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WELL, IT'S CALLED IDIOPATHIC PULMONARY FIBROSIS, OR I-P-F.
ITS NAME IS DAUNTING TO MANY, AND I-P-F REMAINS A FAIRLY RARE
CONDITION, AND UNFORTUNATELY, THE CURRENT PROGNOSIS FOR
PATIENTS WITH THIS DISEASE IS THOSE WITH I-P-F WILL NOT
SURVIVE. NEARLY 40 THOUSAND SUCCUMB TO
THIS DISEASE ANNUALLY, BUT THERE IS SOME GOOD NEWS IN ALL OF
THIS, HOWEVER, AND OUR NEXT GUEST, DOCTOR BILL BRADFORD, A
SENIOR FELLOW AT INTERMUNE CORPORATION, IS HERE TO SHED
SOME LIGHT ON WHAT'S BEING ACCOMPLISHED TO ADVANCE RESEARCH
AND UNDERSTANDING OF THIS ILLNESS.
ALSO JOINING US, LATER ON IN THE SHOW, IN OUR UP CLOSE AND
PERSONAL, A FATHER AND DAUGHTER, HERE, TO SHARE THEIR PERSONAL
STORY. BUT FIRST, WE WANT TO BEGIN WITH
DOCTOR BRADFORD. WELCOME TO THE SHOW.
THANK YOU. IT'S A PLEASURE TO BE HERE,
DANIELLE. IT IS A PLEASURE TO HAVE YOU,
HERE. LET'S START WITH THIS.
WHAT IS I-P-F? I-P-F IS A DEVASTATING, AND
ULTIMATELY FATAL, PULMONARY DISEASE THAT'S CAUSED BY
EXCESSIVE LUNG FIBROSIS. UH, IN I-P-F, THE NORMALLY, VERY
ELASTIC LUNG FILLS WITH SCAR TISSUE.
UH, BECOMES VERY STIFF, AND IT'S VERY DIFFICULT TO BREATHE.
SO, FOR THE PATIENT, THIS TRANSLATES INTO, UH, PROGRESSIVE
SHORTNESS OF BREATH, UH AND INCREASING INABILITY TO PERFORM
NORMAL ACTIVITIES OF DAILY LIVING.
THINGS LIKE WALKING. UH, UM, ET CETERA.
THOSE ARE THINGS WE TAKE FOR GRANTED.
JUST BEING ABLE TO DO SIMPLE THINGS, LIKE THAT.
ABSOLUTELY. YOU'RE ABSOLUTELY CORRECT.
(COUGH). AND AS A NAME, IDIOPATHIC
IMPLIES THE CAUSE OF THE DISEASE IS UNKNOWN.
UM, BUT DESPITE THAT LACK OF UNDERSTANDING OF THE CAUSE, WE,
WE DO KNOW THAT I-P-F IS UNIFORMLY A PROGRESSIVE,
IRREVERSIBLE, AND ULTIMATELY, UH, FATAL DISEASE.
AND TODAY, UNFORTUNATELY, THERE IS NO CURE.
WHAT ARE THE CHARACTERISTICS, DOCTOR, THAT MAKE THIS ILLNESS
SO CHALLENGING TO TREAT? WE CANNOT ACCURATELY PREDICT
WHETHER A PATIENT WILL PROGRESS SLOWLY, PROGRESS RAPIDLY, AND
WHEN THE RATE OF PROGRESSION MAY CHANGE.
OVER THE LAST DECADE, WE HAVE MADE DRAMATIC PROGRESS, UH, IN
THIS FRONT WITH A BETTER UNDERSTANDING OF THE DISEASE,
AND TODAY, THERE'S A VERY VIBRANT COMMUNITY THAT'S FOCUSED
ON PROMOTING DISEASE AWARENESS, UH, AND SUPPORTING DRUG
DEVELOPMENT EFFORTS TO, UH, ATTEMPT TO DISCOVER NEW
THERAPIES. WELL, I-P-F HAS BEEN DESCRIBED
AS THE DEADLIEST DISEASE WE'VE NEVER HEARD OF.
