May 14, 2019

Not too long ago a child with hepatocellular carcinoma – a rare liver cancer – would have few options for treatment and even fewer chances of long term survival.

In most cases, these tumors are discovered so late that surgery and transplant are not viable options to save a young child’s life. Families could hope for recovery, but the odds would not support their optimism.

The good news is that today is a new day.

A multidisciplinary team, led by Dr. Kamlesh Kukreja, expert in Interventional Radiology; is successfully performing the Trans-Arterial Radioembolization procedure – called TARE – on children who have this rare disease.

“There are only three hospitals in the country performing TARE and we are one of them,” Kukreja said. “We are the only one in the state of Texas.”

He added that TARE has the potential to regress tumors and help patients live with their own liver or keep the tumor in control until a liver transplant is available. An additional advantage is that TARE is an outpatient procedure, which allows the child to recover at home.

How it all works

TARE is administered in two steps. Step one involves identifying the angiographic anatomy of the tumor mapping the vascular supply of the cancer to assess how much radiation it can handle, while still keeping the patient safe.

“Two to four weeks after this mapping, the patient returns for the radioactive chemical agent – called Yttrium-90 (Y90) – to be administered directly to the artery supplying the tumor,” Kukreja said. “This administration is targeted and direct, which allows for higher doses of radiation to be administered with minimal side effects; unlike systemic chemotherapy, which has been found to not work well in these cases.”

Record of Success

Kukreja has successfully performed TARE on two patients to date with one patient showing a clear regression in the growth of the tumor allowing complete surgical resection.

Although Texas Children’s is one of the pioneers of TARE in pediatrics, it’s important to note that TARE is a frontline therapy at adult hospitals for patients with the most common type of liver cancer. Accumulating evidence from studies with adult liver cancer suggests that TARE is a promising strategy which may benefit children as well.

The people that make It possible

“We are very proud of what we have been able to accomplish so far and the way we have raised the standard of care for our patient families,” Dr. Kukreja added. “We are also proud that a multidisciplinary team has made this possible.”

The decision to use this approach is first made by a multidisciplinary team involving:

Dr. Prakash Masand, Radiology
Dr. Andras Heczey, Oncology
Dr. Sanjeev Vasudevan, Pediatric Surgery
Dr. John Goss, Transplant surgery

The hands-on clinical teams at Texas Children’s include interventional radiology, nuclear medicine, radiation safety, anesthesia and Dr. Armeen Mahvash, associate professor at M. D. Anderson Cancer Center.

To Learn More

If you know a patient who could benefit from this treatment, please reach out to the department of Interventional Radiology at 832-824-5565 or liver tumor team at https://www.texaschildrens.org/departments/liver-tumor-program. Dr. Kukreja can be reached at kukukrej@texaschildrens.org/kukreja@bcm.edu.

December 6, 2016

12716transplantinside350Seventeen-year-old Shelby Standridge came down with common colds early in her childhood, but nothing out of the ordinary. A severe nose bleed at age 9, however, landed her in the hospital and prompted questions from her parents about the cause of her unexpected illness.

Doctors in her hometown in Alabama thankfully ruled out leukemia, but did a test for cystic fibrosis, which came back positive. Cystic fibrosis is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys and intestine.

At the time doctors tested Shelby for the disease, they determined her liver was already fully involved, almost to the point of end-stage liver failure. She wasn’t yet a candidate for transplant, however, so she continued battling the disease over the next five years. Throughout the past couple of years, though, Shelby’s parents and older sister noticed her lung function was declining.

“Her life had become narrow,” Shelby’s mom, Teresa Standridge, recalled.

As a result, Shelby and her family were referred to Texas Children’s, home to one of the largest pediatric transplantation programs in the nation. The team performed 86 solid organ transplants in 2015, making the program the most active pediatric transplant program in the U.S. last year.

Shelby, her mom, and older sister, Olivia, moved to Houston in October so she could be listed for a double lung and a liver transplant. A grueling wait that was expected to last six to eight months, was a mere week as Shelby underwent a 14-hour lung-liver transplant on November 8.

A multidisciplinary team of surgeons performed the procedure which is deemed so uncommon that Texas Children’s has done, including Shelby, only six of these cases since the inception of the transplant program.

“Shelby’s case is quite unusual,” said the teenager’s pulmonologist Dr. Ernestina Melicoff-Portillo. “There are only a small number of cystic fibrosis patients who have both lungs and the liver affected.”

