>> WHEN FAMILIES FIRST ARRIVE AT CHOP, THEY ARE TRYING
TO FIGURE OUT WHAT WE HAVE AND HOW IT RELATES
TO WHAT THEY EXPECT.
>> THEIR CHILDREN HAVE RARE DISEASES.
THESE DISEASES ARE HARD TO TREAT, HARD TO DIAGNOSE,
HARD TO UNDERSTAND.
>> THE FAMILIES HAVE AN EXTRAORDINARY JOURNEY.
MOST WHO END UP COMING FOR EVALUATION HAVE ALREADY HAD
A BUSY LIFE AND A-- PROBABLY AN INCREDIBLE
JOURNEY JUST TO GET THERE.
>> THEY COME WITH A LOT OF ANXIETY, UNCERTAINTY,
AS TO WHAT THE FUTURE HOLDS.
THERE'S FEAR OF THE UNKNOWN.
>> THE LOGISTICS OF BRINGING CHILDREN HERE TO CHOP
WITH SPECIAL NEEDS AND THORACIC INSUFFICIENCY SYNDROME
ARE DAUNTING.
>> IT TAKES A VERY SOPHISTICATED ORGANIZED GROUP OF PEOPLE.
>> WE HAVE A NURSING PRACTITIONER HERE WHO HELPS
IN COORDINATION AND ALSO ANOTHER INDIVIDUAL
IN COMPLEX SCHEDULING THAT HELPS COORDINATE
ALL THE APPOINTMENTS.
>> THESE FAMILIES, AT TIMES, CAN BE QUITE STRESSED
AND THE DEMAND ON THEM IS GREAT.
AND SOMETIMES YOU NEED TO TAKE A STEP BACK AND SAY,
"ALL RIGHT, THIS IS WHAT WE CAN DO TODAY.
THIS IS WHAT WE CAN DO TOMORROW.
BUT WE NEED TO GET IT ALL DONE."
>> WHAT WE DON'T WANT TO HAPPEN IS HAVE MULTIPLE VISITS
SCHEDULED AT MULTIPLE TIMES AND INCURRING MORE TRAVEL TIME
AND EXPENSE FOR THE PARENTS.
SO IF WE DO OUR ALMIGHTY BEST TO GET EVERYBODY IN WITHIN THAT ONE
OR TWO DAYS TO HAVE THEM SEE EVERYONE THEY NEED TO SEE.
>> SO THEY COME, AND WE'LL BEGIN WITH A HISTORY AND PHYSICAL
EXAMINATION, AND WE'LL TAKE IN ALL THE INFORMATION.
AND THERE'S USUALLY A LOT OF TEACHING AND EXPLAINING
THAT GOES ON DURING THAT EARLY FIRST AND SECOND VISIT.
>> HOWEVER, THERE'S SEVERAL BITS OF INFORMATION THAT ARE CRUCIAL
IN TRYING TO MAKE DECISIONS ABOUT MOVING FORWARD.
THAT INCLUDES IMAGING.
SO THERE'S STANDARD IMAGING, JUST PLAIN X-RAYS THAT GIVE US
AN IDEA ABOUT THE BONY ANATOMY.
>> BUT THERE'S NOW MANY OTHER SOPHISTICATED IMAGING TECHNIQUES
FROM DYNAMIC MRI TO LOW-RADIATION CAT SCANS.
>> WE CAN TAKE THAT NOW AND USE THREE DIMENSIONS TO REALLY PUT IT
ALL TOGETHER WITH A COMPUTER ALGORITHM SO WE CAN SEE
AND LITERALLY SPIN THE SPINE AROUND AND SPIN THE CHEST
AROUND AND MEASURE ITS VOLUME AND GET A BETTER SENSE FOR WHERE
THE RIBS ARE IN RELATION TO THE SPINE IN ALL THREE DIMENSIONS.
>> BECAUSE I WANT TO KNOW IF THE RIBS ARE DENTED IN BECAUSE I MAY
HAVE TO GO IN AT SURGERY AND PULL THEM OUT.
I WON'T SEE THAT ON X-RAY.
I WANT TO KNOW THAT THE RIBS ARE TWISTED INTO AN ANGLE
BECAUSE I WILL HAVE TO REVERSE THAT.
>> AND MOST RECENTLY, MRI HAS ADVANCED SUBSTANTIALLY.
>> CHOP AND MANY OF THE BRILLIANT RADIOLOGISTS WE'RE
FORTUNATE TO HAVE WORKING WITH US HAVE NOW PIONEERED THIS
DYNAMIC MRI TECHNOLOGY THAT YOU CAN SHOW THESE MOVIE CLIPS
OF THE CHILD BREATHING.
>> YOU LITERALLY TAKE A MRI IN 1/3 OF A SECOND.
>> AND THEN WHAT'S DONE IS THE RADIOLOGISTS ARE ABLE TO TAKE
THE IMAGES AND PASTE THEM TOGETHER IN A SEQUENCE
SO THAT YOU CAN SEE HOW THE LUNGS, THE CHEST WALL, THE DIAPHRAGM,
AND THE ENTIRE RESPIRATORY SYSTEM MOVES THROUGH
AN ENTIRE CYCLE OF BREATHING.
>> THIS IS A WHOLE NEW LEVEL OF UNDERSTANDING OF LUNG DISEASE
AND SPINE AND CHEST WALL DEFORMITY THAT NO ONE HAS EVER
REALLY LOOKED INTO.
THE SUPPORT OF THE PULMONARY DIVISION IS CRITICAL FOR THIS.
>> TO TRY AND ASSESS WHAT LIMITATIONS EACH CHILD HAS
THEN BEYOND THAT WHAT THEIR PROGRESSION HAS BEEN.
ARE THEY IN A STATIC POSITION WHERE THEY HAVEN'T HAD ANY
CHANGE IN THEIR CAPACITY, OR ARE THEY CONTINUALLY
PROGRESSING AND HAVING MORE DIFFICULTIES
WITH EACH PASSING YEAR?
>> THEY ALSO ARE STEWARDS OF EFFECTIVENESS OF TREATMENT
BECAUSE OF THEIR EXPERTISE IN INFANT PULMONARY FUNCTION
TESTING, WHICH IS NOT AVAILABLE TO MANY HOSPITALS THROUGHOUT
THE U.S.
>> IT GIVES US THE ABILITY TO LOOK AT DYNAMICS
OF RESPIRATION--HOW MUCH AIR A PATIENT CAN CONCEIVABLY BREATHE
IN WHEN THEY ARE ABLE TO BREATHE IN AS DEEPLY AS POSSIBLE
AND THEN THE SIZE OF THE CHEST CAVITY ITSELF AND HOW MUCH
THE LUNGS CAN EXPAND WITHIN IT.
>> SO THEY ARE A KEY PART OF THE TEAM, AND THEIR EXPERTISE
ENABLES US TO MAKE THE SURGERY SAFER AND MAKE
THE APPROACH MORE INTELLIGENT.