WHAT IS BEING DONE, HERE IN THE UNITED STATES, AND ALSO
GLOBALLY, TO RAISE AWARENESS AND FOSTER TREATMENT?
THERE'S A, VERY MUCH, A GROWING AND INCREASINGLY ACTIVE PATIENT
ADVOCACY MOVEMENT IN THE U-S. WE'RE FORTUNATE TO HAVE A COUPLE
OF EXCELLENT PATIENT GROUPS THAT ARE, UH, DRIVING THESE EFFORTS
TO PROMOTE AWARENESS, AND IN EUROPE, WHERE THERE NOW IS A
THERAPY FOR, UH, I-P-F, UH, THERE'S BEEN A GROUNDSWELL OF,
UH, AWARENESS THAT'S FOLLOWED THAT.
IN TERMS OF TREATMENT, THERE'S A SINGLE DRUG, PIRFENIDONE, A
NOVEL ANTI-FIBROTIC DRUG, UH, THAT'S BEEN APPROVED FOR, UH,
I-P-F. IT'S CURRENTLY AVAILABLE IN, UH,
MULTIPLE COUNTRIES ACROSS EUROPE, ASIA, NORTH AMERICA, AND
LATIN AMERICA. UH, PIRFENIDONE IS NOT CURRENTLY
APPROVED IN THE U-S. UH, WE'RE ACTIVELY COLLABORATING
WITH F-D-A, TODAY, ON, UH, PROVIDING ADDITIONAL DATA TO
SUPPORT AN APPROVAL, HERE. I THINK BETWEEN PIRFENIDONE AND,
UH, A SMALL HANDFUL OF OTHER DRUGS, CURRENTLY IN DEVELOPMENT,
UH, FOR I-P-F, UH, WE ARE OPTIMISTIC THAT OVER THE NEXT
FEW YEARS THERE'LL HOPEFULLY BE A THERAPY FOR PATIENTS IN THE
U-S. WELL, THAT WOULD BE WONDERFUL.
AND, YOU KNOW, HERE'S THE OTHER THING.
YOU TALK ABOUT BABY BOOMERS. RIGHT?
HITTING THEIR 50s AND THEIR 60s. UH, IS THE INCIDENCE OF REPORTED
CASES OF I-P-F INCREASING AS THE POPULATION AGES?
YEAH, DANIELLE. THAT'S, UH, THAT'S A GREAT
POINT. SO, I-P-F TYPICALLY OCCURS IN
INDIVIDUALS OVER THE AGE OF 50. SO, AS WE SEE THE AGING OF THE
POPULATION IS SHIFTING DEMOGRAPHICS.
WE CAN CERTAINLY EXPECT TO SEE VERY MEANINGFUL INCREASES IN
BOTH THE INCIDENCE AND THE PREVALENCE OF I-P-F.
WELL, I WANT YOU TO STAY, RIGHT THERE, BECAUSE WE ARE ACTUALLY
GOING TO TALK TO A PATIENT. COMING UP NEXT, AN UP CLOSE AND
PERSONAL. WE'LL MEET AN I-P-F PATIENT, AND
HIS DAUGHTER. AS BEHIND THE MYSTERY: RARE AND
GENETIC DISEASES CONTINUES. RIGHT HERE, ON THE BALANCING
ACT. WE'LL BE RIGHT BACK.
(MUSIC). (COMMERCIAL BREAK).
(MUSIC). WANT TO KNOW WHAT'S HAPPENING IN
YOUR CITY, TODAY, TOMORROW, OR EVEN THIS WEEKEND?
IT'S AS SIMPLE AS CLICKING ON OUR LOCAL EVENTS FINDER ON
thebalancingact.com. JUST ENTER YOUR ZIP CODE, AND
SEARCH FOR ACCESS TO OVER 150,000 HAPPENINGS, INCLUDING
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CLICK AWAY. (MUSIC).
