There’s no doubt that Anthony has fully recovered from his 5% pancreatectomy that cured his focal hyperinsulinism (HI). At 11 months old, he’s already walking “and climbing all over the place,” says his mother, Renata.
A key factor in his successful medical journey at the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia (CHOP) was an 18F-DOPA PET/CT scan, a special radiology procedure that located exactly what part of Anthony’s pancreas was causing his HI.
In addition to being an early walker, Anthony also has the distinction of being CHOP’s 500th subject in our 18F-DOPA PET/CT research study. The HI Center’s founder, Charles Stanley, MD, pioneered the use of a radiotracer molecular imaging technique in 2004, in collaboration with Abass Alavi, MD, of the Division of Nuclear Medicine Imaging and Therapy at the Hospital of the University of Pennsylvania (HUP). The radioactive medication is synthesized at the UPenn cyclotron under a Food and Drug Administration (FDA) Investigational New Drug (IND) protocol. The ground-breaking test serves as a way to distinguish babies with focal HI (which is curable) from those with diffuse HI (which requires a near-total pancreatectomy and ongoing medication to keep under control).
The radioactive agent 18 F-DOPA is injected into the child’s bloodstream and is transported into the pancreatic beta cells that are overproducing insulin. If the scan shows a bright spot, indicating an increased uptake of 18F-DOPA, the child’s HI is thought to be focal.
PET scan creates a roadmap for surgeon
Around 2004, genetic testing advanced to the point where results were back in a short period of time, providing a diagnosis quickly enough to guide surgery decision-making. Now the 18F-DOPA PET/CT scan is used for patients with genetic mutations associated with a focal lesion to pinpoint where the focal lesion — the cluster of overactive beta cells in the pancreas — is located. The images give surgeon N. Scott Adzick, MD, MMM, a “roadmap” to plan the surgery, ensuring only the smallest part possible of the pancreas is removed. Children whose genetics test do not reveal a known HI mutation (also termed as having “negative genetics”) may have a PET/CT scan to rule out a focal lesion.
The 18F-DOPA PET/CT scans are still provided under an FDA IND study, which requires a lengthy consent process for families and intensive record-keeping. Radiologist Lisa States, MD, principal investigator of the study, and clinical research nurse coordinator Susan A. Becker, BSN, RN, CCRC, manage the testing and work closely with families, including Anthony’s, throughout the process. Dr. States is a world-leader in reading the PET-CT scans, which can sometimes be tricky to interpret correctly.
With 500-plus 18F-DOPA PET/CT scans completed, CHOP has now performed over five times more tests than any other hospital in the world. More than 300 children have had their focal HI cured at CHOP.
‘Best-case scenario’
Under the careful direction of Dr. States, Anthony underwent his PET scan just four days after he arrived at CHOP from his local Michigan hospital, where he had been diagnosed with HI.
Dr. States showed Anthony’s dad, David, the scan, which clearly revealed great news: The lesion was in the tip of the tail of the pancreas, which is the least complicated part to remove. It meant 95% of Anthony’s pancreas would remain and continue to function normally. “We had the best-case scenario,” David says.
Anthony’s happy scenario continued. A week post-surgery, he passed his cure fast with flying colors. Then — after refusing a bottle for his entire stay at CHOP, necessitating feeds through a ng-tube — he chugged a bottle on his last day in the hospital.
“They told us we were discharged after he passed the fasting test,” Renata says. “I had been taking classes on how to use the ng-tube for his feeding, so I knew what to do. But I thought I’d try the bottle one last time since I knew he’d be hungry after fasting. And he took it!”
He’s been eating by mouth — and hitting all his other developmental milestones — ever since.
‘An incredible feeling’
While Anthony’s local hospital was quick to suspect HI and kept his blood glucose levels in a safe range during his month there, the doctors weren’t experts in HI. That’s understandable given it is such a rare diagnosis (1 in 25,000 to 50,000 births). Renata quickly found a Facebook group of HI families and saw that many families raved about the care their children had received at CHOP. She and David researched CHOP online and knew that’s where they wanted Anthony to be treated.
Renata called the HI Center, and nurse coordinator Jordan Evans, BSN, RN, helped arrange Anthony’s transfer to Philadelphia via a medical flight.
“It was an incredible feeling to know CHOP had a whole center dedicated to HI, that they didn’t treat it as some random disease, but really, really knew all about it,” she says. “Once we were at CHOP, I felt so comfortable and wasn’t as scared.”
