From the time she was 3 years old, Jayleen Lovos struggled with painful and disabling episodes of pancreatitis — inflammation of the long, flat gland tucked behind the stomach that secretes digestive enzymes and releases the hormones insulin and glucagon into the bloodstream.

With each flare-up of excruciating abdominal pain, which could land her in the hospital for weeks at a time, the damage to Jayleen’s pancreas worsened.

So, when doctors at Johns Hopkins Children’s Center presented her with an option to have a complex and life-altering surgery — to remove her pancreas and transplant its insulin-producing islet cells into her liver — Jayleen called it a “no-brainer.” She underwent the 12-hour surgery last August.

Now 16 and finishing the 10th grade, Jayleen says, “It’s kind of strange not to have pain after every meal, to be able to eat anything I want. I haven’t had any problems with my digestion since the surgery. And I’m not constantly wondering if and when I’ll have another painful attack that will land me in the hospital.”

A Game Changer

Johns Hopkins Children’s Center is one of only a few academic medical centers in the United States that offers total pancreatectomy with islet autotransplantation (TPIAT). The procedure is designed to alleviate pain for young patients who have a failing pancreas, a condition so painful that some children become dependent on narcotics to control it, though Jayleen managed without powerful painkillers except during her increasingly frequent hospitalizations.

“Jayleen has been through so much I think she developed an incredibly high pain threshold,” says her mother, Gina Tamayo.

TPIAT also aims to restore the body’s ability to make insulin after the pancreas is gone by harvesting insulin- and glucagon-producing islet cells from the pancreas and immediately infusing them into the liver, where they have the potential to take root and thrive.

“Our family and friends are all kind of amazed when we explain it to them,” Tamayo says.

The Children’s Center’s Pediatric Pancreas Center, which is overseen by pediatric gastroenterologist Kenneth Ng, was launched in 2019 to diagnose and treat a variety of pancreatic conditions including acute recurrent pancreatitis and chronic pancreatitis. Since 2021, the Children’s Center has been nationally recognized as an approved National Pancreas Foundation (NPF) Center of Excellence for pancreatitis care.

The center’s deep team of experts in everything from surgery and endoscopy to nutrition and endocrinology makes it possible to offer a procedure as complex and interdisciplinary as TPIAT. There is extensive preoperative testing and counseling, and long-term postoperative care including support for the child and the family to learn to use glucose testing equipment and an insulin pump. The goal: to keep blood sugar in the very narrow safe zone that gives the islets the best possible chance to eventually take over the job of insulin production.

Jayleen’s surgery required the close collaboration of an array of specialists, including two surgical teams — one to remove the pancreas and rebuild her digestive tract, and another to immediately process the pancreas and prepare the islet cells for infusion into her liver. During the procedure, a Johns Hopkins pathologist screened the pancreas and islet cells, because the transplant is contraindicated for kids with cancer.

Genetic testing identified abnormalities that not only put Jayleen at high risk for further deterioration of her pancreas but also for pancreatic cancer.

“That was a big reason our family decided to go ahead with the surgery,” says Tamayo. “And we were relieved that Jayleen’s pancreas turned out to be cancer-free.”

Rapid advances in genetic testing have been game changers in helping families make the decision to have the TPIAT procedure, says nurse practitioner, researcher and transplant coordinator Christi Walsh. Genetic testing identifies the mutations that are the probable causes of recurrent pancreatitis in children and can help determine the likelihood of eventual chronic pancreatitis (a constant inflammation of the pancreas), diabetes and even cancer.

“When I started doing this in 2013, we could only test for four mutations of cells in the pancreas,” says Walsh, who was the transplant coordinator for the Adult Pancreatitis Center (also an NPF Center of Excellence) before she joined Ng to launch the pediatric program six years ago. “Now we can test for up to 100, so we’re catching this younger and younger.

“The sad thing is that patients often come to us after spending years in pain, their doctors mystified and even, in some cases, dismissive: ‘It’s all in your head’ or ‘You’re just drug-seeking.’ And we can now tell them, ‘No, this is what is wrong with you, and this is what we can do about it,’” Walsh says.

