Wednesday, October 25, 2017

Appalachia has high rates of hereditary pancreatitis; relatively new surgery to treat it is available to children 4 and older

People from Appalachia have some of the highest rates of hereditary pancreatitis in the nation, which often means living a life of uncontrollable pain, but a relatively new treatment may offer some hope.

The pancreas produces enzymes that help break down and digest food, and also insulin that controls blood sugar levels. Inflammation of the pancreas is called pancreatitis.

Pancreatitis can be caused by outside factors, like gallstones or alcohol abuse, but it can also be hereditary, which is common in Appalachia.

"In my first year working at Kentucky Children's Hospital, I saw more cases of hereditary pancreatitis than in 14 years at my previous institution," Dr. George Fuchs, a pediatric gastroenterologist, wrote in an article for UK HealthCare, republished in the Lexington Herald-Leader.

Dr. George Fuchs and Mackenzee Walters, an 11-year-old
with hereditary pancreatitis who underwent an innovative
surgery. (UK HealthCare photo)
One of Fuchs's patients with hereditary pancreatitis is 11-year-old Mackenzee Walters from Kenova, W.Va.

In January, Mackenzee underwent a new surgical procedure at Cincinnati Children's Hospital to treat the disease and it has already changed her life, making it one "free of sharp, unrelenting pain," Fuchs reports. Cincinnati Children's is one of the few children's hospitals in the nation to offer this innovative procedure.

The surgery, known as a total pancreatectomy with islet autotransplantation (TPIAT), "involves removing the entire pancreas, saving the islet cells, which produce insulin, and then re-planting those cells in the liver, where they take up residence and ideally resume their essential function," Fuchs wrote.

"The surgery is a new offering for pediatric patients ages 4 and older expecting a life of pain stemming from the disease," UK HealthCare says in a separate article about Mackenzee and the procedure. It described Mackenzee's life before the surgery as one of pain and hospitalizations that required her to miss many school days. She has had to follow a strict diet since she was 3, consisting mostly of liquids.

The story explains that most of MacKenzee's relatives, including her mother, died early in life from complications related to the hereditary pancreatitis. Kim Walters, Mackenzie's current mother and biological aunt, is the oldest surviving family member with the disease.

"Hereditary pancreatitis is pretty much all we've ever known in my family," Walters said. "It's very excruciating every day. I had my children naturally without an epidural; I would rather have a child every day for the rest of my life than live with this disease."

In December 2015, after seeing many doctors around the country, Walters consulted with Fuchs at Kentucky's Children's Hospital because Mackenzee "was suffering from unmanageable pain and taking a potentially dangerous level of medications without much relief," UK HealthCare reports. Fuchs recommended her for the 12-hour TPIAT surgery and after much consideration, Walters and Mackenzee agreed to it.

“I came to the conclusion, if nothing else if she would just be pain-free,” Walters said. “If God chose to take her six months later, at least she would have six months of no pain. And that was our goal: to make her not have pain and be able to play and do what normal kids do.”

Since the surgery, Mackenzee's islets are producing insulin, and every day the amount of supplemental insulin she needs has dropped. Now she no longer requires pain medication, UK HealthCare reports.

“I would predict she has a very high likelihood of becoming insulin-independent,” Fuchs said. “She will no longer live in the hospital, which is what these children do before the procedure because they are in so much pain. I anticipate she will get on with her life, including getting into mischief as most kids do when they become teenagers. Hopefully go on to have a productive, full life.”

Fuchs said he hopes to extend this treatment to all who qualify for it in Eastern Kentucky and surrounding areas.

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