Alicia Journey with Congenital Hyperinsulinism

Congenital hyperinsulinism (excessive insulin production) is the most common cause of persistent or recurring hypoglycemia (low blood sugar) in infancy. Many infants and children affected with any of the various forms of hyperinsulinism have recurring, severe episodes of hypoglycemia both before and after diagnosis that can cause seizures, brain damage, and developmental delay. Since undiagnosed hyperinsulinism or failure of treatment for hyperinsulinism significantly increases the risk of mental retardation and permanent brain damage, prompt diagnosis and aggressive treatment are crucial in the fight against this disease.

Because hyperinsulinism is a rare condition, only occurring in approximately 1/25,000 to 1/50,000 births, few facilities have the equipment and specialists necessary to diagnose and offer the best treatment for this disease. Currently, the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia is the only center in the United States that performs the PET scan test in infants and children to help identify focal or diffuse hyperinsulinism.

If a child requires surgery for hyperinsulinism, parents can expect to stay at the hospital for at least one month. Children with hyperinsulinism require extra care at home in the form of blood sugar monitoring, maintaining regular feeding schedule, administering medications and glucagons. These prolonged hospitalizations and intense medical regimens can be stressful for the parents and families of children with hyperinsulinism.

A patient named Ty inspired Congenital HI Kids. Ty, like other infants born with congenital hyperinsulinism, was born into the fight of his life. He and his family face many challenges in his fight. Congenital HI Kids was formed to help Ty and other children like him in their fight against this disease. ”

"My daughter Alicia, was born five years ago in upstate Ny at 36 weeks. They checked her blood sugar and it was 32 and the only reason they checked it was because she was born early so we are thankful for that. She spent two weeks at the local NICU and then was transfered to a bigger hospital in NY, at this time they tried two different medications,octreotide and diazoxide which were not effective in maintaining her blood sugars. They then did a 95% pancreatectomy and tried to wean her off the dextrose drip but were still unsuccessful. Then they went back in and did a 98% pancreatectomy which fortunately was successful. Although this was a very trying and stressful experience we consider ourselves very lucky that our daughter's HI was caught so early and that she does not have any development issues due to low blood sugars. After a short stint on insulin after surgery, she has not needed any since. She did have a g-tube for over a year that we used to feed her when she wouldn't eat, such as when she was sick and through the night. Now, her blood sugars remain 80-150s, although we are concerned that she may need insulin in the future, we continue to check blood sugars. She also takes pancreatic enzymes with her meals. She is doing so well and she just started kindergarten this year! My son Adam was born this year and we had the hospital implement the newborn protocol and when they checked his blood sugar it was in the 40s! They checked it again and it was still in the 40s so he was transfered to the NICU and was put on a dextrose drip which they were able to successfully wean him off after he passed his fasting study with flying colors. He was able to come home in a week. He is now almost 7 weeks old and is doing great. We live in Idaho and I am a Registered Nurse and my Husband also works at the hospital. We enjoy spending time together as a family and enjoying the outdoors