This content highlights the significant progress in cystic fibrosis (CF) care, enabling individuals to live longer and age beyond what was previously imaginable, and emphasizes the evolving challenges and opportunities in managing aging with CF through a multidisciplinary, holistic approach.
Mind Map
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Welcome. Hello to those joining us
online and to everyone here in Seattle.
I'm honored to welcome you to the second
plenary session of the 39th annual North
American Cystic Fibrosis Conference.
In just a moment, I will introduce our
distinguished plenary speakers who will
deepen our knowledge and understanding
of how we can improve the lives of
people with cystic fibrosis. Before we
move to do that, I'd like to acknowledge
some truly extraordinary individuals in
our care center network.
I will start with the Bonnie Ramsay and
Richard C. Talamo Distinguished Clinical
Achievement Award presented to those
whose careers have profoundly altered
the course of CF care and research. It's
a privilege to stand before you today to
recognize a true pioneer in the field of
pediatric medicine and CF research,
Dr. Frell's journey began with a moment
of compassion and clarity during his
third year of medical school when he
encountered an eight-year-old girl
hospitalized with CF.
He got to know her well and
unfortunately watched her die. On that
day, Phil committed to doing what he
could to prevent such deaths in other children.
children.
Over the past four decades, Dr. Frell
has transformed that commitment into
groundbreaking action. His path was
cemented when Dr. Harry Schwackman
pulled him aside at a conference in 1975,
1975,
commented that newborn screening was the
only hope for early diagnosis and said,
"Phil, as a neonatlogist and CF center
director, you should be the one to
advance the field. And aren't we lucky
that he did? His leadership in the
Wisconsin cystic fibrosis neonatal
screening project initiated in 1984
demonstrated unequivocally
that early diagnosis through newborn
screening paired with aggressive
nutritional intervention could prevent
malnutrition and significantly improve
outcomes for children with CF.
Thanks to Dr. Frell's vision and dogged persistence,
persistence,
every US state and the District of
Columbia now screens newborns for CF.
And as many of you know, his persistence
knows no boundaries so that children
with CF all around the world benefit.
His efforts have not only changed
clinical practice, but have also saved
and improved countless lives.
As a tireless advocate for quality
improvement, he organized the National
Quality Improvement Consortium. He
partnered with the CDC to develop a CFTR
mutation repository and helped establish
best practices for CF newborn screening.
Since 2007, he has served as a cystic
fibrosis national facilitator for CF
newborn screening and quality
improvement, ensuring that the promise
of early diagnosis is matched by
excellence in care delivery.
On a personal note, Phil, I want to
express my deep gratitude for the
partnership we've shared over the years.
When I joined the CF foundation nine
years ago, you graciously welcomed me
into the fold and supported me as I
deepened my understanding of CF newborn
screening. Together, along with many
others in the audience, our partnership
has helped advance health equity in CF
newborn screening. And for that, I'm
sincerely grateful.
I'm also grateful to your family and in
particular your wife, Alice, for sharing
you with us. Alice, Phil asked me to
express his gratitude to you for the
support, patience, and love you've given
It won't come as a surprise that Phil is
accepting the award on behalf of the
The team has worked together for five
decades to develop improved methods of
early diagnosis.
Phil, today we celebrate not only your
scientific achievements, but also your
unwavering dedication to the CF
community. Your legacy is one of
compassion, innovation, and relentless
pursuit of a better future for children
with CF.
Dr. Frell, on behalf of the Cystic
Fibrosis Foundation, your colleagues,
and the countless families whose lives
you've touched, it is my great pleasure
to present you with the Ramsay Talamo
[Music] [Applause]
[Applause]
Now I will hand the microphone to my
colleague Paula Lis who will present the
next awards. [Music]
[Music]
Hello. The Cystic Fibrosis Foundation's
Mary M. KTOS Care Champion Award is
presented to two members of the
multidisciplinary care team who have
made significant contributions in the
field of specialized CF care.
This award honors the memory of Mary's
passion for excellence and commitment to
improving the lives of people with CF
and families through exceptional care
and advocacy.
Our first recipient worked alongside
Mary Ktos for many years. She is
recognized for her leadership, tireless
work ethic, clinical acumen, and her
heart of gold. Her center director notes
that her commitment to people with CF in
clinic, in the hospital, and in the
moments of life in between is unmatched,
making her a lifeline for adults with CF.
CF.
One adult said, "There were times when I
was struggling and things didn't look so great,
great,
but having her there assured me we would
get to get get it through things and it
kept me positive and always looking forward.
forward.
A longstanding and active member of her
cent's mental health team,
she led efforts to improve access to
mental health resources for people with CF.
CF.
Nationally, she has enhanced the role of
the advanced practice provider in CF
care delivery.
I am proud to announce that the first
recipient of this year's Mary M. Kantos
Award is Dr. Dr. Lynn Fes, certified
physic physician assistant, doctor of
physical therapy, and the adult cystic
fibrosis program coordinator at the
cystic fibrosis center of Western New York.
York.
All right. Our second recipient is an
accomplished pharmacist,
author, and educator with exceptional
teaching abilities, mentoring the next
generation of pharmacists in CF care.
She's a pioneer who is establishing a
pharmacist patient centered model of
care at two CF programs. Her dedication
to improving processes and the patient
experience has resulted in the
implementation of modulator therapies,
the development of medication monitoring
tools, and greater efficiency in
planning for and monitoring exacerbations.
exacerbations.
A parent said she was calm in the middle
of what felt like a really big storm. I
am thankful for her expertise,
communication skills, enthusiasm, and
commitment to helping my daughter make
it through the worst time of her life.
I'm pleased to announce that the second
Mary Kos award goes to Hannah Fawn,
clinical professor and clinical
pharmacist specialist at University of Michigan.
Lynn and Hannah, all of us, thank you
for being champions of care for our
community. All right, do it again.
Please join me in welcoming them to the
stage to receive the award. [Music]
[Music] [Applause]
[Applause]
That's great. The Carolyn and C. Richard
Mattingley Leadership in Mental Health
Care Award is given by the Cystic
Fibrosis Foundation each year in
recognition of leadership and commitment
to the mental health and well-being of
individuals with CF.
This award is a tribute to the
Mattingleys and their inspiring devotion
to people with CF through the promotion
of emotional wellness throughout the CF community.
community.
This year's recipient is a leader in the
CF community, both locally and nationally.
nationally.
Her patients wrote of her kindness and
compassion as she helps them navigate
their emotional journey through
adulthood with CF, helping them to
develop strategies for coping with
stress and anxiety.
A teammate notes, "Her passion for the
mental well-being of people with CF is
exemplified by her daily interactions
with her patients and her CF team.
And her commitment to advancing care is
seen by serving on multiple committees.
On a national level, she's brought
mental health into the conversation by
serving on the CF education committee,
the post transplant and CRMS CF spid
guidelines committees, and she is
currently a member of the CF mental
health advisory committee, and you can
thank her for helping co-author many of
those mental health resources that we
have produced.
I am excited to announce that this
year's recipient of the Carolyn and C.
Richard Maddingley Leadership in Mental
Health Care Award is Dr. Lily
Christristen. Lily is a licensed
psychologist and associate professor of
psychiatry and behavioral sciences at
the Medical University of South Carolina.
Carolina.
Please join me in welcoming Lily to this
stage to receive her welldeserved award. [Music]
[Music]
A huge congratulations and thank you to
our award winners. We're just so
fortunate to have such a dedicated group
of CF care team members. To everyone
nominated, thank you for the incredible
work you do every day.
