Teen Taking on Life After Intestine-Liver-Pancreas Transplant

Zero signs of rejection despite her second intestinal transplant, thanks to advanced protocols and innovations at Stanford Medicine Children’s Health

For five years, Diana Topete couldn’t freely eat. She rarely had the chance to enjoy her favorite foods—seafood, tacos, ice cream—with her family and friends. That’s because she didn’t have any intestines.

Instead of eating, Diana was fed intravenously. For 12 hours each day, she was hooked up to parenteral nutrition, or TPN, which delivers liquid nutrients through a central line or semipermanent IV. There’s no pleasure in it, but it kept her alive.

Not being able to enjoy food and having to live on TPN were what motivated the 18-year-old and her parents to go forward with an extremely rare operation—a second intestinal transplant. This time, it would be a multiorgan transplant because she also had advanced liver disease and pancreatic insufficiency. “Going through a small and large intestinal transplant one time is a huge ordeal, and it’s rare. Going through it a second time is so rare that you can count on one hand how many children in the U.S. have done it,” says Ke-You (Yoyo) Zhang, MD, medical director of Intestinal Transplant at Stanford Medicine Children’s Health.

Her first intestinal transplant as a preschooler

Diana had her first intestinal transplant at Stanford Children’s Health when she was 4 years old. She had pseudo-obstruction syndrome—a rare, chronic condition where the muscles in the intestines do not contract normally, causing symptoms that mimic a bowel obstruction. She also had associated liver disease. Put simply, her intestines caused her pain and no longer worked well enough to sustain her growth and nutrition.

Diana’s first transplant performed well for several years, but when she turned 10, the graft (transplanted organ) began to fail. She developed a severe rejection of her graft, and a urologic surgery further damaged her intestines. She became very sick, and her intestines were ultimately removed. No intestines meant being fed by TPN and having an external bag for waste.

“My Stanford doctors tried to save it, but in the end it had to be taken out,” Diana says.

Transplanted intestines are prone to rejection

Intestines and lungs have the highest risk of rejection—likely because they function as barriers to the outside world, keeping foreign threats out. Because the intestines and lungs interface between the environment and the body, they are very immune-reactive, making them prone to rejection after transplant.

“When your body rejects transplanted intestines, bacteria can get into the bloodstream and you can get very sick. And if rejection can’t be controlled, they have to be taken out,” says C. Andrew Bonham, MD, multiorgan transplant surgeon and member of the Pediatric Liver Transplant and Pediatric Intestinal Transplant teams at Stanford Children’s. “That’s what happened with Diana.”

On the waiting list for a liver, a pancreas, and intestines

Diana lived on TPN for five years after having her intestines removed. When she turned 16, she decided she wanted a better life.

“Multiorgan transplants can seem intimidating, and there are certainly risks, but they can also have high rewards,” Dr. Zhang says. “Kids who are referred to us have often run out of options.”

Diana was getting older, and she wanted to get out into the world and experience life. She longed to go to the mall with girlfriends and laugh together over cafeteria food, shop for makeup, and be a typical teenager.

“Our number one goal is to improve the quality of our patients’ lives,” Dr. Zhang adds.

After deciding to move forward with the second intestinal transplant, Diana’s family didn’t hesitate to continue care at Stanford Children’s.

“They told us they hadn’t done a second intestinal transplant on the same person before and that we could research other hospitals if we wanted,” Diana says. “But my mom and I decided that we trusted the doctors and their abilities at Stanford Children’s, and we didn’t want to go anywhere else.”

To perform Diana’s transplant, her doctors needed a donor with all three organs—liver, pancreas, and intestines—in good shape and lacking antibodies that would trigger Diana’s immune system and lead to rejection. It was a lot to ask for, and they expected a long wait. People often wait years for a multiorgan donor match, but Diana got lucky. A donor became available within months.

“When I got the call, I expected it to not be a good match, so I was surprised and excited when it was. Diana had low-level antibodies to some of the donor’s antigens, but not enough to be a problem,” says Dr. Bonham.

A successful track record for multiorgan transplants

Stanford Children’s performs more highly complex multiorgan transplants on children than most other large transplant centers nationwide. Intestinal transplant is available at only a handful of pediatric transplant programs in the country, and the Stanford Children’s program is one of just two on the West Coast.

“We’ve been doing intestinal transplants over 25 years, and our cumulative team experience is approaching 75 years,” Dr. Bonham adds.

The Pediatric Transplant Center at Stanford Children’s is ranked number two nationwide in overall volume and outcomesand offers all organ transplants, including heart, lung, liver, kidney, intestine, and combined transplants. The center also stands apart for its living liver donor program, in which a portion of a live adult’s liver is donated to a child, helping to expand the number of organs available for transplant and shorten wait times for kids.

Prepping Diana for a successful transplant

Because Diana has been cared for at Stanford Children’s most of her life, she’s had the advantage of receiving gold-standard parenteral nutrition (TPN) care from the Intestinal Rehabilitation and Nutrition Support program. The program’s team of dedicated physicians, registered dietitians, nurse specialists, and pharmacists have special training and several years of experience supporting children with intestinal conditions.

