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TODAY, THERE ARE WELL OVER 7 THOUSAND RARE AND GENETIC
DISEASES OBSERVED, AND THE ODDS ARE, ONE OUT OF TEN PEOPLE ARE
DIAGNOSED WITH ONE. IT COULD BE YOU, OR SOMEONE YOU
KNOW. BUT, WHERE THERE'S UNCERTAINTY,
THERE'S ALSO HOPE. GLOBAL AWARENESS AND RESEARCH
EFFORTS KEEP PUSHING MEDICAL BOUNDARIES, AIDING IN NEW
DISCOVERIES, AND IN THE DEVELOPMENT OF TOOLS THAT CAN
HELP EASE THE QUALITY OF LIFE FOR PATIENTS AND CAREGIVERS.
HERE TO SHARE SOME INSIGHT ON SOME ACCESSIBLE AT HOME, AND
ACUTE CARE SOLUTIONS, FOR MANY SUFFERING FROM CONDITIONS LIKE
CYSTIC FIBROSIS, AND NEUROMUSCULAR DISEASE, IS DOCTOR
CHRISTOPHER LANDON, DIRECTOR OF PEDIATRICS AT VENTURA COUNTY
MEDICAL CENTER, AN ADVOCATE OF HILLROM, A LEADING PROVIDER OF
MEDICAL TECHNOLOGIES. WELCOME.
WE ARE SO EXCITED TO HAVE YOU, HERE.
WELL JULIE, IT'S SO, IT'S SO GREAT TO BE HERE.
WELL, I JUST WANT TO ASK YOU, IN BASIC TERMS, WHAT IS A RARE AND
GENETIC DISEASE? WELL, THERE'S SOME 200 THOUSAND
PEOPLE IN THE UNITED STATES, SO THIS IS A RELATIVELY RARE
CONDITION. SOME OF THEM, LIKE CYSTIC
FIBROSIS, THERE ARE ONLY 30 THOUSAND PATIENTS IN THE UNITED
STATES WITH THIS DISEASE. HMMMMM.
SO, ABOUT 80% OF THESE DISEASES DO HAVE A GENETIC ORIGIN.
WE'RE LOOKING, NOW, UH, AT TESTING EVERY BABY, AT BIRTH,
FOR, UH, WHAT THEIR GENETIC POTENTIAL IS.
NOW, WHAT ARE SOME OF THE MOST COMMON PHYSICAL CHALLENGES
ASSOCIATED WITH THE, THESE DISEASES?
WELL, THE HARD PART, UH, YOU KNOW, AS A PARENT, UH, YOU KNOW
WHAT IT'S LIKE. IT'S HARD ENOUGH GETTING YOUR
DAUGHTERS DRESSED IN THE MORNING, NOW TRY ADDING IN, UH,
FEEDINGS THROUGH A TUBE, AND INTO THE STOMACH, DIRECTLY.
UH, LITERALLY BEATING THE MUCUS OUT OF THEM, AS IF THEY'RE
UNABLE TO COUGH, OR THEY'RE DROWNING IN THEIR OWN, OWN
SECRETIONS, HAVE VITAMINS, TIME IN THE HOSPITAL, TIME SPENT AWAY
FROM THEIR FAMILY, TIME IN INTENSIVE CARE UNITS, LIFE AND
DEATH, IT'S, IT'S UNIMAGINABLE. A GOOD FRIEND OF MINE, BOOMER
ESIASON, I WAS A SPORTSCASTER, I WATCHED HIM GO THROUGH THIS WITH
HIS SON, WHERE HE HAD TO PUT HIM ON THE BOARD, AND BEAT THE MUCUS
OUT. HOW DIFFICULT IS THIS FOR
FAMILIES? WELL, IT'S REALLY HARD, AND SOME
OF THESE FAMILIES HAVE TWO AND THREE CHILDREN, FOR EXAMPLE,
WITH CYSTIC FIBROSIS. I HAVE A FAMILY, NOW, IT'S, UH,
TWO YOUNG MEN, UH, WITH NEUROMUSCULAR DISEASE.
