The Children's Hospital of Philadelphia
www.chop.edu
Unraveling the Mysteries of Hyperinsulinism - The Importance of a Comprehensive Center

Laura Ann Warner, NP: There are many children who have subtotal pancreatectomies, without the knowledge whether their disease is focal or diffuse.

N. Scott Adzick, MD: The worst case scenario is to do a near total pancreatectomy on a child, leave a portion of the head of the pancreas and have a focal lesion prove to be within that residual pancreas.

Lori Prinz Halaby, NP: We had an infant who was born and was diagnosed with hyperinsulinism at their local institution and underwent a pancreatectomy to remove half of her pancreas.

Amy Tran, Parent: Our first step was an e-mail and Laura Warner replied to me the day of the surgery. I told her the situation that my daughter's going to go into the O.R. in about half an hour and she was like, "No. No, don't let that happen. We can help you over here and there's different tests that we can do that can help your daughter." And so I put Laura on the phone with my baby's surgeon who was going to operate on her in like a half an hour and they talked and we canceled the operation.

Lori Prinz Halaby, NP: The insurance company wouldn't approve for her to come to our institution.

Amy Tran, Parent: The PET scan was considered experimental, so the state denied us. So we went with the first surgery. It didn't go well. A week later we went with a second surgery and that didn't go well either.

Lori Prinz Halaby, NP: Unfortunately she continued to have severe hypoglycemia so the insurance company finally agreed to have her transported to our center and in our investigation she was found to have a focal lesion in the head of her pancreas. So if she had just been approved to come here in the first place she would have been cured and would not be a diabetic as she is now.

Amy Tran, Parent: For our case if we were here earlier I think that my daughter would have had a much much better life.

Charles A. Stanley, MD: We've tried to establish systems to help consult with referring physicians.

Lori Prinz Halaby, NP: Our goal is to be a resource for them regarding patients with hyperinsulinism.

Charles A. Stanley, MD: To help them in deciding how to manage and diagnose their patients at home and how to decide when babies might need the special expertise that we can provide here.

N. Scott Adzick, MD: We have an enormous clinical experience now, well over 100 cases during the last five to six years, by far the biggest experience in the world.

Lori Prinz Halaby, NP: We have the benefits of having the expertise and skill of radiologists and nuclear radiologists to be able to provide the PET scan procedure to help us differentiate between focal and diffuse disease.

Charles A. Stanley, MD: Expertise from genetics to help identify genetic mutations.

Laura Ann Warner, NP: Our endocrinologist, our pathologist, our surgeons, our surgical nurse practitioners.

Charles A. Stanley, MD: It's very difficult to do this in centers that don't have a lot of experience in hyperinsulinism, because then they only see one or two patients per year.

Laura Ann Warner, NP: We anticipate that from the incidence of hyperinsulinism that we are catching about 25% of the surgical patients in the United States.

Charles A. Stanley, MD: We know that in years past when we didn't have the tools that we have now that surgery probably missed 50% or more of the kids with focal disease.

N. Scott Adzick, MD: Now we can craft highly specific surgical therapy to potentially cure children with focal disease.