Before we move to the plenary, I'm
deeply saddened to share the passing of
one of the Cystic Fibrosis Foundation's
founding parents and a transformative
leader, Doris Tullson.
Doris turned her love for her daughter
Annie into a lifelong mission that
dramatically improved the lives of
people with CF in part by galvanizing
support for essential CF research and
Now, it's a privilege to introduce this
plenary session, a breath of fresh age,
redefining growing older with CF.
Just a few decades ago, the idea of
aging with CF was frankly unimaginable.
But today, today we are witnessing a
profound shift in outcomes thanks to
datadriven care delivered holistically
by multidisciplinary care teams equipped
with transformative therapies and
supported by the relentless advocacy and
resilience of the CF community.
This session is not just about
celebrating longevity. It's about
redefining what it means to live well
with CF across the lifespan for all. As
more individuals with CF reach far
beyond adulthood, we face a new
question. How do we support aging bodies
in what was historically considered a
childhood disease?
Our speakers today, Drs. Ahmed Uler and
Karen von Bberg bring deep expertise to
these questions. They'll explore the
realities of aging as well as
opportunities to innovate as people with
CF grow older.
Spoiler alert, it isn't all about
innovation. It's about each of us, care
team member and individual with CF,
putting into practice the knowledge and
data we already have in our possession.
Most importantly, our speakers will help
us reframe aging not as a limitation,
but an opportunity, a new frontier in CF
care, one focused on thriving and not
just surviving.
It's a distinct honor to introduce Dr.
Ahmed Uler, director of the adult cystic
fibrosis program and Bridg's adult
transition program at the Bigham and
Women's Hospital and Boston's Children's
Hospital CF center. He also serves as
assistant professor at Harvard Medical
School. Dr. Uler earned his
undergraduate degree from the University
of Michigan, his medical degree from
Kansas City University of Medicine and
Biosciences, and completed his residency
in internal medicine and pediatrics at
the Cleveland Clinic, where he also
served as chief.
He went on to complete a fellowship in
pulmonary medicine at Boston Children's
Hospital and has been working as an
adult pulmonologist at Bighamin Women's
Hospital since 2006.
A nationally recognized leader in adult
CF care, Dr. Uler has dedicated his
career to advancing transitional care
models and improving outcomes for
individuals aging with CF.
He's joined today by Dr. Karen von Berg,
a distinguished physical therapist at
the John's Hopkins Cystic Fibrosis
Center, where she provides care to both
children and adults with CF. Dr. Von
Berg earned her Bachelor of Science in
Physical Therapy from Northeastern
University and her doctorate in physical
therapy from Udica College. She serves
as the co-f facilitator of the CF
Foundation CF physical therapist
mentoring program and is a partnership
enhancement program facilitator.
With over two decades of experience in
CF care, she has been a leading voice in
advancing the discipline of physical
therapy in improving respiratory health,
mobility, and overall quality of life
for individuals with CF. Today, she
brings her deep clinical insight to the
evolving conversation on aging with CF.
Amit will kick us off. Please join me in
welcoming him to the podium to help us
redefine what it means to grow older
[Applause] [Music]
[Applause]
like dim the lights.
This is happening. I am humbled to be
here today.
And so on behalf of Karen and I, we are
grateful to Al Faroh for his kind
introductions and to the amazing Whitney
Brown and Quinn Tran and Mike Bole who
was my first mentor when I came out of
the gates as adult CF program director.
And I also want to recognize my amazing
wife Deb and my boys who have only shown
me kindness and patience. And I also
wanted to just say something about my
dad who was an, you know, old school
doctor who I lost earlier this year.
And I want to especially say thank you
to Olivia, Jess, and Jose and the NACSC
plenary planning team for this
opportunity to speak with you, knowing
just how many amazing people out there
they could have asked. And as I was
talking to people this week, I was
thinking about people I would have asked
to do this. And at the end of this, you
might say they should have asked, but
I hope not.
This plenary presentation on redefining
growing older with CF, a subject I'm
passionate about, is made possible
through the dedication of those of us
who got us here, the commitment of you
in the in the audience and listening
online, but especially the resilience of
the cystic fibrosis community. The fact
that we are here to continue to speak
about aging with CF reflects the decades
of progress in science, clinical care,
and advocacy. I mean, all of it. And I
want to express my gratitude to the
individuals and families who entrust us
with their care and to also take a
moment to acknowledge those who are no
longer with us
but whose legacy shape our work today.
And it's in their honor and the future
of everyone with CF today that we gather
here to address the opportunities and
the challenges of growing older with
cystic fibrosis.
So our holistically interconnected
systems and made up of the CF community,
researchers, clinical teams underscore
how we all depend on each other and to
realize the goal set forth in the 1950s
by those families who are getting
together to begin that path to finding a
cure. And the great side effect of all
that innovative and expert care for that
our pediatric colleagues out here in the
audience made possible has resulted in
the largest number of adults with CF and
counting. People with CF are functioning
and breathing better. And this welcome
side effect isn't only for those on modulators.
modulators.
And though we recognize this progress
isn't celebrated in every corner of the
world, yet that will come. Conversations
about properly caring for adults,
starting with the first adult program
back in the 80s that I just learned
about paved the way for all these other
milestones you see behind me and leading
to all the you leading to the plenary
delivered by the amazing Liz Tullis back
in 2022 and now us.
So let's take a closer look at the
numbers and the changing face of CF. In
just over 20 years, we can see the
growing number of people from age 40 to
50 as depicted by the bottom two bars
and how a group of people between ages
30 and 40 went from the fourth largest
cohort to the second.
In response, the CFF registry now
reports out additional data important to
adults like blood pressure and lipid
and showcases our growing number of
people over the age of 50 since 2023.
The CF foundation registry is a living
document that tells us a story and one
such story includes women with CF who
historically have had shorter lifespans
than men. And but that gap of between
the sexes is closing. And though we
still need to better understand the
reasons for it, there are now more women
than men over the age of 60.
Now, aging science has gone mainstream
with the media almost daily shedding
light on what helps us live longer and
healthier and often for a hefty fee. And
maybe they're just targeting me because
they think I'm getting old and I'm
getting a lot of those and I'm interested.
interested.
But there is only one single message to
take away from all of that noise. Just
one. Exercise.
Exercise and movement is still the
single most important answer to the
question, how do we slow down aging?
And almost as important, there's
personalized nutrition and medicines and
restorative sleep, stress resilience.
These all shape how we age.
And for those with a history of chronic
illness, this field offers not just
curiosity, but crucial tools to
decelerate the aging clock. And these
tools often converge on one central
theme that was shared last night,
inflammation and our immune system. We
all know the role of inflammation in CF.
We've been fighting it together for
decades and we're always looking for
that next best anti-inflammatory.
Inflammation is a principal driver of aging
aging
and the age related coorbidities in the
general population. And here are some of
the most common coorbidities which are
also relevant when talking about
accelerated aging and what we plan to
slow down in CF. There is a sclerosis or
damage to the blood vessels around the
heart involving inflammation. Cancer
isn't usually life-threatening unless it
spreads and our immune response can stop
that from happening. If it wasn't for
inflammation in the brain, we wouldn't
see dementia or stroke develop. And when
our immune system engages in
self-directed attack on our own body
tissue, we may deal with joint pains
like in CF related arthritis.
Now, for a quick refresher, this might
take a while. And I wish Jim Schmile was
in the house to come up here, and I'm
sorry he was not able to be here to help
me explain this slide because he helped
create this. So, let's erase that slide.
