The Children's Hospital of Philadelphia
www.chop.edu
Unraveling the Mysteries of Hyperinsulinism - Treatment: Medical Management

Charles A. Stanley, MD: It's important when we're considering a baby with hyperinsulinism and whether we should do surgery to go through the differential causes of hypoglycemia and to differentiate babies that have genetic hyperinsulinism and require surgery from those that have temporary causes. We've come up with some criteria for distinguishing babies that have transient hyperinsulinism. They have histories of birth stress or birth asphyxia or they're low birth weight. We also are able to use the genetic testing to help make that distinction. Most of those babies can be controlled on diazoxide therapy and for that reason don't need to be considered for surgery.

Laura Ann Warner, NP: Medical management of hyperinsulinism is very specific and detailed, so for children who are responsive to diazoxide therapy, they must take the oral solution twice a day and monitor their blood sugars at least two to four times per day. Children who are unresponsive to diazoxide then are started on subcutaneous treatments of octreotide, have the same intense regimen, which consists of subcutaneous injections of octreotide every six or eight hours, as well as very frequent blood glucose monitoring.

Candy Sanchez, Parent: He's six years old with hyperinsulinism and he's been on the same treatment for years. Our endocrinologist didn't know where to go next and how to treat Dio. She referred us here to the endocrinology team.

Leroy Martinez, Parent: They took down a couple of shots and eliminated a couple of shots.

Candy Sanchez, Parent: He was getting it four times a day, every six hours, meaning I was getting up and setting an alarm to get up. Kind of inconvenient for both me and Dio, but they changed that to twice a day and only daily doses of octreotide.

Laura Ann Warner, NP: We provide education to the parents. We provide support to the parents.

Candy Sanchez, Parent: It gets better as they get older.

Lori Prinz Halaby, NP: Typically as these children get older their regimens tend to be less involved and their fasting tolerance improves over time.

Leroy Martinez, Parent: I just want him to be a normal little boy. That's my greatest joy right there. You know just that I can see him actually out there playing with everybody and enjoying himself, enjoying life.