The medicine keeping her alive was also killing her
Posted June 7, 2018 8:50 a.m. EDT
COLUMBIA, South Carolina (CNN) — Emmy Reeves shows off the My Little Pony mural she painted in her sister's room.
She points out other displays of her work around the family home: a portrait of a cat, a painting of a hillside overlooking Lake Superior, a small sculpture of her riding a wolf.
At 13, Emmy has undergone an awakening.
Just months ago, she struggled with the rarest of conditions: She was a child with Type 1 diabetes who was allergic to the insulin shots she needed to stay alive.
She was given an array of antihistamines to dampen her allergic reactions as a means of survival. But the result was that she slept about 20 hours a day -- and the possibility of dying from her daily insulin remained.
The very medicine she needed to stay alive was slowly killing her.
"It's horrifying to know that every day, you would give your child a medicine that, in essence, could kill them," mother Tiffanie Reeves said. "It's just really scary."
For Emmy, every insulin injection felt like fire spreading through her body, from her fingertips to her toes. When her parents tried to hug her, she'd flinch because the pain was unbearable.
The first time she was given an insulin shot, she stopped breathing, fainted and was rushed to the hospital. She was just 4 years old. In the months after, her parents held her down for every insulin shot. She'd scream and shout, "I'll be a good girl!"
"We have to do this because we love you. This is what keeps you alive," her parents would say.
For nine years, she suffered like this. Beyond the pain, she developed cataracts and often broke out into extreme rashes. She was medicated so much, she struggled with memory and an array of other issues.
Her parents tried everything. They took Emmy to top medical institutions and spoke with insulin companies to get the least amount of preservatives in her insulin, because that's what they believed was causing the allergic reactions. Nothing seemed to work.
Desperate, her father wrote an email last year to Dr. Raja Kandaswamy, one of the nation's pre-eminent pancreas transplant surgeons. "Her quality of life is degrading faster than we had hoped, so now we are looking for a solution that will allow her to stop needing insulin, while we find a solution to her allergy," Jack Reeves wrote.
More than 1 million Americans have Type 1 diabetes, an autoimmune condition that destroys the insulin-producing cells in the pancreas. Insulin is a hormone needed to allow sugar to enter cells to produce energy. For most children, the condition is managed through insulin injections and a close monitoring of their blood sugar levels. It is exceedingly rare for a child to have an insulin allergy on the magnitude as Emmy.
Emmy's father wasn't looking for a cure for her diabetes. He was simply hoping to give his daughter an improved quality of life to help manage her diabetes. Her parents feared that she would die within a year if she stayed on the same course of treatment.
But that email set in motion a chain of events, resulting in the nation's first sole pancreas transplant of a child in nearly 20 years. It also led to his daughter being cured of her diabetes.
"It's amazing. She's a totally different child," her mother said. "Every day that she does not reject this organ, I'm going to hug her, even though she may get tired of Mommy hugging her."
'You couldn't touch her skin without her recoiling'
Kandaswamy, the director of the pancreas transplant program at University of Minnesota Health, remembers receiving the email from Emmy's father "like it was yesterday."
Kandaswamy, who has two daughters himself, felt the pain of a father who desperately wanted help for his child. But there was little medical literature to guide or support a pancreatic transplant in a child. There have been other cases in which children receive a new pancreas, but those transplants are usually done in conjunction with kidney and intestine transplants.
Only nine children have received a sole pancreas transplant, Kandaswamy said. The last time a pancreas transplant was done in a child this young, he said, was nearly 25 years ago. That transplant was performed at the University of Minnesota in 1994 on an 11-year-old boy by Kandaswamy's mentor, Dr. David Sutherland, who is considered the father of pancreatic transplants. Unfortunately, that pancreas only lasted six months in the boy.
"Pancreas transplants are generally just not done on children," Kandaswamy said.
But his mentor taught him to always put a loved one in that patient's place and ask yourself, " 'What would you do if it was your mother or your daughter who needed the care?' Ask that question, and you will get your answer."
Kandaswamy said that's exactly what he did when Emmy first visited. "I have two daughters of my own. One of them is very close in age to Emmy," he said. "Looking at her, you could see: 'Hey, if this was your daughter, what would you do?'
"You couldn't touch her skin without her recoiling," said Kandaswamy, who is also a professor in the department of surgery at the university's medical school. "Her quality of life was just deteriorating to the point that this was a cycle that would not be compatible for long-term survival."
He had to educate others in the hospital about why he felt the transplant would work for a child. The university had a new pediatric hospital, and if the surgery didn't go as planned, the institution's reputation could be adversely impacted.
"There was a huge amount of apprehension," Kandaswamy said. "I knew we were taking a big risk in that this may not go well."
A team of more than a dozen doctors was put together to study Emmy's case: surgeons, anesthesiologists, nephrologists, allergists, transplant coordinators, pain management specialists and others.
After a four-day assessment in April 2017 at the University of Minnesota Masonic Children's Hospital, they agreed that a full pancreas transplant was the best option of giving her a better quality of life.
"We didn't make the decision without due thought and process," Kandaswamy said. "We had to do it because of the extenuating circumstances here."
Kandaswamy felt that in the nearly 25 years since that first pancreas transplant on a child, the surgical techniques and post-transplant management have advanced to the point that his team could "successfully perform a transplant in a child this young."
A healthy pancreas would allow her body to naturally produce insulin -- effectively curing her diabetes -- but the surgery carried many risks, including the possibility her body would reject the organ.
"Pediatric patients, they have very active immune systems, and they will try to spit out any kind of organ," Kandaswamy said.