I'm just kidding.
Recently, actually a non-medical family
member asked me this really tough
question. He said, 'What is
inflammation? And so I had to figure out
how to put it simply. The immune system
coordinates both pro-inflammatory and
anti-inflammatory responses. We need
inflammation. It's essential for
defending the body against injury and
but it must be tightly regulated and
balanced by anti-inflammatory processes
to restore and maintain homeostasis.
Otherwise, excessive or unresolved
inflammation can lead to tissue damage
and premature aging in CF.
There's a book called Super Agers by
Eric Toppel, who just happened to be one
of my teachers and mentors from
residency. He outlined five themes or
does still outlining five themes. He's
on a bunch of podcasts. Managing
inflammation and aging. We will focus
today on immunity and lifestyle while
also touching on medicine and vaccines.
And there's so much to say on the topic
of artificial intelligence and we heard
about it at the TDN conference, but we
will leave the role of AI for another
day and hope you got to see some of the
sessions on the use of AI in clinic.
That was yesterday.
So actually Lisa Kunzie and Stephanie
Craig did did a great job. So reading
that their manuscript, you've got it all
figured out. You don't need to listen to
this. Um, but much of they conclude is
once again a consequence of inflammation
and the wear and tear of living life
with CF. Hear that a lot. Think of it
like a fire that never quite goes out.
It keeps smoldering and damaging tissue.
There is damage to DNA as inflammation
directly releases toxic molecules that
chip away at our genetic material and
that raises the risk of cancers in areas
where inflammation is most prominent.
There's a protein stress. Misfolded CFTR
proteins clogging the cell's recycling
system can spark even more inflammation.
And then there's the powerhouse of our
body, the mitochondria. It burns out and
it fuels inflammation once again. Stress
cells stop dividing but don't disappear
and release more of these inflammatory
signals and the body's repair efforts
just get exhausted while enzymes break
down lung tissue leading to scarring and stiffness.
stiffness.
We will talk about telomeirs shortening
in a minute. But all of this woven
together pushes aging more quickly in CF.
CF.
While not a complete reversal of
inflammation, some of the drivers that
we just showed, and these are three of
them just mentioned, are positively
impacted by correcting CFTR with
currently available modulators for those
who are eligible.
And some examples of how modulators help
reduce inflammation includes a study by
McN and Rosenfeld and others observing
infants ages 1 to four months
participating in an openlel ivvictor
study. And this study revealed its
positive impact on imactive trinogen a
marker of inflammation of the pancreas
and fecal calprotectin a marker of
inflammation in the gut. And there are
many other examples that include
ivorvictor and its role in reducing
inflammatory proteins like IL8 and IL1
beta in sputum and IL6 in blood.
Another study by Einerson and Tunny and
others observed an association between
ETI and reduced levels of several other
cytoines and chemocines and these are
those are the molecules that I've
already mentioned that enhance
inflammation and there was a session
just yesterday by Iman Mullen on the
same topic.
But this is where inflammation comes to
life for everyone. Telomeirs. There are
other predictors of biologic age called
pheno age or an even more precise one
called grim age. Although I don't love
that name
which is associate and by the way lots
of cos out there. They're checking their
telomeres all the time. That's on their
little list of uh labs that they check.
But telomeirs are still the most
recognizable. So, we're going to do a
quick lesson. Telomeirs are specialized
structures made of repetitive DNA
sequences or olonucleotides
along with special proteins that are
dangling and capping the ends of these
chromosomes. Imagine the plastic caps at
the end of your shoelaces and these are
called aglets preventing the shoelaces
from unraveling and needing to be
replaced. So, it's just like that. And
these caps similarly protect chromosomes
from damage and from mutating. But with
each cell division these telomeirs shorten.
shorten.
They shorten by chronic stress.
Inflammation drives this and which leads
to telomeir dysfunction and cellular
aging. Think of telomeirs as your
biological clock for those cells and
their gradual shortening over time is a
key process in aging and the development
of chronic disease.
All right, hitting closer to home for
some stress experienced by resident
physicians, particularly during their
first year of training, leads to
increased systemic inflammation and
accelerated telomeir shortening.
Longitudinal corate data show that first
year residents, so if you're first year
resident, close your eyes. And for
everybody else who are physicians, it's
too late.
It's too late. up to six times the
typical annual rate of telmir attrition
compared to population norms. And you
can see the other population norms
there. And this effect is directly
associated with the higher workload and
longer work hours and lots of sleep
deprivation. So we got to watch that.
And as well as markers of inflammation
like C reactive protein and CRP.
In a study of 168 people with cystic
fibrosis with longitudinal data
available for just over half of them, we
see progressive decrease in telmer
length as well.
They especially by no surprise decrease
associated with exacerbations and in men
more than women and also associate with
mutations that are consistent with
greater disease severity.
Now a message for everyone in the
audience and it's going to change the
way you behave. Observational and
interventional studies consistently show
that vitamin D deficiency is associated
with higher levels inflammatory markers
as such as CRP, IL6, and TNF alpha. And
in this observational study with just
over a thousand participants, it should
come as no surprise that your vitamin D
levels were associated with longer
telomeres and further modified by
calcium intake. I mean, I would tell
other people to take vitamin D, but I
wasn't taking vitamin D until I read
this article.
The shortened telomeirs associated with
low vitamin D are thought to represent a
three-year difference in life
expectancy. And that's huge.
So how do we tackle all of this?
Great care is never the work of one
person. And I want to give a shout out
to the amazing pediatric adult CF team
at our combined Brigham and Women's
Hospital in Boston Children's Hospital
CF Center. Can I hear from you guys? [Applause]
[Applause]
Thank you. That was great. And I can't
do this talk alone either. So, please
help me welcome to the stage my friend,
a brilliant physical therapist and a
human being who I am thrilled to share
the stage with tonight is Dr. Karen von Berg.
Berg. [Applause]
[Applause] [Music]
[Music]
Thank you. Before I get started, I'd
like to give a shout out to the peds and
adult teams at Hopkins. I would not be
Likewise to the CF physical therapists
who I've learned from and with over the
years. You guys are the best. [Applause]
[Applause]
to my husband and my three girls who are
home. Well, some of them are home in
Maryland watching from the family room
and one watching from the dorm room in
Jersey. Thank you so much for putting up
with me for the last few months getting
ready for this talk.
and to the people with CF,
including those we've lost. I'm humbled
by how much you've taught me and
extremely honored to be part of your
care team.
My journey in CF began when I started at
Hopkins in 2000, shortly after our adult
program got started, and when most of
our role as physical therapists was
focused on airway clearance and
generally promoting an active lifestyle.
Who knew that 25 years and 24 NACFC's
later, I'd be standing on this stage
talking about aging with CF.
It's been so wonderful working in both
the pediatric and adult clinics to get
to watch our patients grow up, to grow
older with them, and to be with them as
they transition to adult care, begin
careers, build families, and explore new
decades of life.
Since 2000, my role as a PT has evolved
and expanded. We've learned so much
about the complications beyond the lungs
and the pancreas that become even more
critical as people age. Now, I used to
think of CF, the complications and all
the treatments like pieces of a jigsaw
puzzle. You need to put all of the
pieces in or do all of the treatments in
order for things to work. And while I do
love a good jigsaw puzzle, I got that
from my mom who's here in the audience
tonight. Thank you.
um that seemed too static for CF. CF has
lots of moving parts and requires
maintenance, more like a system of gears.
gears.
Speaking of gears, let me introduce you
to link. Link is a metaphor to describe
the complex systems that all connect
with one another within the human body.
