Quality of life – Biliary Atresia Education Day – The Children’s Hospital of Philadelphia (3 of 8)

Quality of life – Biliary Atresia Education Day – The Children’s Hospital of Philadelphia (3 of 8)


>>HE WORKED ALONGSIDE THE
JAPANESE DR. OHI WHO LOOKED AT MANY OF THE JAPANESE PATIENTS. AND IT’S A SMALL STUDY WHERE
THERE’S 21 PATIENTS IN THE UK AND THERE WERE 25 IN JAPAN. AND IN THE UK THERE’S ACTUALLY
NO IMPAIRMENT IN ANY CATEGORY. IF YOU LOOK DOWN– BODY PAIN,
GENERAL HEALTH, MENTAL STATUS, PHYSICAL FUNCTION,
ROLE OF EMOTIONAL HEALTH, ROLE OF PHYSICAL HEALTH, ALL
THE WAY DOWN TO THE BOTTOM– AND YOU LOOK AT THE P VALUES,
AT LEAST IN THIS SMALL GROUP, IT LOOKED LIKE QUALITY
OF LIFE WAS FINE. IN JAPAN–AND IT SHOWS YOU THAT
THERE’S DIFFERENCES FROM COUNTRY TO COUNTRY, THAT THEIR GROUP
OF SURVIVORS–I WASN’T VERY GOOD AT DRAWING THIS CIRCLE–BUT THEIR
GROUP OF SURVIVORS THERE SHOULD BE A NEGATIVE .01 THERE–
HAD, IN GENERAL, FELT THEIR HEALTH WAS POOR. AND THEN DOWN HERE THERE’S
THREE MORE SIGNIFICANT FIGURES. THEY FELT EMOTIONAL, PHYSICAL,
AND SOCIAL FUNCTIONING WERE ALL IMPAIRED. SO YOU WALK AWAY FROM THAT,
AND IT’S A LITTLE CONFUSING. QUALITY OF LIFE IS GOOD. MAYBE IT’S NOT. MAYBE IT’S A SMALL STUDY. MAYBE IT’S THE WRONG FORM. A NUMBER OF PEOPLE IN THIS ROOM
POSSIBLY HAVE TAKEN PART IN THIS. THIS IS PART OF THIS NATIONAL STUDY,
AND YOU FILLED OUT A FORM CALLED THE PEDS QL. IT’S A SHORT FORM THAT ONLY HAS
20-SOMETHING QUESTIONS, AND WE’VE LOOKED AT 158 PATIENTS
AROUND THE COUNTRY AND SO FOR THIS GROUP LOOKING AT HOW YOUR
HEALTH COMPARES IN A U.S. POPULATION IS PROBABLY THE MOST
IMPORTANT THING TO THINK ABOUT. SO THE PATIENTS FILL OUT
A FORM, IF THEY CAN WRITE, AND THE PARENTS FILL OUT
A FORM IF THEY’RE UNDER 18. SO THE 2 TO 4 YEAR OLDS ONLY
THE PARENTS FILL OUT A FORM, AND THE OVER 18 YEAR OLDS ONLY
THE PERSON WITH BILIARY ATRESIA FILLS OUT A FORM. ALONG WITH THAT WE ALSO DO
PHYSICAL EXAMS, LAB STUDIES, AND HISTORY
OF THE MEDICAL COMPLICATIONS AT THE SAME TIME THEY COME IN. AND SO THIS IS A PAPER THAT
WE’RE CURRENTLY WORKING ON WRITING AND, IN THIS OF THE 158,
THERE’S A FAIR NUMBER OF PEOPLE IN EACH OF THESE CATEGORIES. TWENTY-THREE IN
THE 2 TO 4 YEAR OLDS. 43, 43, 37, AND WE NATIONALLY
DON’T HAVE THAT MANY OVER 18. THERE’S 12. ABOUT HALF ARE FEMALE. THE GROUP HAS NORMAL WEIGHTS. SO YOU CAN ALSO INFER FROM
THAT THAT ALL THESE LONG-TERM SURVIVORS ARE GROWING
PRETTY WELL. THEY HAVE–THIS IS TRICEPS
SKIN FOLD, WHICH IS A MEASURE OF ENERGY
STORES WHERE PEOPLE DO PINCHES HERE AND SEE WHETHER OR
NOT YOU PUT ON ENOUGH FAT. AND THAT TOO IS IN
THE NORMAL RANGE. THIS HAS TO DO WITH
COMPLICATIONS. AND THERE IS STILL CHOLANGITIS
AND OCCASIONALLY A BLEED THAT HAPPENS. AND THIS IS RECORDING HOW MANY
CHILDREN HAD ONE EPISODE WITHIN THE PREVIOUS 12 MONTHS. IT’S MORE COMMON TO HAVE IT WHEN
YOU’RE YOUNG THAN WHEN YOU’RE OLD AND HAVING ONE EPISODE DOES
