In 2008, 2-year-old Kayla Gilligan was feeling under the weather. Her father, Robert, noticed that she was having a hard time breathing. He thought maybe Kayla just had a common cold, so he took her for a walk to get some fresh air.
A couple days passed, and Kayla wasn’t feeling any better. Her breathing wasn’t improving, and Robert noticed that her stomach was enlarged.
Robert and his wife, Robyn, decided to take Kayla to their local community hospital. Unfortunately, the hospital did not have the resources to help Kayla. That’s when Kayla was transferred to MemorialCare Miller Children’s & Women’s Hospital Long Beach to get clarification on what was happening and how she could be treated.
After their visit to Miller Children’s & Women’s, Kayla was diagnosed with glycogen storage disorder type 1a – a carbohydrate metabolism disorder caused by a deficiency of glucose-6-phosphatase, an enzyme in the liver and kidneys. This deficiency affects a person’s body from properly using glycogen. When glycogen isn’t properly used, it is stored in the liver and kidney, which causes them to become enlarged. This can result in low levels of energy and continuous low blood sugar, which can lead to delayed growth and development.
Usually, people with this disorder can’t refrain from food for more than one to four hours without becoming hypoglycemic. Hypoglycemia results when blood sugar levels are too low and can cause chronic hunger, fatigue and seizures.
In Kayla’s case, she could not be in a fasting state for longer than three hours. To help prevent hypoglycemia, Kayla’s registered dietitian recommended that she begin consuming cornstarch by mixing it into a sugar-free drink. Cornstarch is a complex carbohydrate that is difficult for the body to digest. As a result, blood sugar remains at a normal level for longer periods of time.
With the help from Natalie Gallant, M.D., board-certified medical geneticist specialist, Metabolic Center, Miller Children’s & Women’s, and Noelle Emanuel, R.D., Kayla’s journey would become easier. From the time she was diagnosed in 2008, the team has worked directly with Kayla and her family to ensure they are receiving the proper guidance to help manage Kayla’s disorder.
Kayla could not have any sugar because of potential damage to her liver, so it was important that the nutrition team helped Kayla learn how to maintain a healthy diet. She continued to consume cornstarch every two to three hours throughout the day and night. Constantly waking up in the middle of the night became routine for Kayla and her parents. Thankfully, her care team was determined to find another possible solution.
In 2015, Kayla participated in a clinical trial treatment for a super starch product called Glycosade®. The trial included extended periods of fasting using the super starch while being monitored by the nutrition team.
“I worked with Kayla every day during her clinical trial. She responded really well to the medication,” says Emanuel. “She went from waking up every two hours to take cornstarch to sleeping six or seven hours without having to eat.”
Kayla’s trial was successful. The nutrition team decided to make Glycosade® an everyday part of her care plan.
Today, Kayla still works with Noelle and the nutrition team. She keeps a food log to track her diet and nutrition habits and checks in every six months to make sure she continues to take the proper steps to live a healthy life.
“I’m so thankful to Noelle and Dr. Gallant,” says Robert. “Because of their help, I still have my baby girl.”