>> AT THE CHILDREN'S HOSPITAL OF PHILADELPHIA
HYPERINSULINISM CENTER, WE SEE APPROXIMATELY 2/3 OF
ALL THE CASES THAT ARE BORN IN THIS COUNTRY
WITH HYPERINSULINISM.
THERE ARE TWO TYPES OF HYPERINSULINISM.
THERE IS THE DIFFUSE FORM AND THERE'S THE FOCAL FORM.
>> SO FOCAL FORM REALLY MEANS THAT JUST PART OF
THE PANCREAS IS AFFECTED AND ONCE THAT PART THAT'S
AFFECTED IS TAKEN OUT, IT'S CURABLE.
THE DISEASE IS CURABLE.
FOR DIFFUSE, TYPICALLY THE WHOLE PANCREAS IS AFFECTED.
ALL THE BETA CELLS ARE NOT FUNCTIONING PROPERLY
AND THE BABIES MAY NEED A NEAR TOTAL PANCREATECTOMY.
>> WE HAVE DIFFERENT TREATMENT OPTIONS FOR CHILDREN
WITH HYPERINSULINISM.
ABOUT HALF OF THE CHILDREN ARE TREATED MEDICALLY.
>> THE PATIENTS THAT ARE RELATIVELY EASILY MEDICALLY
MANAGED AND DON'T NEED SURGERY,
WE'LL SEE THOSE CHILDREN PROBABLY TWICE A YEAR.
THEY'LL COME BACK FOR OUTPATIENT FOLLOW- UP IN SIX
MONTHS AND THEN EVERY YEAR WE'LL ADMIT THEM TO EVALUATE
THEM TO MAKE SURE THAT THEIR BLOOD SUGARS ARE AS STABLE
AS WE THINK THEY ARE ON THEIR CURRENT MANAGEMENT.
>> THE OTHER HALF OF THE PATIENTS THEIR HYPERINSULINISM
CANNOT BE TREATED MEDICALLY, AND SURGERY IS THE ONLY
OPTION FOR AMELIORATING THE HYPOGLYCEMIA.
>> THERE ARE A LARGE NUMBER OF BABIES WITH FOCAL DISEASE
THAT CAN BE CURED BY SURGERY.
>> THE BREAKTHROUGH DIAGNOSTICALLY HAS BEEN
THE DEVELOPMENT OF A PET SCAN TECHNIQUE,
WHICH CAN PRECISELY TELL US WHETHER THE BABY HAS DIFFUSE
DISEASE OR FOCAL DISEASE. AND THEN, WITH A GREAT DEAL
OF ACCURACY FOR THOSE BABIES WITH FOCAL LESIONS,
TELL US WHERE IN THE PANCREAS THE FOCAL LESION IS.
>> THE PET SCANNER IS A SPECIAL CAMERA THAT DETECTS
RADIOACTIVITY.
SO WE USE RADIOPHARMACEUTICALS THAT
ARE INJECTED INTO THE BODY TO STUDY DIFFERENT
BODY PARTS.
>> THE SUBSTANCE, 18 FLUORODOPA,
WHICH IS USED FOR THIS PET SCAN,
IS ACTUALLY PREPARED IN THE PENN CYCLOTRON.
>> THE CYCLOTRON FACILITY PREPARES THE 18 FLUORODOPA
JUST THE MORNING OF THIS STUDY.
>> THE FDOPA RADIOPHARMACEUTICAL IS INJECTED
INTO THE VEINOUS SYSTEM AND TRAVELS TO THE PANCREAS.
IT WILL TRAVEL TO THE BETA CELLS AND WHEN THERE'S
A FOCAL LESION, WE WILL SEE A HOT SPOT WHERE THE FOCAL
LESION IS.
THIS HOT SPOT IS DETECTED BY THE CAMERA.
THERE IS MORE RADIOACTIVITY IN THE FOCAL LESION THAN IN
THE REST OF THE PANCREAS.
>> IT'S A VERY INTUITIVE THING.
YOU SEE A BEACON, QUITE BRIGHT,
THAT LOCALIZES A FOCAL LESION.
A SURGICAL APPROACH AT THE BEGINNING IS THE SAME WITH
EITHER FOCAL DISEASE OR DIFFUSE DISEASE.
A LAPAROTOMY IS PERFORMED IN A BABY WHO IS UNDER GENERAL
ANESTHESIA BY ONE OF OUR EXPERT PEDIATRIC
ANESTHESIOLOGISTS.
A SIDEWAYS WOUND IS MADE ABOVE THE LEVEL OF THE BELLY
BUTTON.
AND THEN THE PANCREAS IS EXPOSED.
