Surgery Restores Blood to Liver & Provides Renewed Life for Young Child

August 21, 2018


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At four years old, Paige, now 10, was diagnosed with portal hypertension. The vein that delivered blood from her intestines to her liver suffered from high blood pressure, resulting in internal bleeding and pending liver failure, as well as other symptoms that could not be explained by her diagnosis. “It just wasn’t adding up,” said Paige’s mother, Victoria Humphreys.

Desperate for answers, Victoria and Andrew, Paige’s father, sought out physicians in their home country of Australia, and also conducted an online search for surgeons around the world who had the expertise needed to perform a life-saving surgery on their daughter. It wasn’t until their quest for answers led them to Lurie Children’s that they began to feel hopeful.

Finding Hope

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A patient’s meso-Rex bypass (MRB - arrow) at six-months post-surgery, shows how the MRB joins the superior mesenteric vein (SMV) to the portal vein (PV) inside the liver, thus bypassing an obstructed main portal vein outside the liver.

Through their search, Paige’s parents found Riccardo Superina, MD, a pediatric surgeon and the head of Transplant Surgery at Lurie Children’s. They connected via email and shared details of Paige’s journey. Instantly, Dr. Superina provided answers to questions the Humphreys posed about their daughter’s condition. With the newfound information in tow, the parents decided that Lurie Children’s was the best place for their daughter to get the care she needed. The surgery, known as the meso-Rex operation, or “rex shunt” for short, would bypass the blocked portal vein and restore venous blood flow to Paige’s liver.

The use of the restorative shunt began at Lurie Children’s in 1997.  First developed in Europe, Lurie Children’s was the first to apply this novel technique in North America and now has the most extensive experience in the world with this unique surgery. Over the last 10 years, Dr. Superina and his team have worked at perfecting the procedure even further.

The Humphrey’s found that Dr. Superina was the only surgeon in the world willing to take her on.

Australia to Chicago

The Humphreys traveled nearly 9,000 miles to Chicago to prepare for Paige’s surgery, bringing along Paige’s siblings Melody, 8, Leyland, 6, and Abel, 2.

Once in Chicago, Joan Lokar, APN, Advanced Practice Nurse for Transplant at Lurie Children’s, took time to speak with Paige’s parents and form a solid relationship with the Humphreys. “In the first 10 minutes, she gave us more answers than we’d had in 10 years,” Andrew said. Once Paige was at Lurie Children’s, she was diagnosed with portopulmonary syndrome. An echocardiogram showed that the liver disorder had affected the blood pressure in her lungs, making the operation and the subsequent recovery more hazardous.

Even with reassurance from others, sending Paige into the operating room caused her parents more turmoil than they had ever experienced, Andrew said. Within just 60 minutes of the surgery start, however, they received the best news of their lives: their daughter would be able to undergo the operation successfully and begin the long road to recovery.

The Road to Recovery

After the successful surgery, Paige recovered in Lurie Children’s Lefkofsky Family Pediatric Intensive Care Unit (PICU). “The amount of care that went into her recovery was mind-boggling,” Andrew said, referring to the round-the-clock care Paige received from PICU physicians, nurses, therapists, child life specialists and more. “Everyone went to every length to make sure she got everything she needed.”

“Directly after the surgery, Dr. Superina said, ‘I didn’t really believe you’d ever make it here,’” Paige’s father recalled with tears in his eyes. “That was a sign of how generous this man was: He didn’t even expect that we’d ever get here, but he still did his best to help out.”

“Dr. Superina saved her life by giving us hope,” he said.

Three weeks into her recovery process, Paige said she felt better than ever before. Until 2018, Paige continued recovering as an outpatient at Lurie Children’s under the supervision of Dr. Superina and the rest of the transplant team, with her family by her side. In May the family returned home to Bora Ridge, a small town near Brisbane, more than six months after first arriving in Chicago. Her portopulmonary syndrome had improved, and it was safe for her to make the long trip back home. They will be returning in the fall as part of the long follow-up process necessary to ensure good long-term graft function. Their long-term hope is to raise funds to send more children to Lurie Children’s who are afflicted with portal vein thrombosis like Paige.

“Her successful treatment here is a tribute to the hepatobilary operating room team including anesthesia and nursing at Lurie Children’s,” said Dr. Superina “and the multidisciplinary effort that guided her recovery, which included PICU, cardiology, the pulmonary hypertension team and the nurses that cared for Paige during her long stay in Chicago.”