Link will be making appearances
throughout this plenary to help us take
a look at what happens if a gear gets
rusty and stops turning and how can we
keep the CF gears running efficiently as
people with CF grow older.
One example of how different systems are
connected is posture. So let's take a
look at what poor posture feels like and what
what
people sitting up a little bit
straighter. Um, let's take a look at
what poor posture feels like and what
can result from poor posture. So, I'd
like everyone in the audience and those
of you at home to slouch down in your seats.
seats.
Let your hips slide to the front edge of
your seat. Hunch your spine forward.
Roll your shoulders in. Drop your chin
to your chest. Keep it there.
Take a breath in
and out.
And then you can sit back up with good
posture. Please
remember what that felt like. We're
going to come back to it later. When our
body isn't aligned correctly, first and
foremost, our breathing is restricted.
Additionally, our joints, muscles, and
even internal organs can't function at
their best. We then begin to see
impairments such as pain, urinary
incontinents, and fractures. These
things may restrict ability and
willingness to be active or to do airway
clearance which can lead to more lung
infections. Then bring on the
antibiotics and with more exposure to amoglycosides
amoglycosides
people with CF may not experience just
loss of hearing but also vestibular
impairments feeling dizzy and offbalance.
offbalance.
When compounded with normal age related
changes, people with CF are at even
higher risk for falls.
PT specific tests and measures to screen
exercise capacity, strength, balance,
and more will help guide personalized
interventions to prevent and address
these impairments. But we shouldn't wait
until people are reporting these concerns.
concerns.
Mary Massie, a PT guru for all things
pulmonary and neuromuscular, published
this article back in 2005.
She stressed the importance of early
intervention, stating it's easier to
prevent than to correct a postural
problem. With CFTR modulators and other
treatment advances allowing people to
live longer, it's becoming much more
apparent and important to intervene at a
young age, to build strong muscles and
bones, to address postural concerns and
breathing mechanics, to establish
healthy habits, to not only live longer
but to live stronger with a high quality
of life. So to the pediatric clinicians
in the audience, we may be talking about
aging and growing older today, but so
much of what allows people with CF to
live into adulthood starts when they're
in your care.
Just like the gears of our friend Link
working together, the multiddisciplinary
care team is interconnected to make sure
people with CF are well cared for. It
takes a team and good communication
amongst the team to make sure all the
moving parts of living with CF are welloiled.
welloiled.
And while we're well-versed in how the
core members of the CF team work
together as people with CF grow older
and we're seeing additional
complications and co-orbidities, the
core team is compelled to partner with
primary care providers and other
specialists which we'll hear about
throughout this plenary. Last, but
definitely not least, I want to
highlight the main input gear on the
team. The gear that gets the system
started and keeps it going, the
individual with CF. They are the expert
that truly drives the machine that is
their body and their care. The rest of
us, the clinical team, are the
supporting gears. It is so important for
us as clinicians to listen and learn
from the lived experience of the person
with CF.
So before Attjoins me on stage to grind
through the gears of this talk, let's
hear from the experts themselves about
what it's like to grow older with CF. [Music]
[Music]
One of the perks of aging with CF is
that you have an abundance of doctors. [Music]
[Music]
I feel like I'm on one side of the
fence, but CF puts me on the other side
anyway. I'm like, I don't belong over
here. My people are over there. My
things, my stuff is over there. My life
is over there. CF was like, "No, you're
here now." I'm trying to learn to be aged
aged
while also trying not to rule out what
I finish my treatments, I go to the gym
or I go to the pool or I go out for a
run. Definitely as you age um your
exercise has to change and the
medications we take weaken our tendons
and our joints can cause little minute
fractures in in bones. I used to be a
runner who ran every day of the week.
Now if I run two days out of the week
that's it. It's a constant journey of
discoveries with age. Um but aren't we
What I'm discovering is that this circle
is actually becoming equally, if not
more important than my clinic team. When
I finally found a gynecologist who was
qualified in cystic fibrosis, I was
blown away. She opened my eyes to how as
a female with this disease and now in
menopause, how your world can be changed
just by a doctor having that background.
It wasn't until I found these these
trained gynecologists who got me on the
right hormone therapy. That's when
things changed for me. Oh my goodness. I
mean, there's no looking back.
CF ages you in ways that aren't always so
so
obvious. Sometimes they're subtle. You
can't breathe, so you do less. And
because you do less, your body stops
responding when you need it to do
things. It is um a chain reaction and
everything you do there's a give and a
take. I was on troy for a little while
and one of the side effects of that was
was hearing loss which does not bode
well for a musician. So we stopped
taking it immediately.
>> I got I got to go meet with the
aiologist. But at first it was this like
I don't know if it's cuz I'm just
getting older. I don't know if it's just
years of loud music. The unfortunate
thing with hearing loss is that it too
is progressive and there's no like
undoing. There's there's no reset
button. So
>> I think what the clinic care teams need
to know is we're not just CF. We're more
than CF. We're more than the lungs, the
pancreas, and the sinuses. We react to
medications differently than the next CF
patient. The clinic teams need to keep
those open minds. Te certain teachers
had students write what they wanted to
when I was leaving. [Music]
I find myself being okay with being greedier
greedier
about life. At first, it was like I just
want to be able to see, you know, my
niece and nephew and little brother
graduate. And now I've watched all three
of my siblings graduate. My nephew
graduates this year and now they're
succeeding and I'm like I still want to
I mean, we've watched that so many times
and each time it just touches us in a
way like as if we're watching it for the
first time. It's really amazing and
truly brilliant. And thank you to Jen
and Brandon to inviting us into your
lives into the places that you exercise
into listening join you listening to you
jam Brandon and then also into your
living rooms and your in you know in his
uh Brandon's case my dear friend Brandon
his bedroom and in their own words
exemplifying the constant journey of
discoveries with age and he will be a
really hard act to follow but Karen and
I are back here symbolizing the role of
teams working together to slow down or
stop inflammation. And we will follow
this invisible thread of inflammation uh
throughout this talk as we navigate
through a small sample of body systems
and impairments. Um these are critical
as people age and the gears that can
rust without proper attention. And we
hope this plenary helps foster
conversation and collaboration across
the care team and with other specialists.
specialists.
>> So why is this topic important to us? We
already showcased data on improving life
expectancy and we already have done so
much to decelerate the aging process,
but we have work to do since aging still
is accelerated in CF. People living with
complex illness offer a powerful window
into how inflammation accelerates aging
and even helps us understand the biology
of aging itself. In CF, we see heart
disease, kidney disease, and cancer
appearing earlier. And as highlighted by
a figure showing age and sex adjusted
rate ratios by our colleagues Ria Arya
and Anne Stevenson from Canada in a
publication from this summer, we do need
to remember that this study in large
part was done before modulators.
However, it is still important to note
that people with CF may experience
cardiovascular disease and cancer 20
years earlier and if you've been
transplanted 30 years earlier.
So, let's turn our attention to the
leading cause of death in the general
population and previously one of the
more underestimated gears in CF, the
cardiovascular system.
Just as inflammation drives pulmonary
and gastrointestinal disease, it also
causes wear and tear on our
cardiovascular gear. Some examples shown
here once again implicate persistent
inflammation as it drives vascular
aging. In addition to hypoxmia and sleep
disordered breathing, CFTR itself plays
a role with loss of function linked to
abnormal vascular tone. CF-reated
diabetes is increasingly common and adds
to cardiovascular risk and the legacy CF
diet highfat low fiber may fuel
dysipidemia and metabolic syndrome
>> and this may be confusing but on the
flip side when you do get modulators it
is associated with increased number of
people with high blood pressure and
there's an increased evidence of
negative impact on lipids.
