Congenital Hyperinsulinism Center

Rianna's Story

For Rianna Sommers, 17 years of tracking her blood sugars has been like a trek through the mountains: the highest highs and the lowest lows, with plateaus in between. That’s how it is when you have congenital hyperinsulism (HI) and need a near-total pancreatectomy to control the disease.

“Sometimes I stumble,” says Rianna, who sees her glucose levels range from 30 to 400 on a normal day. “But I embrace my disease. I can’t imagine myself without it. It’s an active part of my life.”

Rianna, from Plainview, N.Y., on Long Island, was diagnosed with hyperinsulism at 10 days old and, when traditional drug therapies didn’t control it, had 98 percent of her pancreas removed at The Children’s Hospital of Philadelphia. The surgery made her HI much more manageable, and a controlled diet — supplemented by GI tube feedings — kept her from suffering seizures and other complications that could have affected her development.

Even when she needed to add medications to control her sugar levels — Precose® for highs — the 2 percent of her pancreas that remained kicked out enough natural insulin that she avoided insulin injections until she turned 16.

“I liked having normal HI,” says Rianna. “That’s what I was raised with. Now I’m more like a type 1 diabetic, and I treat myself with shots of insulin. I preferred it when I didn’t have to give myself injections all the time.”

But, as she has done since she was small, she’s taken control of her care as it entered its latest phase. She administers the basal (long acting) shot of insulin each evening and bolus (short acting) shots before each meal. She counts carbs and monitors her glucose. “I’ve been pretty independent since I was 11, when I started e-mailing my doctors directly and making my own appointments,” she says. “My mom always taught me, ‘This is your life, your health. You need to take care of yourself.’”

“I’m a firm believer that kids need to understand the basic fundamentals of their disease,” says Amy Sommers, her mother. “By 3, she was testing her own blood. By 7 or 8, she was setting up the pump for her tube feeds at night. I felt she needed to be as independent as possible.”

That’s the message Rianna shares with younger HI patients through a Yahoo group she started to open a discussion among kids with HI. “We have about 15 members from all over. I provide mentoring and try to answer questions from my own experiences,” she says.

Rianna was also well prepared for her transition to Muhlenberg College this fall. She plans to study pre-medicine and become a pediatric endocrinologist. “It’s not just because I have HI,” she says. “I took an advanced placement honors class in biology and I loved learning all about the endocrine system. What can I say? I’m a nerd.”

Muhlenberg, in Allentown, Pa., is a lot closer to CHOP than Plainview — whether Rianna is coming to Philadelphia for her twice-a-year checkups or if she were to need immediate care. “I love it at CHOP,” she says. “When I tell my friends I’m going to the hospital, they say, ‘That’s too bad.’ I tell them, ‘No, it’s great there. I always have such a good time.’ Since I’ve been coming to CHOP for so many years, I know so many people, and it’s always good to see them again.”
 

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