A 12-year old South Carolina girl with Type 1 diabetes and what her parents describe as an intense allergy to insulin therapy is set to undergo a rarely performed whole pancreas transplant at the University of Minnesota.
Jack and Tiffanie Reeves, parents of Emmy, say they will take her on a cross country trip to diabetes camp in California, including a stop at Universal Studios in Orlando to “build memories,” and then move her temporarily to Minnesota with her mother as they wait for a pancreas to become available.
The surgeon who will perform the transplant, Dr. Raja Kandaswamy, Director of the Pancreas and Intestinal Transplant Program at the University of Minnesota , says Emmy’s transplant will be the first one done at his center in the past 15 to perhaps 20 years. And while Kandaswamy points to Emmy’s situation as unique, he also says he feels that pancreas transplantation is an option too few pediatric endocrinologists consider for patients. “There is not as much enthusiasm as there should be,” Kandaswamy said. “The diabetes community is told [with a transplant] they are just trading off one set of risks for another.”
In some cases, he disagrees. “I think it can be a viable choice,” he said.
Kandaswamy said that in recent years, steroid use in transplant patients has been cut down to near zero, meaning less harsh medications after a transplant. “We are looking at a new era of transplant,” he said. “The nature of it has changed.” He added that when islet cell transplantation is approved for minors, he foresees transplantation becoming more popular. But an islet cell transplant is a relatively simple procedure that infuses insulin-producing cells into the liver’s portal vein via a catheter. A full organ transplant is vastly more complicated, and according to the Mayo Clinic, “it’s not unusual for pancreas transplant recipients to experience an acute rejection episode within the first few months after the procedure.” The Reeves family had hoped, when contacting Dr. Kandaswamy, to get Emmy an islet cell transplant. They are yet to be approved for children though, and thus, the full organ transplant was suggested.
Dr. Kandaswamy points out that Emmy will be placed on a lifetime of immunnnosupressents that can lead to possible side effects, such as kidney damage, down the road. “No drug is without risk,” he said. In addition, her pancreas will have a shelf life of about eight to ten years, meaning other transplants will be in her future.
Dr. Kandaswamy first came in contact with the Reeves family after Mrs. Reeves published an online account of her daughter’s apparent allergic reaction to insulin or one of its ingredients, and their struggle to find solutions. A reader of the post reached out to Reeves and suggested she contact the University of Minnesota.
According to documents kept by the Reeves and shared with ASweetLife, Emmy’s first reaction to insulin happened just days after her diagnosis, while she was still in the hospital. At the injection site, the family saw red spots and Emmy experienced itchiness and respiratory distress. While they were able to avoid the respiratory reaction by changing the brand of insulin, the skin reactions continued. Over time, those reactions varied from itchy red spots to painful rashes. Over the past eight years, the family has tried varied insulins, and believes the allergic reaction is most likely to a product in insulin called Polysorbate-20 that functions as a stabilizer.
The Reeves family, with medical guidance, treated the reaction with allergy medications, and attempted treatment with steroids as well. Antihistamines helped curb the reaction, but left Emmy lethargic most of the time. Those side effects, Dr. Kandaswamy said, the pain and hypersensitivity along with the side effects of the drugs, greatly impacted his team’s decision to go forward with the transplant. “[The treatment drugs] are making a zombie out of someone,” he said.
In some cases an insulin allergy is minimized with an extremely low carb diet (thus cutting back on the amount of insulin needed and therefore, often, on the reaction). Another technique is “desensitization,” a process of helping the body go with as little insulin as possible for a period of time and then re-introducing it to the body.
For Emmy, her parents said, the options weren’t viable. Emmy was put on a low carb diet for a short period of time combined with a high dose of the steroid, prednisone. In their notes, the family wrote that “The downside [of the diet] was that Emmy was hungry all the time and we had to increase her fats which had us worried about her heart health.”
Desensitization, Mr. Reeves said, was not attempted. “We never did desensitization. It had to do with being logistically impossible to do.” In their notes, the family outlines a period of time trying to get the process to happen in California, but giving up when a specific drug was unattainable and doctors stopped calling them back.
Dr. Kandaswamy, though, does not have an issue with this, since in his opinion, regardless of her insulin allergy, Emmy’s hypoglycemic unawareness, a condition in which a person with diabetes does not experience the symptoms of low blood sugar, gave his team all they needed to give the transplant the go ahead.
He said their research into Emmy’s condition found that she has extreme hypoglycemic unawareness. While that is never noted in the parents’ notes, (and both parents confirm Emmy has never fallen unconscious in reaction to insulin, she has experienced blood glucose as low as 20 without feeling it, Dr. Kandaswamy said. “Even if she tolerated [a low carb program to minimize the allergic reaction], she has profound hypoglycemic unawareness,” he said.
Mrs. Reeves, however, said that hypoglycemia unawareness is not the reason she decided to allow her daughter to be transplanted. “The hypos of 22 and not feeling them really wasn’t and isn’t on our list of reasons. I could see how it would be on his and he did explain it as one of three reasons.” Emmy’s parents simply want her to be able to take insulin without pain or distress.
And so, after camp, Emmy and her mother will settle in Minnesota and wait for a pancreas to become available, something Mrs. Reeves said they don’t expect to take too long since there is so little demand for pediatric pancreases. And while she is nervous, Reeves says she’s ready, despite the worries of a whole organ transplant surgery, a long recovery, the medications needed after, possible complications of the surgery, and of course, knowing a transplant will be needed once again in the future. The idea of her daughter living without pain and with a “semi-normal life” is her driving force.
“Emmy is tired of the tests, tired of the doctors,” Mrs. Reeves said. “Had we done this years ago, yes, she’d be on immunosuppressants but the life she would have had would have been so much better. She would have gone to school (she is home schooled). She would have been able to go over to a friend’s house to play. Yes, there would have been some other things to deal with, but her quality of life would have been so much better. What she’s living now? This is not a life. There are days when I wish we could have done this at a younger age. Because the potential here for her to have a semi-normal life? It’s big.”
Mr. Reeves, who said his family has suffered a long series of medical crises over the years, said he’s just hoping to be able to breathe. “I’m hoping this transplant fixes a lot of problems,” he said. “It’s not going to be perfect. But if we could get a success in this house? That would be awesome.”
I am glad there is an option out there for her.