WELCOME BACK, EVERYBODY. THIS MORNING, WE ARE TALKING
WITH DOCTOR BILL BRADFORD, SENIOR FELLOW AT BIOTECHNOLOGY
COMPANY, INTERMUNE, AND MEDICAL EXPERT ON IDIOPATHIC PULMONARY
FIBROSIS, OR I-P-F. WE ARE ALSO PLEASED TO HAVE,
JOINING US, I-P-F PATIENT BOB WILLIAMS, AND HIS LOVELY
DAUGHTER, HEATHER FAVER, BOTH HERE TO SHARE HOW THEY'RE
DEALING AND MANAGING BOB'S I-P-F AS ONLY A CARING FAMILY CAN.
WELCOME TO YOU, BOTH. THANK YOU.
THANK YOU. THANKS FOR BEING HERE, FOR THE
SECOND HALF OF THIS DISCUSSION, DOCTOR, AND I WANT TO BEGIN WITH
YOU, WITH THIS QUESTION. FROM A MEDICAL PERSPECTIVE, HOW
IMPORTANT IS IT THAT FAMILY IS INVOLVED WITH GETTING AN I-P-F
PATIENT THROUGH EVERYTHING THAT THEY HAVE TO GO THROUGH, LIVING
WITH AND MANAGING THIS DISEASE? PATIENTS WITH I-P-F REALLY CAN
RELY HEAVILY ON CAREGIVERS AND LOVED ONES TO HELP THEM MANAGE
THE VARIOUS ASPECTS OF THEIR ILLNESS.
BOB, I WANT TO BRING YOU IN, HERE.
HOW ARE YOU FEELIN'? I'M FEELIN' PRETTY GOOD.
(COUGH). AS DOCTOR BRADFORD SAID,
EARLIER, THE RATE OF PROGRESSION IS DIFFERENT WITH EVERY, UH, IN
EVERY PATIENT. UM, I'M AT THE POINT, RIGHT NOW,
WHERE I CAN STILL DO MOST EVERY, EVERY DAY, NORMAL FUNCTION, AND
DO IT EFFECTIVELY. HOW WERE YOU, I WANT, HOW WERE
YOU DIAGNOSED? AND, AND WHAT WAS, WHAT WAS THAT
LIKE? WELL, THAT WAS VERY, UH,
PROVOKING AND, UH, LIFE CHANGING EVENT.
SURE. I WENT TO MY, UH, DOCTOR, AFTER
A BRIEF BOUT OF PNEUMONIA, AND CURED THE PNEUMONIA, AT LEAST WE
THOUGHT, AND I WENT BACK FOR A FOLLOW UP.
AT THAT TIME, HE STILL HEARD THE CRACKLING NOISES WITHIN MY LUNG,
AND REFERRED MY TO A PULMONOLOGIST.
SUBSEQUENTLY, AFTER A SERIES OF TESTS, INCLUDING AN OPEN LUNG
BIOSPY, IT WAS DETERMINED THAT I HAD PUL--- I-P-F.
HOW OLD WERE YOU? I WAS 62 AT THE TIME.
HOW LONG AGO WAS THAT? APPROXIMATELY 2 YEARS.
AND, HERE YOU ARE. YEP, AND HERE I AM.
I AM SO GLAD, AND I KNOW THAT YOUR DAUGHTER IS SO GLAD, AS
WELL. HEATHER, HOW HAS YOUR
DAD'S ILLNESS, UH, IMPACTED YOUR FAMILY?
WELL, IRONICALLY ENOUGH, IT'S BROUGHT US CLOSER TOGETHER.
WE REALLY BECAME A REAL STRONG UNIT.
BUT, I-P-F IS A HORRIBLE DISEASE, AND IT'S UNIMAGINABLE.