In adults, pancreatitis is often caused by lifestyle choices in areas such as diet and alcohol, but for children, the cause of recurrent pancreatitis is nearly always genetic, Ng says. And because it’s genetic, recurrent pancreatitis episodes are highly likely. Each episode may damage the pancreas further, eventually leading to chronic pancreatitis. When this occurs, patients may have daily pain, and the pancreas may begin to lose key functions such as the ability to make enzymes to digest food (exocrine insufficiency) and to generate important hormones (which indicates islet cell destruction), eventually leading to diabetes.

Ng worries that families may wait too long to commit to TPIAT because between episodes, the child is often pain–free, and there is no way to predict when pancreatic function will be lost.

“One thing I tell community pediatricians is that it’s a serious red flag if a child has had more than one episode,” Ng says, “and I would hope they’d refer them to a program like ours, where we have the team and resources to take care of everything they need and hopefully prevent bigger problems long term. It’s a matter of knowing who needs to be followed, identifying risk factors and supporting patients with medical, nutritional, endoscopic or, if needed, surgical therapies like TPIAT.” Jayleen, for instance, was for years prescribed a low-fat diet to try to prevent pancreatitis.

Ng is among only a handful of physicians in the U.S. who are trained in pediatric endoscopic retrograde cholangiopancreatography, a procedure using a side-viewing flexible camera to detect and treat problems of the bile and pancreatic ducts. “Jayleen had a buildup of stones and a narrowing of the pancreatic duct, so I was able to eliminate some of the backflow and put in a stent, just to relieve some of her symptoms and buy her time to get to the transplant,” Ng says.

For Jayleen Lovos, the debilitating pain of pancreatitis is finally behind her.

Managing Blood Sugar

Pediatric surgeon Daniel Rhee led the team that removed Jayleen’s pancreas.

“Scary as the process sounds, we have excellent outcomes, and the biggest benefit is that kids don’t have to worry about ever having pancreatitis attacks again,” he says.

Jayleen’s surgery “was the longest 12 hours of my life,” her mother recalls. “I don’t know what we would have done without Christi Walsh, who kept giving us progress reports while we were waiting. And Dr. Rhee was wonderful. He was so great at explaining everything — he drew pictures for us, he answered all of our questions. He just gave us a lot of confidence.”       

The most challenging part, Jayleen and her mother agree, was that removing the insulin-producing pancreas meant Jayleen would immediately become diabetic.

“That was pretty scary at first,” says Jayleen, “and we had to learn this brand new thing — how to manage my diabetes — while I was recovering from surgery. But my mom and my stepdad and my brothers and sister were wonderful. They were like an assembly line, comforting me when I felt overwhelmed, and just helping me figure it out.”

It was a litany of critical new skills, says Tamayo, but keeping Jayleen’s blood sugar under control will maximize the chance of the transplanted islets making her eventually insulin-independent. “A few times, we got pretty emotional, but we were just determined to do it right,” Tamayo says.

About 40% of children who undergo TPIAT eventually become completely insulin-independent, says Walsh. “And the fact that Jayleen is able to manage her blood sugar so well means those islet cells are working,” Walsh says. “Without the islet cells, diabetes is very difficult to manage.”

“Jayleen and her family are just a dream,” says Kristin Arcara, the team’s pediatric endocrinologist and diabetes specialist. Arcara started working with Jayleen before the surgery and continues to monitor her. “The most important thing is that she is now pain-free. Every kid who’s had this surgery tells me they’d much rather have diabetes than not know when the next pancreas attack is going to come.”

Jayleen says she has become used to managing her blood sugar and taking digestive enzymes before every meal. For the first time in her life, she can eat pizza with her friends without worrying about pain, and she’s back to being a straight-A student, despite missing most of the first quarter of the school year to recover from the surgery. She has decided to someday work with children as a health care practitioner.

“When she told me that’s the career path she wants to take, I just got chills, because she knows what it’s like to have a severe health challenge and can help them navigate it,” her mother says. “We are all just so proud of her.”