>> Yeah. And we're also seeing much higher
rates of people who are overweight and
obese. So again, we're seeing a gear
that wasn't expected to turn this long
and it's starting to show signs of strain.
strain.
>> Yeah. I was having a conversation with
our division chief and he reminded me
about lung function as it relates to the
Framingham heart study which has been
count uh counting I'm sorry which has
been following over 5,000 people in
counting and it's still going in
Framingham Massachusetts for a long time
with an eye on cardiovascular outcomes
and disease and we all heard the
messages of high blood pressure,
smoking, cholesterol and how it impacts
cardiovascular disease. However, what we
don't talk about and we bury is vital
capacity or lung function and how it
impacts and has an oversized role in
cardiovascular risk, especially in
women. So, in this talk about everything
other than the lungs, we want to make
sure that you know the message is clear
that we are still focused on maintaining
the best lung function. >> Yeah.
>> Yeah.
>> So, Karen,
how do we protect that cardiovascular
gear during the lifespan of someone with
CF? Well, it starts with screening,
including annual lipid panels and blood
pressure monitoring, especially for
those with CF-reated diabetes who are at
higher risk and regular diabetes
screening for those without CFRD.
In some cases, we might even consider
stress testing and vascular imaging such
as coronary artery calcium scores to
catch early changes. And we need to work
with primary care providers on more
aggressive management.
>> Absolutely. Primary care providers are
so critical and it would be great to
have a CF specific cardiovascular risk
score and we're grateful that there are
multiple groups that are working on this
including both here and overseas and
including a young investigator Evan
Manning who's a cardiologist working
with Alex Despotes who you've seen her
work and but she's not here because just
had a baby and hopefully she's not
watching and she's getting some sleep.
>> We also have other lubricants to support
this gear. Nutrition is a powerful lever
in controlling inflammation. And while
we still need to support adequate
caloric intake, we can do it with more
heart-healthy strategies.
>> Yeah, there are foods that can be
divided into those that are
pro-inflammatory and those that reduce
inflammation. So, the great thing is
speak to your dietitian. You have one
right there. To ensure people are
minimizing the inflammatory foods they
they're intaking, including those rich
in omega sixes and eating more
monoounsaturated fats and foods that
contain high amounts of fiber, omega-3s,
polyphenols, which are compounds that
come from plants. Mhm.
>> So while CFTR modulators may reduce
systemic inflammation, improve insulin
secretion and vascular uh function,
there are medications like hormone
therapies and also statins. Now statins
have high anti-inflammatory properties
and the American Diabetes Association
recommends starting them if you're over
the age of 40 and have diabetes and
that's not happening universally.
Additionally, we know lifestyle is
critical and including again better
sleep, reduction stress, and of course, exercise.
exercise.
>> I'm so glad you mentioned exercise.
Exercise is a dualaction lubricant here.
It improves cardiovascular fitness and
insulin sensitivity. Data show that even
low to moderate activity can improve
endothelial function, lower blood
pressure, and regulate glucose metabolism.
metabolism.
>> Yeah. So the take-home message,
cardiovascular disease is outcome that
is not inevitable and screening is no
longer optional in CF. So reduce
inflammation through exercise and
healthy eating and the Mediterranean
diet is good example of how to combat
heart disease. So thanks mom. I'm Mediterranean.
Possibly one of the most overlooked
gears of CF is pain. It's a powerful
disruptor and more common as we age with
significant negative impact on quality
of life, mental health, and daily
functioning. And though CFTR modulators
can dial down inflammation,
pain prevalence, and severity do not
correlate with modulator use. A recent
survey by Ward and others shows that up
to 70% of adults with CF report chronic
or recurrent pain, much higher than in
people without CF. And the most commonly
reported areas include headaches, joint
and backachches, and GI pain.
>> Yeah, chronic pain is often hidden by
stigma and bias. Too often it is brushed
off as just infection related and
leaving patients to feel like they have
to tough it out. And clinicians are
hesitant to ask those questions. But
pain is more than a symptom. It is its
own dysfunctional gear and slowing
movement affecting sleep, mood,
breathing, and treatment adherence.
These are quotes from an editorial
written by three people with CF. Nure,
Cade, and Casey.
Pain is awful, but being disbelieved is excruciating.
excruciating.
>> And if you haven't already, I would
highly recommend you read that article.
It truly sends a powerful message.
The good news is pain can be managed and
not just with medication. Like any gear
under the stress of inflammation, it
just needs the right lubrication,
thoughtful, individualized and
multidisciplinary care. First, we have
to actually look at it. Make pain
assessment a routine part of clinic
visits, not just when someone is sick.
We need to identify patterns early. The
newly published CF foundation pain
guidelines recommend routine screening
with a promised pain scale to capture
both pain intensity and functional
impact. For non-verbal adults, we have
the observational flax scale. And
there's also the very recognizable
visual analog scale. Any level of
endorsed pain should be followed with a
comprehensive clinical assessment and
appropriate interventions.
>> And we're broadening our
pharmacologicals toolbox for pain and
cystic fibrosis moving towards more
balanced and multimodal approach. And
depending on the source of the pain,
there are a variety of options. While
there are role for medications including
opioids, medications alone, even just
anti-inflammatories are never the whole
solution. They work as part of a broader
multimodal pain management plan. So,
what else should that plan include?
>> Well, there's a lot more beyond
medications, and movement is one of our
most powerful tools. Regular structured
exercise can help prevent stiffness,
preserve strength, improve mood, and
reduce systemic inflammation.
Even short activity snacks throughout
the day can slow the pain gear. And when
help is needed, this is where physical
therapy can play a critical role. PTS
can assess biomechanics, postural
patterns, and muscle imbalances which
may be contributing to pain.
>> I could really go for an activity snack.
I am starving. I haven't eaten all day.
And let's not forget about the role of
mental health. People with CF who have
chronic pain are more likely to
experience emotional distress, which in
turn amplifies pain perception. So
mindfulness and re uh relaxation
techniques can lower that perception of
pain and also help people manage
flare-ups. Karen, you taught me this.
Incorporating cognitive behavioral
therapy along with acceptance and
commitment therapy alongside physical
rehab improves both pain and mental
health. So improving sleep is also
critical. Better rest raises pain
thresholds and supports mental health.
And as we hear from people with CF, they
don't expect the CF team to be experts
in pain, but we should partner with them
in solving it. And that may likely
involve educating other specialists
about CF. And the take-home message
here, in short, pain is not just a
symptom. It's a gear of its own and a
byproduct of stress on the body over
time. With early recognition and a
maintenance crew of a team, we can
reduce inflammation and get this gear
moving again.
>> Yeah. Closely related to pain is another
gear that is critical for our function
and maintain our quality of life and
that's the muscularkeeletal system.
>> Yeah. Let's think of the bones and
muscles as the foundation gears in the
complex machine of the human body.
Without a strong stable muscularkeeletal
system, other gears such as breathing,
posture, pain, even glucose metabolism,
cardiovascular health, and more can't
function properly.
Let's start with muscles. Studies
comparing adults with CF to healthy
peers consistently show lower muscle
power and reduced peripheral muscle
strength, including lower hand grip
force and weaker thigh muscles. These
deficits can impact participation in
daily life, respiratory performance and
overall survival.