NOT MEAN THAT YOU’RE GOING TO HAVE MULTIPLE EPISODES. HEALTHY KIDS GET CHOLANGITIS. GI BLEEDS ARE MUCH RARER. SO THAT YOU CAN SEE THAT THE
TOTAL IS 11 OUT OF 158 PATIENTS. SO ABOUT LESS THAN 10% WILL HAVE
A GI BLEED AFTER THE AGE OF 2. WE LOOKED AT THEIR LAB STUDIES
AND, FOR THE MOST PART, THESE KIDS ARE STILL CLEARING
THEIR JAUNDICE OR ARE WAITING FOR A LIVER TRANSPLANT. AND THE OLDEST GROUP HAS
A LITTLE BIT OF JAUNDICE. THE REST ARE NORMAL,
NOT JAUNDICE AT THOSE AGES AND THEN YOU CAN SEE THAT
YOU STILL HAVE A LITTLE BIT OF– ALT IS LIVER INFLAMMATION. ALBUMIN IS NORMAL IN TERMS
OF PROTEIN STORES GOING ALL THE WAY DOWN. ALL OF THESE NUMBERS HEMOGLOBIN,
PT OR INR ARE NORMAL. WHITE COUNT IS, IN GENERAL,
BASICALLY NORMAL AS WELL AS PLATELET COUNT. SO THAT’S WHAT THE GROUP
OF PATIENTS LOOK LIKE. AND WE GAVE THEM THE PEDS QL. THESE ARE JUST SAMPLES OF
THE WAY IN WHICH WE ASSESS QUALITY OF LIFE. SO FOR PHYSICAL QUESTIONS
THE QUESTIONS ARE: I FEEL PAIN. IT’S HARD FOR ME TO READ. IT IS HARD FOR ME TO RUN. IT IS HARD FOR ME
TO PLAY SPORTS. I HAVE LOW ENERGY. AND WHEN THE CHILD FILLS THAT OUT,
THEY RATE IT FROM 0 TO 4. THEY NEVER FEEL THAT,
OR THEY ALWAYS FEEL THAT. AND EMOTIONAL QUESTIONS
HAVE TO DO WITH: I FEEL SAD.
I FEEL SCARED. I HAVE TROUBLE SLEEPING. SOCIAL QUESTIONS ARE: I HAVE TROUBLE GETTING
ALONG WITH OTHER KIDS. KIDS TEASE ME. AND THERE’S A FEW OTHER
QUESTIONS. AND FOR SCHOOL: IT’S HARD TO PAY ATTENTION
IN CLASS. I FORGET THINGS. IT’S HARD TO GET ALL
MY HOMEWORK DONE. THIS IS THE DATA. AND PHYSICALLY, THIS 158
PATIENTS ARE PHYSICALLY, COMPARED TO OTHER GROUPS,
ARE PHYSICALLY NORMAL. SO THAT FOR THAT GROUP OF KIDS
WHO DON’T GET TRANSPLANTED, THEY MOVE ONTO THIS 2 TO
25-YEAR-OLD AGE GROUP WITHOUT PHYSICAL DIFFICULTIES
FOR THE MOST PART. AND YET, FOR REASONS WE DON’T
UNDERSTAND, AND I’LL TRY TO TEASE IT OUT, THESE SCORES,
EMOTIONAL, SOCIAL, SCHOOL IN PARTICULAR,
AND PSYCHOSOCIAL IS REALLY A COMBINATION OF THESE THREE
AND TOTAL IS COMBINATION OF ALL FOUR OF THESE–THESE OTHER ASPECTS
ARE THE THINGS WHERE KIDS SEEM TO HAVE SOME ISSUES,
AND WE DON’T KNOW WHY. THE PARENTS HAD THE SAME
PERCEPTION AS THE KIDS. IF YOU MATCHED THE PARENTS
IN EACH OF THESE DIFFERENT AGE GROUPS 2 TO 4 YEAR OLDS
AND GOING ALL THE WAY UP, THE PARENT AND THE CHILD
PERCEIVED THEIR HEALTH THE SAME WAY. SO THAT WAS SORT OF INTERESTING
SO THAT PARENTS ARE GETTING IT RIGHT FOR WHATEVER REASON. AND SO THEY WERE MATCHING
THAT THE SCHOOL FUNCTIONING WAS THE AREA WHERE THERE
WERE THE MOST DIFFICULTIES, AND PHYSICAL WAS
THE LEAST DIFFICULT. SO TO TRY TO TEASE THIS OUT,
WE TO DO SOMETHING CALLED “MULTIVARIATE ANALYSIS,” AND WE
USE ALL THAT OTHER DATA THAT WE HAD: THEIR HEIGHT, THEIR WEIGHT,
THEIR LAB VALUES, THEIR CHOLANGITIS,
THEIR ASCITES, EVERYTHING THAT WE
COULD THINK ABOUT, AND TRY TO FIGURE OUT WHY
ARE THERE IMPAIRMENTS IN CERTAIN AREAS? AND WHAT WE FOUND IS FOR
THE CHILDREN SCORED LOWER IF HEMOGLOBIN WAS LOW, IF THE
SKIN FOLD FOR THE TRICEPS SKIN FOLD WAS LOW, OR IF ASCITES