PEOPLE JUST DON'T KNOW ABOUT THE DISEASE.
I JUST WANT TO HELP RAISE AWARENESS, AND LET PEOPLE KNOW
THAT IT'S OUT THERE, AND MY FATHER'S SUFFERING.
YOU KNOW, IT'S FUNNY WHEN YOU ALL WALKED OUT HERE TO SIT DOWN,
AND I LOOKED AT BOB, THE FIRST THING I THOUGHT WAS, SUCH A
SWEET SPIRIT. YOU CAN FEEL---.
UH HUH. ---THAT, RIGHT?
YEAH. EMANATING FROM HIM, AND, AND IT
CERTAINLY, I KNOW THIS HAS TO BE DIFFICULT FOR EVERYBODY.
IT'S VERY DIFFICULT. UH HUH.
YEAH. YEAH.
BUT, THE FACT THAT YOU GUYS ARE TOGETHER, AND YOU'RE A UNIT, AND
YOU'RE WORKING YOUR WAY THROUGH THIS, I THINK IS WONDERFUL.
I WANT TO GET SOME FINAL THOUGHTS FROM YOU, AND I'M GONNA
END WITH YOU, DOCTOR. BUT BOB, I WANT TO ASK YOU.
WHEN YOU, YOU KNOW, YOU SEE YOUR DAUGHTER SITTING HERE, AND SHE
GETS EMOTIONAL WHEN SHE THINKS ABOUT, YOU KNOW, CERTAINLY WHAT
COULD HAPPEN DOWN THE ROAD, HOW IMPORTANT HAS THE FAMILY SUPPORT
BEEN FOR YOU, AND WHAT WOULD YOU LIKE TO SAY WITH REGARDS TO HOW
YOUR FAMILY HAS HELPED YOU? WELL, FIRST OFF, I'D LIKE TO
THANK THEM. UM, MY WIFE, AND MY YOUNGEST
DAUGHTER, AND MY NEXT YOUNGEST DAUGHTER--- I HAVE THREE
DAUGHTERS, FORTUNATELY--- AND, THEY'VE BEEN JUST THERE, ANY
TIME I NEEDED THEM. AS EVERY KNOWS, THERE IS NO
CURE, BUT IN THE MEANTIME, I'LL JUST KEEP FIGHTING THE FIGHT,
AND GO ON FROM THERE. I CAN'T IMAGINE WHAT HE'S GOING
THROUGH. UM, AND I CERTAINLY DON'T WANT
HIM TO WORRY ABOUT US. I WORRY ABOUT HIM, EVERY DAY.
EVERY DAY. DOCTOR, WE'VE GOT JUST ABOUT 30
SECONDS REMAINING. ANY CLOSING COMMENTS THAT YOU
WOULD LIKE TO SHARE? GET THE DIAGNOSIS MADE EARLIER,
UH, AND ACCELERATE THE DEVELOPMENT OF NOVEL THERAPIES
FOR THIS DISEASE. WELL, WE HOPE THAT WE'VE BEEN
ABLE TO SHED MORE LIGHT ON IT, HERE, THIS MORNING, AND THANK
YOU ALL---. IT HAS.
---FOR BEING WITH US, AND SHARING YOUR STORY.
THANK YOU. THANK YOU, DOCTOR, FOR SHARING
THIS GREAT INFORMATION, WITH US. THANK YOU.
AND, IF YOU'D LIKE ADDITIONAL INFORMATION ON I-P-F, VISIT
coalitionforpf.org., ALSO pulmonaryfibrosis.org.
FOR THE LATEST ON MEDICAL ADVANCES AND THERAPEUTIC
APPROACHES, VISIT intermune.com. THAT'S intermune.com, OR SHARE
WITH US ANY PERSONAL STORIES YOU HAVE, RELATED TO TODAY'S TOPIC,
BY VISITING US ON FACEBOOK AT THE BALANCING ACT.
IT'S THE SOCIAL THING TO DO.