>> CF related bone disease is well
recognized complication and it appears
much earlier than in the general
population. Data from the CF foundation
registry show that about one in four
adults with CF have low bone mineral
density, but some single center studies
show that it's almost 50%. And in the
general population about 30% of women
over 65 have osteoporosis but it is
nearly 40% in women with cystic
fibrosis. So the risk is even greater
after transplant unfortunately where 45
to 73% of people experience bone loss
and the steepest loss of bone happens in
that first year after transplant.
>> So Karen, what's causing these changes
to bone and muscle? Your favorite topic.
>> Yeah. While I'm met, there are many
causes of bone disease and we'll speak
to just a few of them tonight.
>> CF-reated diabetes, poor calcium intake,
and malabsorption of vitamins's D and K
can slow down the muscularkeeletal
gears, and vitamin K is especially
important for bone mineralization.
Post-ransplant, amunosuppressives and
steroids lead the way. Estrogen
deficiency especially around perry and
early menopause lead to rapid loss of
bone and muscle mass and therefore
increased fracture risk. Reduced
physical activity activity is yet
another contributor.
>> Yeah. And inflammation once again the
culprit here. >> Again
>> Again
>> inflammation promotes bone loss by
signaling bone breakdown and inhibiting
the building of bone. And this occurs
through a lot of different pathways, but
one example is wrinkle, a newer
pharmaceutical target that may not be
appropriate for everyone, but when
activated in the absence of CFTR,
promotes muscle and bone breakdown.
>> Great. And here's the encouraging part.
We know how to maintain and even
strengthen this gear at any age, but
like any machine, it requires early
investment, regular maintenance, and
coordinated care.
Muscle is one of the most adaptable
tissues in the human body. First, in
children, building muscle mass is
critical. In this conceptual model from
Gru and colleagues, we see what happens
to muscle strength over time. For people
in the general population who
participate in sports and have normal
levels of physical activity. For people
with CF to maintain close to that muscle
strength, they need high levels of
physical activity with resistance
training and sport participation.
And for those who are more sedentary
with decreasing activity um in the later
decades, we see even steeper declines in
muscle strength. So to sum up what is
shown here, people with CF have an
uphill battle and have to do more to
build and maintain muscle strength.
>> So what do you suggest they do, Karen?
Well, I'm glad you asked. >> Good.
>> Good.
>> Early resistance and functional training
when young helps to maximize peak muscle
mass, serving as a buffer for later
years and also a strong predictor of
peak bone mass as well as lifelong
mobility. In adulthood, programs should
be tailored to match each person's
baseline strength, fracture risk,
motivation, and readiness to exercise.
This includes resistance training,
jumping exercises for those who are
able, and posture correction to prevent
vertebral stress.
For individuals who are less active,
have advanced lung disease or are at
higher risk for fractures, we can start
with lower impact options and gradually
build up.
>> Yeah, an individualized approach is best
and whatever you can do. And while we
don't have any large studies specific to
muscle and exercise outcomes from people
taking CFR modulators, there are some
emerging randomized control trials. So
Sosa Pradeski and others have showed
that a remotely supervised resistant
training program with moderate to high
intensity improved lower limb muscle
strength and body composition compared
to those who didn't exercise. And then
Clayton colleagues compared adults with
CF taking ETI to healthy controls and
found similar outcomes in terms of body
composition and peripheral muscle mass function
function
suggesting that modulators enable
muscles to catch up when paired with exercise.
exercise.
>> Yep. And in addition to exercise and
nutrition, early detection and
monitoring of bone and muscle health is
key. In the US, the median rate for DEXA
or bone density screening in CF is 66%. We need to do better. Low bone mineral
We need to do better. Low bone mineral density is treatable. Newer tools such
density is treatable. Newer tools such as vertebral fracture assessment and
as vertebral fracture assessment and frack scores should be considered to
frack scores should be considered to help with risk prediction. Please refer
help with risk prediction. Please refer to the bone disease clinical care
to the bone disease clinical care guidelines for more detailed information
guidelines for more detailed information on screening and interventions. And for
on screening and interventions. And for muscles, tracking body composition
muscles, tracking body composition rather than weight and body mass index
rather than weight and body mass index will help to guide discussions and
will help to guide discussions and assess if interventions are actually
assess if interventions are actually improving muscle mass.
improving muscle mass. >> And we need to broaden our CF team. We
>> And we need to broaden our CF team. We need to work with our pharmacists as we
need to work with our pharmacists as we do our endocrinologists, gynecologists,
do our endocrinologists, gynecologists, and other team members to determine the
and other team members to determine the best therapy or combination of therapies
best therapy or combination of therapies based on an individual's risk.
based on an individual's risk. Pharmacologically there are
Pharmacologically there are bisphosphinates and there are CF term
bisphosphinates and there are CF term modulators as we've learned modulators
modulators as we've learned modulators as we learned that help with bone
as we learned that help with bone density as well as new biological
density as well as new biological pathways that go beyond hormone therapy.
pathways that go beyond hormone therapy. For example, recently published work by
For example, recently published work by Petia on amino acid supplementation
Petia on amino acid supplementation shows promising results on how to
shows promising results on how to interrupt muscle mass loss and systemic
interrupt muscle mass loss and systemic inflammation in the general population.
inflammation in the general population. creatine has shown benefits in aging
creatine has shown benefits in aging populations for muscle strength, energy,
populations for muscle strength, energy, and brain health. However, hidden and
and brain health. However, hidden and invisible kidney disease does occur in
invisible kidney disease does occur in CF. So, this is something we need to be
CF. So, this is something we need to be cautious about.
cautious about. >> Yeah,
>> Yeah, strong bones and muscles allow us to
strong bones and muscles allow us to climb the stairs, carry groceries, play
climb the stairs, carry groceries, play with our kids, and so much more. Our
with our kids, and so much more. Our take-home message with early investment,
take-home message with early investment, regular screening, ongoing maintenance,
regular screening, ongoing maintenance, which includes inflammation management,
which includes inflammation management, and a coordinated team approach, we can
and a coordinated team approach, we can keep this power gear turning smoothly,
keep this power gear turning smoothly, keeping the rest of the system in
keeping the rest of the system in motion.
motion. >> Now, we've seen how inflammation drives
>> Now, we've seen how inflammation drives pain and muscularkeeletal complications,
pain and muscularkeeletal complications, and how they all misalign our gears,
and how they all misalign our gears, disrupting everything from movement to
disrupting everything from movement to mood to motivation. But behind the
mood to motivation. But behind the scenes, there's another system
scenes, there's another system manipulating inflammation as well, and
manipulating inflammation as well, and that's hormones.
that's hormones. >> Yeah, hormones can have a significant
>> Yeah, hormones can have a significant impact on aging, particularly in the
impact on aging, particularly in the form of pmenopause and menopause in
form of pmenopause and menopause in women and testosterone decline in men.
women and testosterone decline in men. These hormonal shifts affect many
These hormonal shifts affect many systems throughout the body. And while
systems throughout the body. And while there's fluctuation of multiple hormone
there's fluctuation of multiple hormone levels, we're going to focus on two
levels, we're going to focus on two today, estrogen and testosterone.
today, estrogen and testosterone. Chronic inflam infections, inflammation,
Chronic inflam infections, inflammation, frequent use of gluccocorticoids, and
frequent use of gluccocorticoids, and poor nutritional status all affect
poor nutritional status all affect hormone deficiency in both men and
hormone deficiency in both men and women. CFTR dysfunction also plays a
women. CFTR dysfunction also plays a role in women. CFTR dysfunction can lead
role in women. CFTR dysfunction can lead to ovarian dysfunction. And age, which
to ovarian dysfunction. And age, which we can't stop, is another cause of
we can't stop, is another cause of declining hormone levels. For women
declining hormone levels. For women after surge during pregnancy, we see a
after surge during pregnancy, we see a steep decline in estrogen during
steep decline in estrogen during pmenopause and menopause around age 50
pmenopause and menopause around age 50 and slightly earlier for women with CF.