THE PANCREAS HAS A HEAD, A NECK,
A BODY, AND A TAIL.
FOR BABIES WITH FOCAL DISEASE,
IF THERE IS NOT A VISIBLE OR PALPABLE HINTS TO WHERE
THE FOCAL LESION IS, NOW–WITH PREOPERATIVE PET
SCANNING–I KNOW WHERE TO ZERO IN.
FOCAL LESIONS CAN BE AS SMALL AS TWO OR THREE
MILLIMETERS IN SIZE.
SO SOMETIMES IT CAN BE TRICKY EVEN WITH THE
PREOPERATIVE INFORMATION TO FIND IT.
IF FOCAL LESION IN THE TAIL OR THE BODY OR THE NECK
OF THE PANCREAS, IT'S STRAIGHT FORWARD TO EXCISE AND MAY
INVOLVE TAKING OFF THE END OF THE PANCREAS, ALONG
WITH THE FOCAL LESION, OR JUST THE FOCAL LESION ITSELF,
IF IT'S NOT CLOSE TO THE MAIN PANCREATIC DUCT,
WHICH IS SORT OF THE PIPELINE THROUGH WHICH PANCREATIC
SECRETIONS RUN.
AND WE DON'T WANT TO CAUSE AN INJURY TO THAT DURING
THE OPERATION.
FOCAL LESIONS IN THE HEAD OF THE PANCREAS ARE MORE
COMPLEX BECAUSE THERE'S ALL THIS IMPORTANT TRAFFIC THAT
GOES THROUGH OR AROUND THE PANCREATIC HEAD.
IF YOU TAKE A FOCAL LESION OF THE HEAD OUT,
IT'S IMPORTANT TO SAVE THE BODY AND THE TAIL
OF THE PANCREAS.
BRING UP A LOOP OF BOWEL, A RUEX-EN-Y LOOP,
SO THAT PANCREATIC SECRETIONS CAN DRAIN INTO
THE INTESTINE WHERE THEY BELONG AND SO THAT WE
PRESERVE THE NORMAL ISLET CELLS AND THAT NORMAL
PORTION OF THE PANCREAS.
IF THERE ARE ABNORMAL FINDINGS–CONSISTENT WITH
DIFFUSE DISEASE–THEN A NEAR-TOTAL PANCREATECTOMY
IS DONE, SAVING ONLY A TINY NUB IN THE PANCREAS BETWEEN
THE DUODENUM AND THE COMMON BILE DUCT.
THIS HOPEFULLY WILL HELP IN THE MANAGEMENT
OF THE DISEASE IN TERMS OF PREVENTION OF BRAIN
THREATENING HYPOGLYCEMIA.
BUT IT'S REALLY NOT A CURE.
SOME BABIES DO QUITE WELL AND DON'T NEED MUCH IN
THE WAY OF ADDITIONAL MEDICAL THERAPY.
OTHERS CAN HAVE RECURRENT HYPOGLYCEMIA AND NEED
ADDITIONAL MEDICAL THERAPY.
AND THERE'S A THIRD GROUP THAT DEVELOPS INSULIN
DEPENDENT DIABETES.
>> THE TREATMENT OF A COMPLEX DISEASE LIKE HYPERINSULINISM
REALLY REQUIRES A MULTIDISCIPLINARY TEAM.
>> SEVERAL PHYSICIANS, SEVERAL NURSE PRACTITIONERS,
NURSE COORDINATORS, RADIOLOGIST.
>> SOCIAL WORKERS, SURGEONS, PATHOLOGISTS.
>> NEONATOLOGISTS.
>> AND RESEARCH SUPPORT.
>> THAT'S A LOT OF PEOPLE.
>> YOU REALLY NEED THAT COMPREHENSIVE GROUP
OF EXPERTS TO PROVIDE A HIGH DEGREE OF CARE THAT THESE
CHILDREN REQUIRE.
BECAUSE OUR PATIENTS COME FROM OVERSEAS AND FROM ALL
OVER THE UNITED STATES, WE WORK VERY HARD AT
ESTABLISHING COLLABORATION WITH THE LOCAL PHYSICIANS
THAT ARE GOING TO BE FOLLOWING THESE FAMILIES
LONG TERM.
>> IT'S REALLY IMPORTANT FOR FAMILIES TO FEEL THAT THEY
HAVE A TEAM THAT THEY KNOW, A TEAM THAT THEY'VE BEEN
ABLE TO BUILD RAPPORT WITH, A TEAM THAT WILL LISTEN
TO THEM.
IT REALLY IS A COLLABORATION BETWEEN THE REFERRING
HOSPITAL, THE FAMILY– ONCE THEY GET TO US–
AND THEN OUR TEAM AS A WHOLE.