MADISON, Wis. – The first thing Emily Sughroue will tell you about her daughter Lucy is that the 7-year-old is a character, with a passion for dance and a mischievous grin.
The second thing is that trying to stop a 7-year-old from itching is nearly impossible.
“We always kept her nails short and she had to wear footed pajamas to sleep so she wouldn’t itch her skin raw at night,” Emily said.
The itching was a result of progressive familial intrahepatic cholestasis, referred to medically as PFIC2, a rare inherited condition. Lucy was diagnosed with PFIC2 when she was 5 months old. Children with PFIC2 have impaired bile acid secretion and as a result, bile acids build up in the liver cells, leading to extreme itchiness and poor growth and weight gain.
Most children with PFIC2 need a liver transplant before the age of 10, according to Dr. Katryn Furuya, medical director of the pediatric liver transplant program at the UW Health Transplant Center.
“They either have end-stage liver disease, or they develop a cancerous tumor in their liver that requires a transplant,” said Furuya, who is also a professor of pediatrics at the University of Wisconsin School of Medicine and Public Health. “Since I first started to see Lucy in 2020, we screened her every six months looking for signs of liver cancer in addition to monitoring how well her liver was working.”
During routine lab tests in April 2023, a blood test indicated that Lucy had liver cancer. Following an ultrasound and MRI, her liver care team confirmed she had liver cancer and began the process of listing Lucy for a transplant and treating the cancer.
“While this was something a parent never wishes for their child, my husband and I had this sense of peace knowing that we had exactly the right doctors at the right time,” Emily said. “It’s as though all the stars aligned, and Lucy was right where she needed to be.”
To treat the cancer, Dr. Eric Monroe, pediatric interventional radiologist, UW Health Kids, was called upon to perform trans arterial chemoembolization, a minimally invasive, image-guided treatment for liver cancer. During the procedure, the physician inserts a catheter into an artery in the child’s leg, using it to deliver tiny beads of chemotherapy directly to the tumor in the liver. While common in adults, UW Health is one of the few health systems in the country that offers this procedure for children.
“This was considered a bridge to transplant since we did not know how long Lucy would have to wait for a new liver,” said Monroe, who is also an associate professor of radiology at the UW School of Medicine and Public Health. “The treatment worked very quickly; when we tested her blood and looked again with an MRI shortly afterward, it was clear that the chemoembolization had done its job.”
Less than a month after the procedure, on June 21, the Sughroue family received the call that a liver had become available for Lucy. Dr. Kelly Collins, surgical director of the pediatric transplant program at the UW Health Transplant Center, and associate professor of surgery at the UW School of Medicine and Public Health, performed the surgery. She was assisted by Dr. David Al-Adra, transplant surgeon, UW Health, and assistant professor of surgery, UW School of Medicine and Public Health.
“We have the privilege of being part of a well-oiled machine,” Collins said. “There was a team that flew to where the organ was, and when it came here, we had a highly trained team that prepared Lucy for a successful surgery.”
Lucy stayed at American Family Children’s Hospital for eight days following her surgery. While there were some hard days, Emily said the child life specialists at the hospital eased the entire family’s stress with toys and movies.
“It’s still anxiety-provoking every time we get labs,” Emily said. “But we’ve tried hard to make life as normal as possible. We don’t want her to feel that this defines her. We tell her that every time she sees her scar, it should remind her of how brave she is.”
Following Lucy’s transplant, the blood test for liver cancer was rechecked and the results were negative, and the chances that the cancer had spread were small, according to Furuya.
“It takes a village to provide care for a successful pediatric liver transplant, it takes a team of specialists,” Furuya said. “We are fortunate that UW Health Kids has so many resources available for children, so that when they get sick we can provide that highly specialized care that will allow them to survive and return to being kids.”
In the year since her transplant, Lucy has jumped back into activities including swim lessons and dance classes near her home in Dane County.
“This is a chance for her life to start again,” Furuya said. “Seeing her thrive and have a more normal life is the best thing ever and I am so excited for her.”
