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>> IF PATIENTS DON'T RESPOND TO MEDICAL THERAPY THEN WE HAVE
OTHER DIAGNOSTIC TECHNIQUES THAT WE USE.
>> CLINICALLY IF YOU HAVE A BABY WITH DIFFUSE DISEASE NEXT
TO A BABY WITH FOCAL DISEASE THEY LOOK EXACTLY ALIKE,
CLINICALLY.
SO IT WAS OUR TASK TO TRY TO DIFFERENTIATE THE TWO,
BECAUSE OBVIOUSLY TREATMENT OF THE TWO GROUPS IS COMPLETELY
DIFFERENT.
>> OUR DAUGHTER, CAROLINE, WAS BORN ON JUNE 13,
AT CENTRAL BAPTIST HOSPITAL IN LEXINGTON, KENTUCKY.
SHE WAS PRONOUNCED HEALTHY AND WE WERE DISCHARGED FROM
THE HOSPITAL AND GETTING READY TO GO HOME WHEN A VERY ASTUTE
NURSE NOTICED AND THOUGHT THAT CAROLINE SEEMED COLD
AND A LITTLE BIT LETHARGIC.
>> WE WERE WORRIED ABOUT THE USUAL THINGS THAT YOU
WORRY ABOUT.
YOU KNOW YOU WANT THEM TO BE HEALTHY AND YOU WANT THEM TO
HAVE TEN FINGERS AND TEN TOES, BUT WE HAD NEVER HEARD ABOUT
HYPERINSULINISM.
>> WHAT'S REALLY BEEN EXCITING IN THE LAST FIVE YEARS IS THE
REALIZATION THAT THERE ARE A LARGE NUMBER OF BABIES WITH
FOCAL DISEASE THAT CAN BE CURED BY SURGERY AND SO WE HAVE A MUCH
MORE DIRECTED APPROACH TO 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.
>> WE KNEW THAT WE HAD HYPERINSULINISM,
BUT WE DIDN'T KNOW WHAT TYPE OF HYPERINSULINISM WE HAD
AND THE PET SCAN WAS SOMETHING THAT THEY ARE WORKING WITH HERE
TO TRY AND HELP IDENTIFY WHETHER YOU'RE DEALING WITH A FOCAL ISSUE,
OR IF IT WAS DIFFUSE.
>> THE WAY THE PET SCAN WORKS IS BASED ON RADIOPHARMACEUTICALS.
THAT MEANS DRUGS THAT ARE LABELED
WITH RADIOACTIVE MATERIAL.
WE SEND THESE DRUGS INTO THE BODY AND ASK THEM,
"TELL US WHAT'S HAPPENING IN THE BRAIN.
TELL US WHAT'S HAPPENING IN THE HEART.
TELL US WHAT'S HAPPENING IN THE PANCREAS."
>> YOU INJECT RADIOACTIVELY LABELED DOPA AND COLLECT IMAGES
OF THE PANCREAS AND IN THE FOCAL LESION IT'S ACCUMULATED WITHIN
THE CELL AND AS IT RELEASES RADIOACTIVITY THAT'S THEN
DETECTED TO MAKE AN IMAGE OF THE PANCREAS THAT SHOWS WHERE
THE FOCAL LESION IS.
>> A FOCAL LESION ON THE PET SCAN LIGHTS UP LIKE
A LIGHT BULB.
>> IT'S A VERY INTUITIVE THING.
YOU SEE A BEACON, QUITE BRIGHT, THAT LOCALIZES A FOCAL LESION.
>> SO IT TELLS THE SURGEON EXACTLY WHERE TO GO TO LOOK
FOR THE FOCAL LESION AND IT ALSO ALLOWS US TO VERY ACCURATELY
DISCRIMINATE BETWEEN BABIES THAT HAVE DIFFUSE DISEASE AND THOSE
THAT HAVE FOCAL DISEASE.
>> THE SUBSTANCE 18-FLUORODOPA IS USED FOR THIS PET SCAN,
IS ACTUALLY PREPARED IN THE PET CYCLOTRON.
>> THIS IS NOT A DRUG THAT YOU CAN PUT ON A SHELF
AND USE IT WHENEVER YOU WANT.
THIS IS THE CASE WITH PENICILLIN, ASPIRIN.
BUT IN OUR CASE THESE ARE RADIOACTIVE DRUGS
AND HAVE A FINITE HALF-LIFE.
THEN IN A SHORT PERIOD OF TIME THEY WILL DISAPPEAR.
>> WE CURRENTLY HAVE TO DO THE PET SCANS UNDER A FOOD
AND DRUG ADMINISTRATION IMD PROTOCOL.
SO IT'S AVAILABLE HERE AT CHILDREN'S HOSPITAL
OF PHILADELPHIA, BUT IT'S NOT WIDELY AVAILABLE ACROSS
THE U.S.
>> PLANNING AND SCHEDULING YOUR PET SCAN REQUIRES
A MULTI-DISCIPLINARY TEAM APPROACH BETWEEN THE CYCLOTRON,
WHO MUST MAKE THE 18-FLUORODOPA, ON THE DAY OF THE STUDY
AND THE PET CENTER.
>> WE HAVE TO DETERMINE THE DOSE THAT WE HAVE TO GIVE TO
THE CHILD BASED ON THE CHILD'S BODY WEIGHT AND BY THEN
WE HAVE POSITIONED THE CHILD IN THE MACHINE AREA.
THE ANESTHESIOLOGIST HAS INDUCED ANESTHESIA AND THE DOSE
IS READY TO BE INJECTED.
>> SO WHAT HE WANTS TO KNOW IS WHERE DOES THIS FALL IN THE CT?
>> COMBINING PET WITH CAT SCAN ALLOWS US TO SHOW THE DISEASE
SUPERIMPOSED ON THE STRUCTURE FOR THE SURGEON TO FIND
EXACTLY WHERE HE SHOULD GO.
SO HAVING, OF COURSE, FUNCTION, WHICH IS WHAT WE DO WITH PET
AND THE STRUCTURE, WHICH IS WHAT THE CAT SCAN SHOWS GIVES
HIM A ROAD MAP AS TO WHERE THINGS ARE.