Haiden Taylor, who suffers from cystic fibrosis and chronic pancreatitis, underwent a two-part surgery over two days to relieve her pain and maintain her body’s ability to produce insulin.
By Maureen Gilmer, IU Health senior journalist, mgilmer1@iuhealth.org
Haiden Taylor hasn’t had the easiest life. The 11-year-old has been in and out of the hospital for years, dealing with cystic fibrosis and chronic pancreatitis.
Pain has been her constant companion.
But now, thanks to the efforts of a multidisciplinary team of doctors, nurses and specialists at Riley Hospital for Children, she has a little bit of hope.
Haiden underwent a rare two-part surgery earlier this summer to relieve her near-constant pain while also managing her body’s ability to produce insulin.
It’s called a total pancreatectomy with islet auto transplantation. TPIAT for short. And she is the first pediatric patient within IU Health to have it done.
DEBILITATING DISEASE
Chronic pancreatitis is an inflammatory disease that leads to scarring of the pancreas, increasing levels of pain and repeated hospitalizations. The pancreas is important because it regulates digestion and sugar levels in the body, so although it can be removed to alleviate pain, people are left without the cells that make insulin and other hormones that help maintain safe blood sugar levels. These people develop diabetes.
In Haiden’s case, doctors, in consultation with her parents, Billy and Megen Taylor, felt that removing her pancreas was the best option. The important part for her is that it does not have to mean a life of insulin shots. That’s where the islet auto transplantation comes in.
Dr. Nicholas Zyromski performed the operation to remove her pancreas (the source of her pain), gall bladder and part of her small intestine, then reconstructed her digestive system. A lab team isolated her insulin-producing cells from the pancreas, and the next day, the interventional radiology team at IU Health University Hospital reinfused her islet cells into her liver.
The hope is that they will produce enough insulin to help her lead a normal life. There’s no guarantee, but the procedure has worked in adults within IU Health and elsewhere.
Pediatric gastroenterologist Dr. Brian McFerron said typically a year out from the procedure, a third of patients may require insulin, a third may require a little with meals, and a third may require none.
“It’s been a very tough road for Haiden,” he said. “Chronic pancreatitis can be debilitating. This procedure is a way of hopefully giving her a chance at leading a normal life.”
FAMILY MOVED TO BE CLOSER TO RILEY
Haiden’s chronic pancreatitis had reached the point where there were no additional interventions to control her pain or to stop the ongoing inflammation and scarring in her pancreas, Dr. McFerron said.
“We met with the family numerous times, and they had done plenty of research and knew what the procedure entailed. We all agreed to proceed.”
Haiden’s family was so committed that they moved from southern Illinois to Indianapolis to be closer to Riley, he said.
“We were honored by that. We have all the pieces in place for the team it takes to successfully do this procedure,” Dr. McFerron said, “including preoperative evaluation, surgery, the transplantation and close follow-up afterward. It takes a very large team – surgery, GI, pulmonology, endocrinology, dietitians, pain management, mental health services and critical care. All of those people played a role.”
Dr. Zyromski agrees: “We are able to deliver this type of high-quality, high-complexity care in the setting of a functional multidisciplinary team.”
Haiden was hospitalized for 23 days in June at Riley, before and after the surgery. She went home for about a week, then returned for a week mostly due to abdominal discomfort, her mom said. That back and forth is lessening now, and Haiden was at Riley a couple of weeks ago for a checkup with pediatric endocrinologist Dr. Juan Sanchez, one of her many doctors and among her favorites, her mom said.
“He’s awesome,” she said.
Another favorite is Dr. Molly Bozic, a pediatric gastroenterology specialist.
“We have a list of doctors a mile long,” Megen said. “We have met a lot of special nurses, too.”
Haiden hopes one day to be one of those nurses. At Riley, of course.
STRONG AND WISE
The sixth-grader was pulling an IV pole around with her in the lobby of Riley’s Simon Family Tower earlier this month, something she is used to doing. She can eat, but it’s not always easy. The IV pumps formula into her thin frame to bump up her nutritional intake.
She is wearing a shirt made by her aunt that says, “Pancreatitis survivor – It’s not for the weak.” Next to her, Megen wears a shirt with the message “Cystic Fibrosis Awareness.”
“She’s the strongest, kindest child I have ever met,” Megen says of her daughter. “She’s like an old soul, wise beyond her years. Sometimes you wouldn’t know anything is wrong with her.”
She’s human though, and when the pain flares up, her spirits can sag. Counseling services, part of that multidisciplinary team approach, take the edge off and help her navigate her illness.
She doesn’t let it keep her down for long, her mom said. The family which includes Haiden’s two brothers, goes camping and boating nearly every weekend. Haiden also likes to ride dirt bikes, play video games and bake cookies.
HOPEFUL SIGNS
It’s too soon to tell how well her body will adjust post-surgery, but doctors are encouraged by her progress.
“She is doing well. She is progressing as we would expect, and we are certainly hopeful,” Dr. McFerron said.
Chronic pancreatitis is a challenging disease to treat, Dr. Zyromski explained. It most often afflicts adults who have abused alcohol or are cigarette smokers.
“There is a very small percentage of chronic pancreatitis patients whose disease stems from a genetic mutation. Haiden is one of those patients,” he said. “The treatment of this disease is extremely challenging. There’s no medical therapy, and in fact surgical therapy aims to treat the consequences of the disease. Haiden’s situation is that she has a variant that we call small duct pancreatitis and the treatment for that is removing the entire pancreas. TPIAT is accepted as standard therapy for very highly select patients with chronic pancreatitis and for sure patients with a genetic predisposition like Haiden has.”
The surgery protocol, developed in the 1970s at the University of Minnesota, is done in only a handful of medical centers in the country, including IU Health.
Dr. Zyromski said he saw Haiden a few weeks ago, and she definitely is improving, though it will take several months to see if the islet cells transplanted to her liver will produce more insulin.
“Her sugar regulation has been quite good,” he said. “The endocrine team at Riley is helping take care of that. She’s got a long way to go, but I think she’s going to be OK.”
Photos submitted and by Mike Dickbernd, IU Health visual journalist, mdickbernd@iuhealth.org
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