“Our intestinal rehabilitation/intestinal failure teams are meticulous at caring for TPN lines, caring for bags, administering TPN, and staying on top of labs, good nutrition, and hydration to set transplant patients up for success,” Dr. Zhang says.

Before her transplant, Diana was put through a rigorous desensitization routine to remove antibodies that she had developed against the first and failed graft, and against blood products from past transfusions. Desensitization basically removed potentially harmful antibodies that put Diana at risk for rejecting her next transplanted intestines.

“If we hadn’t desensitized her, she would have rejected right away,” Dr. Zhang adds. “It helped that we had time to be thoughtful and deliberate, involve subspecialists, and take a team approach because her need for transplant wasn’t urgent.”

Multiorgan transplants are more complex than single organ transplants and often require additional collaborative care between multidisciplinary teams and specialists. In addition to the care that Diana received from the transplant and intestinal rehabilitation teams at Stanford Children’s, she and her family received emotional support from Child and Adolescent Mental Health, general support from Social Services, nutritional support from Clinical Nutrition, and kidney failure care from Pediatric Nephrology.

“It takes a large team to fully manage all aspects of care for this special group of patients. The fact that we all work so well together is what helps our patients succeed,” says Ashley Pedroza, PA-C, who is with the intestinal and liver transplant programs.

According to the Organ Procurement and Transplantation Network (OPTN), only 12 children in the United States had a liver-intestine-pancreas transplant in 2022.

Transplant day for Diana

When an intestine fails, scar tissue is left behind and can complicate a multiorgan transplant, making surgery more complex. Yet the 12-plus-hour transplant surgery went without a hitch. The three organs arrived intact as one unit, and Dr. Bonham was able to place them all together and attach them to corresponding veins to establish blood supply.

“The surgery went really well. There are technical aspects of a multiorgan transplant that make it slower going, but it went well,” he says.

As another step against organ rejection, Diana received plasmapheresis—a practice not common in kids but helpful for certain children who are receiving a second transplant. Basically, it’s a plasma exchange. A large IV took out all of Diana’s blood from her body and replaced it to remove antibodies that might still be in her blood.

“My family and I were all surprised that my recovery went pretty fast and there was no rejection,” Diana says. “I received the best care.”

Diana’s care team talks about what a joy she was to care for in the hospital. They found her to be determined and willing to put in the work to ensure that her recovery after the transplant went well. They describe her as a rising star who is warm and positive, and someone who takes things in stride.

“When she started to heal and feel better, you could just see this light in her that shined so brightly,” Pedroza says.

Diana spent just 14 days in the hospital after transplant.

Better-than-average outcomes for intestinal transplant

Historically in the United States, the three-year survival rate for donated intestines is about 60%. This means that within three years, over half of all transplanted intestines are lost to rejection.

“At Stanford Children’s, we have not lost a graft since 2014 because we have gotten much better at managing rejection, even compared to five years ago,” Dr. Bonham says.

He names advanced protocols within the Intestinal Transplant program for these great results, which include checking for antibodies in both the child and the donor organ beforehand to lower the chance of rejection, employing better matching methods, and using more effective antirejection drugs. Also, transplanting intestines along with a liver helps because the liver is naturally protective against rejection.

“When we get an offer, we run it through our histocompatibility (HLA) lab to determine if a donor is compatible with a recipient. The lab compares blood samples of the patient and the donor to see if the patient has pre-formed antibodies to the donor organ, putting them at high risk of rejection,” Dr. Bonham says. “It really helps us achieve great outcomes.”

Home and feeling good

Since leaving the hospital, Diana has been receiving weekly follow-ups to check her bloodwork for antibodies and to ensure that she is healing well. After transplant and still today, Diana has not had any signs of organ rejection. It’s a testament to making a good donor match and to the desensitization efforts made prior to transplant.

“Her antibodies were high before transplant, and after transplant she has had zero antibodies, something that is nearly unheard of for a second transplant,” says Katie Harmann, PA-C, with the intestinal and liver transplant programs.

Diana is enjoying getting back into life and eating food again. She still has some TPN for now, but the team’s treatment goal is to have her off of TPN and living without a bag by the end of the year. She’s making great progress toward that goal.

“At my checkups, I’ve only received good news so far,” Diana says. “I’m feeling good.”

Stanford Children’s recently launched a Pediatric Motility Program, and Diana will be the first multiorgan transplant patient to have a motility study at Stanford, an innovative practice that’s not common across the nation. A motility study looks to answer the question of why problems occur by placing catheters within the intestines that measure how they move food and liquid along their path. Targeted treatments are then recommended.

A bright future for Diana

“I plan to start nursing school this fall, and I’d like to work with children. Partly because of my experience at Stanford Children’s and partly because I’ve always wanted to be a nurse. When I was little, I would play with dolls and pretend I was a nurse,” Diana says.

The transplant team hopes she will come back after she gets her degree to work with them.

In honor of Donate Life Month, Stanford Children’s says thank you to everyone who has given the gift of an organ or donated a family member’s organs. Organ donors save and improve lives, like Diana’s.

“I really appreciate my organ donor’s family for donating organs to me,” she says. “I feel so much better.”

Learn more about our Pediatric Liver Transplant services at livertransplant.stanfordchildrens.org.