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.
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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.
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.
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.