THEY'RE ON VENTILATORS AT HOME, THEY ESSENTIALLY HAVE AN
INTENSIVE CARE UNIT IN THEIR HOUSE, AND THE MOTHER'S A
WORKING TEACHER, AND SOMEHOW, SHE BALANCES ALL OF THIS IN HER
LIFE, AND THESE, THESE BOYS HAVE A LIFE.
THEY'RE WATCHING FOX NEWS, AND UH, THEY'RE AGAINST OBAMACARE,
THOUGH THERE'S A LOT OF MONEY BEING SPENT ON THEIR CARE AT
HOME. (LAUGHTER).
SO, BUT IT'S SOMETHING WE INSIST,---.
THERE'S SOMETHING,---. ---SOCIETY.
---YEAH. YOU KNOW, WE, WE LEARN FROM
THEM. WE DO.
WE LEARN FROM THESE FAMILIES. WE DO.
FROM THE SACRIFICE. UH, THEY, THEY GIVE US
UNDERSTANDING ABOUT WHAT OUR OWN LIVES ARE, ARE ALL ABOUT.
WELL, WHEN WE COME BACK, WE'RE GONNA TAKE A CLOSER LOOK AT SOME
OF THESE RARE AND GENETIC DISEASES, AND THE TECHNOLOGY
OFFERING NEW HOPE, YES, AND, A GREATER QUALITY OF LIFE TO
PATIENTS AND CAREGIVERS. SO, DON'T GO AWAY.
WE'LL BE RIGHT BACK. WELL, HI, EVERYBODY.
WELCOME BACK. I'M HERE WITH DOCTOR CHRISTOPHER
LANDON, DIRECTOR OF PEDIATRICS AT THE VENTURA COUNTY MEDICAL
CENTER, AN ADVOCATE OF HILLROM, A LEADING PROVIDER OF MEDICAL
TECHNOLOGIES. WE'RE TALKING ABOUT RARE AND
GENETIC DISEASES, AND I JUST WANT TO GET RIGHT TO THIS, THE
SERIOUSNESS OF THESE, AND HOW IT EFFECTS FAMILIES.
WELL, FOR EXAMPLE, ONE IS CYSTIC FIBROSIS.
UH HUH. ONE IN TWENTY PEOPLE IS A
CARRIER OF THE GENE. THEY MEET THAT OTHER ONE IN
TWENTY, AND ABOUT ONE IN, UH, FOUR OF THEIR CHILDREN WILL HAVE
CYSTIC FIBROSIS. THIS IS UH, A PROGRESSIVE
DISEASE, THICKENING THE MUCUS IN THE CHEST, THEY, UH, DIE A
PULMONARY DEATH. THEY DROWN, LITERALLY, IN THEIR
OWN MUSCUS. AND, UH, SOME 25 YEARS OR SO
AGO, HILLROM, UH, AH, NOW OWNS THE VEST SYSTEM, UH, THEY CAME
UP WITH A WAY TO GET THE MUCUS OUT WITH HIGH FREQUENCY CHEST
WALL OSCILLATION, AND IT REALLY ALLOWED THE PARENT, FINALLY, TO
HAVE A LITTLE FREEDOM, AND FOR THE CHILD TO HAVE SOME CONTROL
OF THEIR, OVER THEIR OWN LIVES, AND TO SPEND LESS TIME IN THE
HOSPITAL, LESS TIME OUT OF THE HOME.
UH, RIGHT NOW WE'RE SCREENING FOR IT, UH, AT BIRTH, AND IN
MOST OF THE STATES, UH, SO THAT AT BIRTH WE CAN NOW IDENTIFY
CHILDREN WITH CYSTIC FIBROSIS, AND GET ALL OF THESE THERAPIES
GOING EARLIER. SO, WHAT MEDICAL TECHNOLOGY IS
THERE, AVAILABLE, FOR PATIENTS WHO ARE IN NEED OF REGULAR
AIRWAY CLEARANCE THERAPY? WELL, THERE'S THE VEST SYSTEM,
AS WE'VE SPOKEN ABOUT. IN A HOSPITAL, THEY CAN BRING
THEIR VEST SYSTEM INTO THE HOSPITAL, AND OFTEN TIMES, THE
HOSPITALS THEMSELVES HAVE THEM. SOME 10 THOUSAND PATIENTS A DAY
ARE USING THEM, BUT IN ADDITION, THEY'VE DEVELOPED THE METANEB
SYSTEM. THIS USES PULSES OF AIR, UH, TO
HELP MOBILIZE THE SECRETIONS, GETTING THOSE THICK SECRETIONS
ON UP AND OUT. IT ALSO ALLOWS THEM TO
AEROSOLIZE MEDICATION, AS WELL. GET IT DEEPER INTO THE LUNG SO
THAT IT NOW CAN BE PART OF THEIR LIVE, TWO AND THREE TIMES A DAY,
THEY CAN HELP. A, UH, YOU BRUSH YOUR TEETH.
