>> WE HAVE DIFFERENT
TREATMENT OPTIONS FOR CHILDREN WITH
HYPERINSULINISM. ABOUT HALF OF THESE CHILDREN
ARE TREATED MEDICALLY WITH A COMBINATION OF DRUGS THAT
SUPPRESS INSULIN SECRETION AND OTHER
MEDICAL MANAGEMENT. FOR THESE CHILDREN THAT
RESPOND TO MEDICAL THERAPY WE HAVE ONLY TWO
MEDICATIONS THAT ARE USED. THE FIRST LINE OF
THERAPY IS DIAZOXIDE, A MEDICATION THAT IS USED IN
AN ORAL PREPARATION IS VERY EFFECTIVE IN SUPPRESSING
INSULIN SECRETION IN MILDER FORMS OF HYPERINSULINISM. SOME REQUIRE TREATMENT WITH
A SECOND MEDICATION THAT IS CALLED OCTREOTIDE. OCTREOTIDE IS AN INJECTABLE
DRUG THAT AGAIN SUPPRESSES INSULIN SECRETION. >> THERE ARE SEVERAL TOOLS
THAT WE HAVE TO MEDICALLY MANAGE PATIENTS. SOME ARE ORAL MEDICATION,
INJECTABLE MEDICATION, AND THE THIRD IS SUGAR WATER
THROUGH A FEEDING TUBE. >> THEY CAN'T JUST
DRINK FORMULA OR JUICE OR SOMETHING ONCE EVERY TWO
HOURS TO KEEP THEIR BLOOD SUGARS UP. THEY NEED A CONTINUOUS RATE
OF SUGAR TO PREVENT THEM FROM HAVING
SEVERE HYPOGLYCEMIA.
>> WE WORK TOGETHER WITH
THE FAMILIES TO MAKE THE DECISIONS THAT WOULD ALLOW
US TO ESTABLISH THE BEST MEDICAL MANAGEMENT
FOR THEIR CHILDREN. >> 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 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. >> AND TYPICALLY THOSE KIDS
WE ANTICIPATE WILL IMPROVE OVER TIME. BUT SOMETIMES THEY MAY
NEED LIFE-LONG THERAPY FOR MEDICATION. >> HE IS 6 YEARS OLD
WITH HYPERINSULINISM. AND HE'S BEEN ON THE
SAME TREATMENT FOR YEARS. ENDOCRINOLOGISTS DIDN'T KNOW
WHERE TO GO NEXT AND HOW TO TREAT DALE AND SHE REFERRED
US HERE TO THE ENDOCRINOLOGY TEAM HERE. >> THEY TOOK DOWN A COUPLE
SHOTS AND ELIMINATED A COUPLE SHOTS, BUT I JUST
WANT HIM TO BE JUST A NORMAL LITTLE BOY.
THAT'S MY GREATEST
JOY RIGHT THERE. YOU KNOW, JUST THAT I CAN
SEE HIM ACTUALLY OUT THERE PLAYING WITH EVERYBODY
AND ENJOYING HIMSELF. ENJOYING LIFE. >> IN HALF OF OUR PATIENTS
THE HYPERINSULINISM CAN BE TREATED MEDICALLY. AND
THOSE CHILDREN WILL REQUIRE MEDICAL THERAPY FOR MOST
OF THEIR LIVES, BUT THAT CONTROLS THE HYPOGLYCEMIA. IN THE OTHER HALF OF THE
PATIENTS, THE HYPERINSULINISM CANNOT BE TREATED MEDICALLY.
AND SURGERY IS THE ONLY OPTION FOR AMELIORATING
THE HYPOGLYCEMIA..
