Portal Hypertension: Taylor’s Story
Meet Taylor
Taylor has overcome more health challenges at 13 years old than most people face their entire lives. After an early fight against cancer as a toddler, she developed life-threatening portal hypertension as a pre-teen. The Liver Care Center at Children’s Mercy Kansas City was able to give Taylor a surgical solution to get her healthy and back home.
Taylor was diagnosed with stage III neuroblastoma at 18 months. Her treatment plan used almost every tool in the box: chemotherapy, tumor-resection surgery, stem cell transplant, radiation and antibody therapy. Most of Taylor’s cancer care was at Blank Children’s Hospital in Des Moines, Iowa, except for the stem cell transplant, which they did at Children’s Mercy. She finished her treatment and was declared in remission in 2013.
In 2015, Taylor’s family moved to Kansas City from Iowa. Taylor was a mostly healthy kid, though she tended to get a little sicker than her four siblings when they came down with sniffles. When the entire family got Influenza A the week of Thanksgiving 2022, they thought her lethargy was just Taylor’s body taking a little longer to fight back, as usual.
“I felt really woozy and sick,” Taylor remembered. When she began experiencing gastrointestinal bleeding, they realized this wasn’t the run-of-the-mill flu.
The problem: Portal hypertension
Taylor’s parents took her to an urgent care and then to an emergency room as her condition deteriorated. She was transported by ambulance to Children’s Mercy, where the Gastrointestinal (GI) team did an initial scope and discovered varices—weak, twisty abdominal veins prone to bleeding.
“If something doesn’t feel right, it’s probably not right,” said Taylor’s mom, Julie. “I’m glad that we went to the urgent care [when we did]; we might not have made it to the hospital.”
“I remembered sitting there with the IV in my hand, but I don’t remember that much,” said Taylor, whose hemoglobin levels had fallen steeply.
The GI team called Voytek Slowik, MD, transplant hepatologist. Dr. Slowik was able to come in on Thanksgiving to band Taylor’s varices endoscopically and stop the current bleeding.
“Banding is a temporary measure,” explained Dr. Slowik. “You get rid of the varices that are there, but then you have to do something to fix the underlying disease.”
Dr. Slowik and the rest of Taylor’s Liver Care team did a CT scan, liver biopsy and interventional radiology tests to arrive at a diagnosis of portal hypertension. Her portal vein, which takes blood from the abdominal organs to the liver, had developed a clot.
"When you have a portal vein blood clot, it can cause the blood vessels in your body to try to figure out a new way to get back to the heart,” explained Dr. Slowik. That’s what varices are, a “reroute” that doesn’t work very well.
In addition to gastrointestinal bleeding, portal hypertension can also cause abdominal swelling, an enlarged spleen with low platelet numbers, disorientation and other complications. Chronic illnesses, like cancer, raise the risk of blood clots and the portal hypertension that often follows, but they can happen in the general population, too.
Fortunately, the Children’s Mercy Liver Care Team has a surgical option that provides a long-term solution for portal hypertension without resorting to a liver transplant: Meso-Rex Shunt surgery.
The solution: Meso-Rex Shunt surgery
Bhargava Mullapudi, MD, Division Director, Transplant Surgery, came to Children’s Mercy the same year Taylor’s portal hypertension reached a crisis point. Complex liver surgeries are one of his specialties, and he explained how Meso-Rex Shunt surgery works:
“When the portal vein gets clotted off, there’s no way for the blood to get back to the heart other than the abnormal channels, the varices,” said Dr. Mullapudi.
Surgeons take a piece of the jugular vein from the patient’s neck to create a new pathway around the clot. It goes between the superior mesenteric vein and the area of the liver called the Rex recess. (Meso-Rex!)
“Now the blood goes through the liver and out of the liver (as it is supposed to), so you’re back to utilizing your liver for blood flow and processing,” said Dr. Mullapudi. “When done early, Meso-Rex Shunt surgery will avoid worsening liver dysfunction and a potential transplant. In certain cases, the liver is too fibrotic or cirrhotic, and other types of shunt surgeries or a transplant may be needed.”
Taylor continued to experience bleeding before the scheduled surgery, so she was admitted to the PICU, where they could keep her stabilized and maintain her strength before surgery. Physicians banded her varices multiple times before her Meso-Rex Shunt surgery on January 23, 2023.
“Everybody just stayed calm and professional, so it kept us calm,” said Julie. “She was in a very precarious position, and your mind goes to the worst-case scenario, but they were good at checking on us. Dr. Mullapudi would come in for a half-hour and just chit chat. It was so nice.”
A reroute that works
By the time the 23rd arrived, Taylor was so ready to be done with the hospital that she wasn’t even nervous before the surgery. But for her family, waiting for Taylor to get out of the operating room brought flashbacks to her earliest cancer treatments.
“I told somebody my body knew how to respond to the shock because we had been through it,” said Julie. “I think we had 20 people waiting with us, so that helped pass the time.”
Taylor’s surgery went well, but because she had been so sick beforehand, recovery took a little longer. “It was weird,” Taylor said of the recovery time. “It was just hard to eat.” Taylor slowly regained her appetite and her strength, and her platelet counts rebounded. She was discharged on Jan. 31, 2023.
By late spring, she was feeling more like herself and able to enjoy spending time with her family again. She was on anti-coagulants and then aspirin to prevent clotting but has been off medication since February 2024. Going forward, she’ll have regular blood work, liver function tests and ultrasounds to make sure that the shunt is staying open.
“Our goal is for these patients is to grow up and never require a transplant or have bleeding issues,” said Dr. Mullapudi. Sometimes very young Meso-Rex patients will need to have their shunts dilated or redone as they grow. “Taylor was old enough that it is unlikely she will need anything more done.”
Taylor is back to living a healthy life and sees a physical therapist and chiropractor in addition to her regular Liver Care check-ins. She likes reading, hanging out with her sisters and swimming in the summer.
“She’s brilliant,” said Dr. Slowik. “She’s been through a lot. She toughed it out and recovered.”
Throughout the entire process, Julie said their family was comforted by how the Liver Care team made sure they had all the information they needed and took time to answer questions.
“They were all great,” said Julie. “If Dr. Mullapudi hadn’t been here, we would have had to travel to Chicago for treatment ... or I don’t even know. I don’t like to think about what would have happened. We’re just thankful he was there.”