THAT'S GREAT. AND, THEY NEED TO BRUSH THEIR
LUNGS. THEY NEED TO GET THAT MUCUS OUT.
THAT INFECTED MUCUS OUT. WELL, WHAT WE WERE SAYING IS
THAT BEFORE THEY WERE SO DEPENDENT ON THEIR PARENTS, AND
AT A CERTAIN AGE, A CHILD WANTS SOME FREEDOM.
THEY, THEY DON'T WANT TO BE, YOU KNOW, THIS VEST REALLY GIVES
THEM THAT FREEDOM. WELL, IT GIVES THEM THAT
FREEDOM, AND, AND NOW, WHEN I STARTED IN THIS, UH, KIDS LIVED
TO AGE TWO WITH CYSTIC FIBROSIS,---.
WOW. ---AND THEN, TWELVE, AND THEN,
19, AND NOW THEY HAVE LIVES. THEY'RE NOT, THEY'RE NOT, UH,
SOMEONE WITH CYSTIC FIBROSIS, THEY'RE A PERSON, AND THEN THEY
HAVE A DISEASE THAT'S CALLED CYSTIC FIBROSIS, BUT THEY HAVE A
LIFE TO LIVE. ARE THERE OTHER MOBILE
ALTERNATIVES OUT THERE? WELL, UH, THERE'S SOME THINGS
THAT REQUIRE, UH, COORDINATION. SO, FOR OUR CHILDREN WITH
NEUROMUSCULAR DISEASE, ANOTHER OTHER PROBLEM,---.
RIGHT. ---UM, FOR THEM, THEY CAN'T
COORDINATE THESE THINGS. THEY'RE CHILDREN WITH SEVERE
CEREBRAL PALSY, AND UH, OTHER, OTHER INHERITED GENETIC DISEASES
WHERE THEY CAN'T MANAGE A COUGH, AND SO, THEY'RE (COUGH) (COUGH).
ALL THE TIME? THEY CAN FEEL THAT MUCUS IN
THEIR CHEST. AND SO, AGAIN, IT ALLOWS THEM
SOME FREEDOM, AND, AND MY GIRLS ARE NOW YOUNG WOMEN, AND GETTING
MARRIED. MY, MY BOYS ARE NOW YOUNG MEN,
AND GETTING MARRIED. AND, THEY HAVE AN INDEPENDENCE
THEN, FROM, FROM NOT HAVING TO DEPEND ON THEIR HUSBAND OR THEIR
WIFE FOR THAT. AND, ARE HILLROM'S THE VEST
AIRWAY CLEARANCE SYSTEM, ARE THEY AVAILABLE TO ANYONE?
YES. THERE'S OVER 24 THOUSAND
PHYSICIANS WHO PRESCRIBE IT, OVER 124 THOUSAND PATIENTS WITH
IT IN THEIR HOME, AND EVERY DAY, IN THE HOSPITAL, OVER 10
THOUSAND PATIENTS ARE USING IT. THIS IS, YOU KNOW WHAT?
SHAKE MY HAND. YOU'VE CHANGED---.
(LAUGHTER). ---THE WAY CAN TREAT THIS
DISEASE. THANK YOU, SO MUCH, FOR JOINING
US, TODAY. IT'S JUST SUCH A PLEASURE TO
KNOW THAT THIS KIND OF WORK IS ONGOING, AND I JUST WANT TO SAY,
THE BALANCING ACT, WE STAND FOR THE CURE.
JOIN US, WEEKLY, AS WE CONTINUE TO LOOK BEHIND THE MYSTERY OF
RARE AND GENETIC DISEASES. THERE IS HOPE, AND IT STARTS
WITH AWARENESS.