Now, two weeks post-transplant, Shelby is feeling “ten times better” than she did and “can enjoy more in everyday life.” Her dad, Brian Standridge, noted that he hasn’t seen her grin so wide in years.

Dr. John Goss, medical director of Transplant Services, said the expertise of and the collaboration with the clinical staff and the two different surgical teams are what made Shelby’s outcome a success.

“This type of procedure only could happen at a place like Texas Children’s where our transplant program continues to earn its reputation as one of the best pediatric transplant programs in the country,” he said.

Click here to watch KHOU 11 News’ story about Shelby’s dual-organ transplant.

February 3, 2015

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Texas Children’s pediatric gastroenterologist Dr. Sanjiv Harpavat is working to ensure every newborn is screened for biliary atresia, a rare but deadly liver disease affecting 1 in 10,000 births in the U.S. It’s the number one reason for liver transplants in infants.

Biliary atresia occurs when the bile duct connecting the liver to the small intestine is blocked. The buildup of bile in the liver scars the tissue so rapidly that most infants need a liver transplant to survive.

“Infants with biliary atresia have more scarring after the first few months of life than a chronic alcoholic’s liver,” said Harpavat. “An important way to improve their outcomes is by detecting and treating the disease early before symptoms appear.”

Just like a hearing screen is a standard test for all newborns in the U.S., Harpavat hopes to implement a newborn screening program nationwide for biliary atresia. While this may seem like an uphill battle, it’s not if you’re armed with compelling data and research.

In a retrospective study published in the journal Pediatrics, Harpavat, along with a team including Department of Pathology Chairman Emeritus Milton Finegold, examined lab records of 61 children with biliary atresia referred from their birth hospitals to Texas Children’s for care. Unlike healthy infants, all newborns with biliary atresia had persistently high levels of conjugated bilirubin (CB) in their blood, which indicates a problem with liver function.

“While the total bilirubin levels of newborns are routinely checked after birth, most pediatricians are trained to look for the unconjugated bilirubin that determines the severity of jaundice in newborns,” Harpavat said. “The other type of bilirubin – direct or conjugated bilirubin – is recorded but often ignored.”

In collaboration with neonatologists at Texas Children’s Newborn Center, Ben Taub Hospital, St. Luke’s Episcopal Hospital in The Woodlands and St. Luke’s Vintage Hospital, Harpavat is leading a large, multicenter prospective screening study to validate a blood screening tool that measures CB levels in newborns shortly after birth and retests newborns with high CB levels at their 2-week pediatrician checkup to confirm biliary atresia. “The rescreen eliminates false positives since many healthy newborns have slightly high CB levels at birth while their liver is going through a critical transition period,” Harpavat said.

Of the 11,500 newborns screened in this ongoing study, 121 had high CB levels. After repeat testing, 12 babies had high CB levels and two were diagnosed with biliary atresia. This disease can only be treated with a liver transplant or the Kasai procedure, which removes the abnormal bile duct and connects the liver to the small intestine to create a new duct.

“Preliminary data suggests infants who undergo Kasai before 30 days of life have a better outcome and may have less chance of needing a liver transplant,” said Texas Children’s pediatric surgeon Dr. Mary Brandt, who performs roughly 10 to 12 Kasai operations a year at Texas Children’s. “Waiting until there is significant scarring in the liver increases the chance of needing a liver transplant.”

Texas Children’s Neonatologist Dr. Joseph Garcia-Prats, who helped implement a screening program for biliary atresia at Texas Children’s and Ben Taub General Hospital based on Harpavat’s research, says biliary atresia screening should be a standard of care for all newborns. “Unlike a stool color card test, the newborn CB screening test diagnoses the disease before infants get sick and it is inexpensive.”

To mobilize statewide support, Harpavat has collaborated with longtime newborn screening advocate and Texas Children’s Neonatologist Dr. Charleta Guillory to encourage Texas and other states to add biliary atresia to their newborn screening panel.

Harpavat has presented his findings to the Texas Medical Association, the Texas Pediatric Society’s Fetus and Newborn Committee, the March of Dimes, and the State Newborn Screening Advisory Committee that Guillory co-chairs. Harpavat also is promoting his research at pediatric conferences around the country.

“The more compelling statistics we present in the state of Texas, the more credibility we’ll have in pushing forth this newborn biliary atresia screening test on a national level,” said Guillory.

Harpavat’s goal is to screen 70,000 newborns in his study before presenting his findings to national health screening panels, like the National Screening Advisory Committee.