and slightly earlier for women with CF. And testosterone decline
And testosterone decline also begins a bit earlier for men with
also begins a bit earlier for men with CF and steadily declines as part of
CF and steadily declines as part of starting yeah your aging process
starting yeah your aging process starting in your 40s and 50s.
starting in your 40s and 50s. >> Yeah. Symptoms of hormone deficiency for
>> Yeah. Symptoms of hormone deficiency for men include low libido, erectile
men include low libido, erectile dysfunction, infertility, and
dysfunction, infertility, and osteoporosis.
osteoporosis. Only about one in 10 men with CF have
Only about one in 10 men with CF have had their testosterone checked. And of
had their testosterone checked. And of those men, low testosterone is seen in
those men, low testosterone is seen in about onethird with a median age of just
about onethird with a median age of just 44 years.
44 years. >> And compare that to only 6 to 12% of men
>> And compare that to only 6 to 12% of men under the age of 60 in the general
under the age of 60 in the general population. So younger age middle-aged
population. So younger age middle-aged men with CF are showing rates similar to
men with CF are showing rates similar to or even higher than people over the age
or even higher than people over the age of 60 with without CF in the general
of 60 with without CF in the general population.
population. >> Yeah. And women may experience a wide
>> Yeah. And women may experience a wide variety of symptoms and symptom severity
variety of symptoms and symptom severity due to declining estrogen. And I think
due to declining estrogen. And I think the women over 40 in the audience will
the women over 40 in the audience will agree with me about these symptoms,
agree with me about these symptoms, especially those from dinner Wednesday
especially those from dinner Wednesday night.
night. >> Should have been invited. Should have
>> Should have been invited. Should have been invited.
been invited. >> Sorry. Sorry we didn't invite you.
>> Sorry. Sorry we didn't invite you. >> We've heard from women with CF like
>> We've heard from women with CF like Daniela if you attended symposium 4
Daniela if you attended symposium 4 yesterday. how they're unsure if these
yesterday. how they're unsure if these symptoms are due to CF, a reaction to a
symptoms are due to CF, a reaction to a medication, low blood sugar, or aging
medication, low blood sugar, or aging and perry menopause.
and perry menopause. >> And menopause is deservedly having its
>> And menopause is deservedly having its moment in our discourse and is of course
moment in our discourse and is of course of great interest to us because it
of great interest to us because it represents an opportunity to make a
represents an opportunity to make a difference and to limit the impact of
difference and to limit the impact of inflammation.
inflammation. The menopausal transition and early
The menopausal transition and early post-menopause period represents a
post-menopause period represents a window of opportunity to preserve bone
window of opportunity to preserve bone mass and prevent heart disease. Exercise
mass and prevent heart disease. Exercise specifically focused on building muscle
specifically focused on building muscle is key during this time period in
is key during this time period in addition to other therapeutic options
addition to other therapeutic options including hormone therapy for those who
including hormone therapy for those who are eligible. Yeah, whether we deliver
are eligible. Yeah, whether we deliver it systemic, transermal, or otherwise,
it systemic, transermal, or otherwise, when estrogen is present during
when estrogen is present during pmenopause, it can have a positive
pmenopause, it can have a positive long-term impact on bone density and
long-term impact on bone density and muscle strength as well as heart health
muscle strength as well as heart health and cognition.
and cognition. >> We have the menopause specific quality
>> We have the menopause specific quality of life instrument to help with
of life instrument to help with screening. In addition to the CFresh
screening. In addition to the CFresh website, which has resources such as
website, which has resources such as priority questions to help guide your
priority questions to help guide your discussions and then make the
discussions and then make the appropriate referrals.
appropriate referrals. >> Yeah. While in that process of referring
>> Yeah. While in that process of referring a patient to that expert, you need to
a patient to that expert, you need to consider some potential risk factors in
consider some potential risk factors in CF. Venus thrombboic disease or blood
CF. Venus thrombboic disease or blood clots, heart disease. A big no no is
clots, heart disease. A big no no is liver and bilary disease and prostate
liver and bilary disease and prostate cancer in men
cancer in men >> and breast cancer and general cancer
>> and breast cancer and general cancer risk in women with hormone therapy made
risk in women with hormone therapy made the headlines when the women's health
the headlines when the women's health initiative first reported its findings
initiative first reported its findings in 2002. and based on the headlines of
in 2002. and based on the headlines of relative risk, but the absolute risk was
relative risk, but the absolute risk was actually quite small. However, we know
actually quite small. However, we know CF's chronic inflammation raises other
CF's chronic inflammation raises other cancer risks. So, the recommendation is
cancer risks. So, the recommendation is for continued cancer screening and a
for continued cancer screening and a treatment plan that's devised
treatment plan that's devised collaboratively with our expert
collaboratively with our expert partners.
partners. >> Exercise, diet, and social connections
>> Exercise, diet, and social connections are key for everyone, but especially if
are key for everyone, but especially if hormone therapy is not an option. There
hormone therapy is not an option. There are also non hormonal medications and
are also non hormonal medications and cognitive behavioral therapy which can
cognitive behavioral therapy which can help manage some of the symptoms.
help manage some of the symptoms. >> So our take-home message for everyone,
>> So our take-home message for everyone, we need to screen for symptoms and refer
we need to screen for symptoms and refer to specialists to discuss best treatment
to specialists to discuss best treatment options to address hormone deficiency
options to address hormone deficiency and ultimately reduce inflammation for
and ultimately reduce inflammation for each individual.
each individual. >> As I mentioned before, people with CF
>> As I mentioned before, people with CF can get cancer years earlier than others
can get cancer years earlier than others without CF. It isn't surprising that yet
without CF. It isn't surprising that yet again chronic inflammation plays a
again chronic inflammation plays a critical role and cancer is most
critical role and cancer is most prevalent where inflammation exists.
prevalent where inflammation exists. We're seeing many different types of
We're seeing many different types of cancer. Some are directly related to CF
cancer. Some are directly related to CF while others are influenced by age or
while others are influenced by age or age related conditions. The higher rates
age related conditions. The higher rates of GI cancers, for example, tie back to
of GI cancers, for example, tie back to gastroesophageal reflux disease, bowel
gastroesophageal reflux disease, bowel inflammation, and diabetes leading to
inflammation, and diabetes leading to oxidative stress and DNA damage. Risk is
oxidative stress and DNA damage. Risk is further increased when we add in
further increased when we add in impaired mucosal barriers, changes in
impaired mucosal barriers, changes in the gut microbiome, bowel obstruction,
the gut microbiome, bowel obstruction, altered immune responses, and perhaps
altered immune responses, and perhaps lifestyle factors such as that highfat,
lifestyle factors such as that highfat, low-fiber diet, vitamin D deficiency,
low-fiber diet, vitamin D deficiency, and low levels of activity. So, I'm at
and low levels of activity. So, I'm at what else is driving this elevated
what else is driving this elevated cancer burden?
cancer burden? >> Yeah, that's a million dollar question.
