness said. “They truly are remarkable.”

Now nearly seven months following their life-changing surgeries, both women are embracing new routines. Marshall is adjusting to a new heart-healthy diet that includes the occasional brat and cheeseburger. She’s also enjoying more physical activity with fewer limits than before her surgery.

“I take it one day at a time, and each day gets better,” she said. “This wouldn’t be possible without the unwavering strength of my sister, our children and our mom. I am ready to embrace this new chapter of life alongside the people I love the most.”