Lucy enjoying kindergarten after liver transplant

She knows what it’s like to stay in the hospital, undergo countless blood tests and go through a major surgery most adults have never experienced. And she’s got a cool scar and a stuffed liver to prove just how brave she is.

At just 5 months old, Lucy was diagnosed with progressive familial intrahepatic cholestasis type 2 (PFIC2), a rare inherited condition. Children with PFIC2 have impaired bile secretion from the liver. This reduces bile flow and results in accumulation of bile salts in the liver cells, which damages the liver.

At this stage, Lucy’s most uncomfortable symptom was severe itching. She took special medication, which only partially worked, and had to wear pajamas with feet on them so she couldn’t scratch (a herculean effort for a five-year-old).

Lucy’s parents, Emily and Steven, knew she would likely need a liver transplant someday, but they were holding out hope that she might defy the odds. However, according to Dr. Katryn Furuya, medical director of the UW Health Kids Liver Transplant Program, most children with PFIC2 need a liver transplant before the age of 10.

“They either have end-stage liver disease, or they develop a cancerous tumor in their liver that requires a transplant,” said Dr. Furuya, who has been Lucy’s hepatologist since 2020. “We were following her closely to monitor her liver function and were screening her every 6 months for liver cancer — it is important to diagnosis it early.”

And they did. During routine lab tests in April 2023, Lucy’s providers noticed something was off. An ultrasound confirmed it — she had liver cancer.

A bridge to transplant

At that point, several UW Health doctors became involved, including Dr. Margo Hoover-Regan, pediatric hematologist/oncologist, and Dr. Eric Monroe, pediatric interventional radiologist. The transplant team at UW Health American Family Children’s Hospital began the process of evaluating and listing Lucy for a liver transplant, but no one knew how long she would have to wait for a liver to become available. So, they turned to Dr. Monroe, who is known for performing the TACE procedure.

TACE, which stands for trans arterial chemoembolization, is a minimally invasive, image-guided treatment for liver cancer. During the procedure, the interventional radiologist 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.

“It was considered a bridge to transplant,” said Emily. “We didn’t know how long we’d have to wait. Dr. Monroe’s intervention eased a lot of angst knowing we could fight this cancerous tumor until transplant. Even though this was something a parent never wishes for their child, my husband and I had a sense of peace knowing that we had exactly the right doctors at the right time. It’s as though all the stars aligned and Lucy was right where she needed to be.”

While TACE is common in adults, UW Health offers it for children, too.

“The treatment worked very quickly,” Dr. Monroe said. “When we tested her blood shortly afterward, it was clear that the chemoembolization had done its job.”

And then, on June 20, the Sughroue family received a call that a liver had become available for Lucy.

Lucy’s new liver

Now it was time for even more experts to come on the scene. Dr. Kelly Collins was the UW Health transplant surgeon who started working with Lucy when she was diagnosed with cancer. Dr. Collins was the one who had suggested Dr. Monroe treat the cancer while they were waiting for a liver to become available. Now, it was time for Dr. Collins to perform a lifesaving liver transplant.

“We have the privilege of being part of a well-oiled machine,” Dr. 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 surgery.”

Dr. David Al-Adra, transplant surgeon, helped Dr. Collins perform the surgery.

Lucy stayed at American Family Children’s Hospital for eight days following her surgery. The first few days were hard, her mom said, but Child Life specialists at the hospital kept her busy with toys and movies. And as far as Lucy was concerned, that funny-sounding organ in her body was finally working just as it should be.

Moving forward

Lucy started kindergarten this fall and she’s enjoying both swim and dance lessons. According to her mom, if you look at her, you would never be able to tell she had transplant surgery just a few months ago.

“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.”

She gets to wear whatever pajamas she wants (the itching is gone) and has the best story to tell during circle time at school.