WAS PRESENT. IF ASCITES IS PRESENT, IT’S EASY
FOR ME TO UNDERSTAND THAT THAT’S GOING TO MAKE LIFE DIFFICULT. THESE OTHER TWO ARE
REALLY A LITTLE SUBTLE, AND I WAS SURPRISED. AND PROBABLY WHAT IT SHOWS IS
THAT THOSE KIDS WHO ARE MOST TIRED, WHO HAVE A LOW
HEMOGLOBIN, WHO POSSIBLY HAVE LESS ENERGY
STORES ARE THE ONES WHO ARE HAVING MORE SCHOOL STRUGGLES. AND WE HAVE TO LOOK INTO THIS
MORE AND SEE WHETHER OR NOT THAT’S REALLY TRUE. AND THE PARENTS, I THOUGHT
THAT THIS WAS FUNNY. EVEN THOSE THEY GOT IT RIGHT,
THEY GET IT RIGHT FOR THE WRONG REASONS. THE PARENTS SCORED THEIR CHILD
LOWER IF THEY HAD THINGS THAT THEY COULD SEE WERE ABNORMAL. SO THEY SCORED THEIR CHILDREN
LOWER IF THEY LOOKED JAUNDICED. THAT WAS BY FAR AND AWAY
THE BIGGEST PREDICTOR OF WHETHER OR NOT THEY RANKED THEIR CHILD
AS FUNCTIONING MORE POORLY, AND YET THE CHILD DIDN’T SEE IT
THAT WAY BECAUSE THEY DON’T LOOK AT THEMSELVES. THERE WAS NO SIGNIFICANT
CHANGE IN SCORES ACROSS DIFFERENT AGE GROUPS. IT DIDN’T MATTER WHETHER
OR NOT YOU WERE IN FIRST GRADE OR IN YOUR FIRST YEAR OF COLLEGE
WHETHER OR NOT YOU SCORED DIFFERENTLY ON THAT TEST. WE THEN COMPARED IT TO DIFFERENT
TYPES OF CHRONIC ILLNESS TO SEE WHETHER OR NOT YOU COULD
GET A BETTER UNDERSTANDING OF WHAT THIS IS. AND IT’S A LOT DATA HERE, BUT
THE BARS I’D LIKE TO YOU FOCUS ON IS THE FIRST GREEN ONE
IS BILIARY ATRESIA AND THE RED AND WHITE DOTTED ONE ARE
NORMAL, HEALTHY CONTROLS. SO THAT PHYSICALLY–
IS THIS NOT LINED UP EXACTLY? PHYSICALLY CHILDREN WERE FINE
COMPARED TO THE HEALTHY CONTROLS AND REALLY SIGNIFICANTLY BETTER
THAN SOMEBODY WHO HAS CANCER. THEIR PSYCHOSOCIAL, FUNCTIONING
WHICH IS THAT COMPOSITE GROUP, IS FOR SOME REASON LOWER,
AND IT MAY BE ENERGY LEVEL, AS I HINTED AT. SAME THING. SO WHERE THERE’S A STAR UP HERE,
THOSE ARE WHERE THERE’S SIGNIFICANT DIFFERENCE. SO IN THE PSYCHOSOCIAL,
EMOTIONAL, SOCIAL, THERE WERE DIFFERENCES. AND SCHOOL WAS THE BIGGEST
AREA OF CONCERN. SO THAT PAPER IS IN PROGRESS,
AND DATA IS BEING ANALYZED. AND THE REASON WHY WE DO THIS
IS TO TRY TO GIVE OURSELVES NEW IDEAS OF THINGS THAT
WE SHOULD FOCUS ON. RATHER THAN FOCUSING EXCLUSIVELY
ON THE YOUNGER KIDS AND PREVENTING CHOLANGITIS AND DOING
THESE MEDICAL THINGS IS TO TRY TO LOOK INTO, “WHAT ARE SOME OF
THESE SOFTER FINDINGS THAT MIGHT HELP US FOCUS OUR MEDICAL
ATTENTION LONG TERM?” SO, IN SUMMARY, I WANT TO SAY
THAT GROWING UP WITH BILIARY ATRESIA CAN BE OKAY. IT’S A PRETTY CORNY PICTURE,
ESPECIALLY FOR THE OLDER KIDS. OVERALL, CHILDREN AND YOUNG
ADULTS WITHOUT LIVER TRANSPLANTATION CAN
DO EXTREMELY WELL. THEY’RE EMPLOYED, BUT WE’D
LIKE THEM TO LIVE LIFE TO THEIR FULLEST. WE EXPECT MARRIAGES FOR THEM. WE EXPECT CHILDREN AND JOBS
AND SOMEHOW USING THIS QUALITY OF LIFE TOOL WE MAY BE
ABLE TO TEASE OUT, “WHAT ARE THE MEDICAL THINGS THAT
MIGHT HOLD THEM BACK?” AND THAT’S IT FOR THE BEGINNING.

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