Emmy and her mother would also need to move to Minnesota from South Carolina to be near the hospital the moment a pancreas became available.
Before she headed north, though, Emmy created a bucket list: eat jambalaya in Louisiana, feast on a steak in Texas, visit the Grand Canyon and see her friends from diabetes camp in California, where she once lived.
The family hit the road. Emmy got to do everything on her list.
It was, her parents say, a farewell trip, should she not survive.
False alarms and the magic moment
Emmy and her mother arrived in Minnesota on July 15. They settled into the Ronald McDonald House, where families stay for free during visits at the hospital. They then had to wait for a pancreas to become available.
Three times, the calls came that an organ might be available. Each time turned out to be a false alarm. The second was on Thanksgiving.
At one low point, Emmy suffered a severe allergic reaction in January; she stopped breathing and had to be rushed into emergency care. Still, she battled on.
Then came the moment. On the afternoon of February 9, Kandaswamy called Emmy's mother with news that a pancreas was available. Emmy was watching a documentary about wolves, her favorite animal, with her tutor at a museum.
Mom let her finish watching the film and then told her it was time. They needed to rush back, gather up some belongings and get to the hospital. When they got to the Ronald McDonald House, the place was abuzz. Word had spread like wildfire that Emmy's moment had arrived. Families and staff cheered them on and wished them well.
At the hospital, Kandaswamy greeted them in the lobby. He explained that a procurement team had gone to get the organ about an hour away in St. Cloud -- that there was still a possibility it might not be a proper fit but that he was optimistic.
Emmy was prepped for the surgery so she would be ready when the pancreas arrived.
About 3 a.m., Kandaswamy conferenced in Jack, who was back in South Carolina with Emmy's younger sister. "I think we are going to go ahead. The organ looks good," he said.
On Emmy's 210th day in Minnesota, the transplant began. That number isn't lost on the family: She was wheeled into the operating room on 2/10.
As Emmy underwent surgery, Mom clutched a tattered yellow stuffed animal named Ducky, who was given to Emmy after she was diagnosed with diabetes.
The surgery took more than four hours. "It was a great, great feeling when we got her done," Kandaswamy said. "But I knew in my mind that the job was only half done. We were excited but also cautiously optimistic at that point."
The doctors had to make sure the new pancreas -- what Emmy nicknamed "Player 2" -- worked properly, that her body didn't reject it and that her body could tolerate the immunosuppressive drugs she needed after the transplant.
She responded well the first week, but in the second week after the transplant, her body began to reject the organ. Her immune system saw it as foreign and tried to attack it.
"I had to come and tell her that her body is in rejection," her mother said. "We're not sure what's going to happen in the future."
They decided to try a stronger immunosuppressant. "We're already down the rabbit hole, Mom. We gotta do what we gotta do," Emmy said.
Ever since, her pancreas has functioned as hoped.
Dr. Melina Bellin, the pediatric endocrinologist with University of Minnesota Health who worked with Emmy, said it was "touch-and-go" early on, but everyone was relieved when Emmy's health turned around.
"It's particularly rewarding when you're at that point in the beginning when you're not 100% sure it's going to turn out the way you wanted," she said. "It definitely makes it that much better when it does."
Kandaswamy added, "We were able to treat it effectively and got it turned around."
With the healthy new pancreas, he said, her diabetes has been cured. She no longer needs insulin shots or the antihistamines that she was taking to try to limit her allergic reactions. The hospital slowly weaned her off all of those medications that were making her extremely drowsy and "blunting her consciousness," Kandaswamy said.
"Then we saw the real Emmy. She started really blossoming," he said. "It's like a cloud has been lifted off her consciousness so she can actually express all of this stuff in a big way."
One of the greatest moments of his career, Kandaswamy said, was when Emmy hugged him: "There was nothing more fulfilling than seeing her walk up, give me a hug and say, 'I want to go back home.' It's the greatest thing I've ever heard."
Pancreas transplants are not recommended for most children with diabetes, but Kandaswamy said he hopes this case will open the way for children with rare conditions like Emmy who could benefit from a pancreas transplant. "I think this will be an index case that will help spark that awareness," he said.
Speaking up to help others
In the family home in Columbia, South Carolina, Jack and Tiffanie Reeves are still in near disbelief at how well Emmy is doing. She's thriving in ways they could only dream of. Beyond her artwork, she's started playing the piano. She can go on hikes. She can shoot hoops. She can play with her younger sister, Abby.
"She's getting a childhood, finally. She's getting to be able to be a kid," her mother said. "I mean, this child's achieved so much in just the three months since transplant. It's going to be pretty incredible to get to see what she'll be able to do in the next few years."
The family agreed to tell Emmy's story in hopes that it can help other children suffering as she did. They know of four young children around the world with a similar insulin allergy as she.
"If our experience could help even one kid, I think it would be worth it," Tiffanie said.
Emmy is not out of the woods. The first year after a transplant is especially critical. Her body could still reject the organ. The family tries not to dwell on that. "We still live life, and we don't let the worry and stress of that keep us and our daughters from living the life that we need to," her father said.
Emmy hopes to be a zoologist one day.
For now, she's shy around CNN's camera and doesn't want to talk with cameras rolling. When they're off, she stands next to a window overlooking her backyard and a decaying swing set.
She says she's drawn sketches of what she envisions back there: a greenhouse, a new swing set, a fire pit and a fence.
When the Make-A-Wish Foundation reached out, she told them she didn't want to travel anywhere fancy or meet a famous star. Instead, she shared her vision of a new backyard, a place where she can finally be herself.