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There’s no doubt that Anthony has fully recovered from his 5% pancreatectomy that cured his focal hyperinsulinism (HI). At 11 months old, he’s already walking “and climbing all over the place,” says his mother, Renata.
A key factor in his successful medical journey at the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia (CHOP) was an 18F-DOPA PET/CT scan, a special radiology procedure that located exactly what part of Anthony’s pancreas was causing his HI.
In addition to being an early walker, Anthony also has the distinction of being CHOP’s 500th subject in our 18F-DOPA PET/CT research study. The HI Center’s founder, Charles Stanley, MD, pioneered the use of a radiotracer molecular imaging technique in 2004, in collaboration with Abass Alavi, MD, of the Division of Nuclear Medicine Imaging and Therapy at the Hospital of the University of Pennsylvania (HUP). The radioactive medication is synthesized at the UPenn cyclotron under a Food and Drug Administration (FDA) Investigational New Drug (IND) protocol. The ground-breaking test serves as a way to distinguish babies with focal HI (which is curable) from those with diffuse HI (which requires a near-total pancreatectomy and ongoing medication to keep under control).
The radioactive agent 18 F-DOPA is injected into the child’s bloodstream and is transported into the pancreatic beta cells that are overproducing insulin. If the scan shows a bright spot, indicating an increased uptake of 18F-DOPA, the child’s HI is thought to be focal.
PET scan creates a roadmap for surgeon
Around 2004, genetic testing advanced to the point where results were back in a short period of time, providing a diagnosis quickly enough to guide surgery decision-making. Now the 18F-DOPA PET/CT scan is used for patients with genetic mutations associated with a focal lesion to pinpoint where the focal lesion — the cluster of overactive beta cells in the pancreas — is located. The images give surgeon N. Scott Adzick, MD, MMM, a “roadmap” to plan the surgery, ensuring only the smallest part possible of the pancreas is removed. Children whose genetics test do not reveal a known HI mutation (also termed as having “negative genetics”) may have a PET/CT scan to rule out a focal lesion.
The 18F-DOPA PET/CT scans are still provided under an FDA IND study, which requires a lengthy consent process for families and intensive record-keeping. Radiologist Lisa States, MD, principal investigator of the study, and clinical research nurse coordinator Susan A. Becker, BSN, RN, CCRC, manage the testing and work closely with families, including Anthony’s, throughout the process. Dr. States is a world-leader in reading the PET-CT scans, which can sometimes be tricky to interpret correctly.
With 500-plus 18F-DOPA PET/CT scans completed, CHOP has now performed over five times more tests than any other hospital in the world. More than 300 children have had their focal HI cured at CHOP.
‘Best-case scenario’
Under the careful direction of Dr. States, Anthony underwent his PET scan just four days after he arrived at CHOP from his local Michigan hospital, where he had been diagnosed with HI.
Dr. States showed Anthony’s dad, David, the scan, which clearly revealed great news: The lesion was in the tip of the tail of the pancreas, which is the least complicated part to remove. It meant 95% of Anthony’s pancreas would remain and continue to function normally. “We had the best-case scenario,” David says.
Anthony’s happy scenario continued. A week post-surgery, he passed his cure fast with flying colors. Then — after refusing a bottle for his entire stay at CHOP, necessitating feeds through a ng-tube — he chugged a bottle on his last day in the hospital.
“They told us we were discharged after he passed the fasting test,” Renata says. “I had been taking classes on how to use the ng-tube for his feeding, so I knew what to do. But I thought I’d try the bottle one last time since I knew he’d be hungry after fasting. And he took it!”
He’s been eating by mouth — and hitting all his other developmental milestones — ever since.
‘An incredible feeling’
While Anthony’s local hospital was quick to suspect HI and kept his blood glucose levels in a safe range during his month there, the doctors weren’t experts in HI. That’s understandable given it is such a rare diagnosis (1 in 25,000 to 50,000 births). Renata quickly found a Facebook group of HI families and saw that many families raved about the care their children had received at CHOP. She and David researched CHOP online and knew that’s where they wanted Anthony to be treated.
Renata called the HI Center, and nurse coordinator Jordan Evans, BSN, RN, helped arrange Anthony’s transfer to Philadelphia via a medical flight.
“It was an incredible feeling to know CHOP had a whole center dedicated to HI, that they didn’t treat it as some random disease, but really, really knew all about it,” she says. “Once we were at CHOP, I felt so comfortable and wasn’t as scared.”
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