>> Yeah, that's a million dollar question. CFTR dysfunction is associated with
CFTR dysfunction is associated with multiple pathways of increased cancer
multiple pathways of increased cancer risk and whether CFTR modul modulators
risk and whether CFTR modul modulators reverse this is still yet to be
reverse this is still yet to be determined. It also drives inflammation
determined. It also drives inflammation increasing cytoines and chemocines
increasing cytoines and chemocines altering signaling pathways and
altering signaling pathways and increasing pathways like TGF beta betain
increasing pathways like TGF beta betain and NFCappa B. So when CFTR functions
and NFCappa B. So when CFTR functions normally it actually acts as a tumor
normally it actually acts as a tumor suppressor gene. One of its roles is
suppressor gene. One of its roles is maintaining epithelial integrity and
maintaining epithelial integrity and inhibiting something called epithelial
inhibiting something called epithelial mezzenymal transition or EMT.
mezzenymal transition or EMT. However, disregulation of EMT is what
However, disregulation of EMT is what contributes to cancer being more
contributes to cancer being more aggressive or spreading by enabling
aggressive or spreading by enabling epithelial cells to lose polarity, lose
epithelial cells to lose polarity, lose that cell adhesion and acquire mezenymal
that cell adhesion and acquire mezenymal traits or in other words characteristics
traits or in other words characteristics of the surrounding tissue. And
of the surrounding tissue. And essentially they blend in and they gain
essentially they blend in and they gain migratory and invasive capabilities.
migratory and invasive capabilities. These characteristics are what's
These characteristics are what's essential for tumor progression,
essential for tumor progression, invasion and metastasis. So even when a
invasion and metastasis. So even when a tumor from someone who doesn't have CF
tumor from someone who doesn't have CF acquires a CFTR mutation, that tumor
acquires a CFTR mutation, that tumor becomes more aggressive or malignant.
becomes more aggressive or malignant. Low CFTR expression in tumors correlates
Low CFTR expression in tumors correlates with advanced stage disease, lymph node
with advanced stage disease, lymph node metastasis and poor prognosis. So add in
metastasis and poor prognosis. So add in transplant related imunouppression which
transplant related imunouppression which impairs tumor surveillance and this gear
impairs tumor surveillance and this gear becomes especially vulnerable.
becomes especially vulnerable. >> But the good news is we have the tools
>> But the good news is we have the tools to keep the cancer prevention gear
to keep the cancer prevention gear moving but only if we use them early
moving but only if we use them early consistently and equitably. Many people
consistently and equitably. Many people still think of cancer as something that
still think of cancer as something that happens later or to other people but the
happens later or to other people but the data support the exact opposite.
data support the exact opposite. So the number one message is screening.
So the number one message is screening. And if you listen to Conan O'Brien like
And if you listen to Conan O'Brien like I do, he loves a good colonoscopy.
I do, he loves a good colonoscopy. >> Don't we all?
>> Don't we all? >> The CF Foundation currently recommends
>> The CF Foundation currently recommends colonoscopy starting at age 40 or age 30
colonoscopy starting at age 40 or age 30 for those who had lung or had a yeah had
for those who had lung or had a yeah had a transplant. And of course consider
a transplant. And of course consider earlier screening or other risk factors
earlier screening or other risk factors or concerning symptoms. If other
or concerning symptoms. If other concerning symptoms are present, you
concerning symptoms are present, you need to repeat screening every five
need to repeat screening every five years or based on findings more
years or based on findings more frequently.
frequently. >> Yeah. In a 2022 registry review, less
>> Yeah. In a 2022 registry review, less than 25% of eligible adults with CF had
than 25% of eligible adults with CF had undergone colarctal cancer screening.
undergone colarctal cancer screening. Today, the numbers screened and
Today, the numbers screened and therefore those avoiding cancer are
therefore those avoiding cancer are increasing. However, they are still not
increasing. However, they are still not high enough at only 43%.
high enough at only 43%. >> This is not negotiable. People, we must
>> This is not negotiable. People, we must do better. And colonoscopy has been
do better. And colonoscopy has been shown to reduce mortality in this
shown to reduce mortality in this population. So at our center Steve
population. So at our center Steve Freriedman when he finds and removes a
Freriedman when he finds and removes a precancerous polip it's a cause for
precancerous polip it's a cause for celebration because we know we avoided
celebration because we know we avoided another case of colorectal cancer and we
another case of colorectal cancer and we are grateful to the NYCF team to share
are grateful to the NYCF team to share their results with us and you may have
their results with us and you may have seen them uh you know report their
seen them uh you know report their results and in the largest study of its
results and in the largest study of its kind they evaluated whether non-invasive
kind they evaluated whether non-invasive stool testing predicts findings of
stool testing predicts findings of polyps or colon cancer. While
polyps or colon cancer. While non-invasive stool testing was
non-invasive stool testing was preferred, I get that to colonoscopy,
preferred, I get that to colonoscopy, they are not recommending stool studies
they are not recommending stool studies due to this high false negative rate and
due to this high false negative rate and poor correlation with colonoscopy
poor correlation with colonoscopy findings.
findings. >> Here's a summary of recommendations
>> Here's a summary of recommendations beyond colonoscopy.
beyond colonoscopy. Cancer is no longer an abstract future
Cancer is no longer an abstract future threat. It's part of the present- day
threat. It's part of the present- day experience of living longer with CF. And
experience of living longer with CF. And bringing that gear into focus is part of
bringing that gear into focus is part of comprehensive forwardthinking CF care.
comprehensive forwardthinking CF care. This means ensuring people are getting
This means ensuring people are getting their mammograms and testicular exams as
their mammograms and testicular exams as well as age related screens for skin and
well as age related screens for skin and prostate cancer
prostate cancer >> and and for those with chronic
>> and and for those with chronic imunuppression we need to screen even
imunuppression we need to screen even earlier and additionally screen for
earlier and additionally screen for things like lymphoma and HPV related uh
things like lymphoma and HPV related uh cancer. So dermatologic exams, papsmears
cancer. So dermatologic exams, papsmears and even prevention with the HPB vaccine
and even prevention with the HPB vaccine are really important not to forget and
are really important not to forget and also see our pancreatic cancer screening
also see our pancreatic cancer screening improvement project on poster 724.
improvement project on poster 724. So, we need to talk openly about cancer
So, we need to talk openly about cancer risk, including obtaining a good family
risk, including obtaining a good family history, maybe something we weren't
history, maybe something we weren't doing so as much before. And remember,
doing so as much before. And remember, family members who are carriers of the
family members who are carriers of the CFR mutation are also at increased risk,
CFR mutation are also at increased risk, slightly increased risk of cancer as
slightly increased risk of cancer as well compared to general population.
well compared to general population. >> So, the take-home message, comprehensive
>> So, the take-home message, comprehensive screening is important. We need to
screening is important. We need to target inflammation and modify those
target inflammation and modify those risk factors for developing cancer. And
risk factors for developing cancer. And please don't forget to recommend
please don't forget to recommend exercise to both mitigate cancer risk
exercise to both mitigate cancer risk and help improve outcomes for those with
and help improve outcomes for those with cancer.
cancer. Today, we've covered just a handful of
Today, we've covered just a handful of the gears that drive growing up and
the gears that drive growing up and growing older with CF. Yet, there are so
growing older with CF. Yet, there are so many others we just couldn't get to in
many others we just couldn't get to in our limited time up here.
our limited time up here. >> And we didn't forget about hearing loss
>> And we didn't forget about hearing loss and run. It's definitely a topic we need
and run. It's definitely a topic we need to address and needs more attention for
to address and needs more attention for sure. Yeah, it's really a lot. So, let's
sure. Yeah, it's really a lot. So, let's take a moment for a breather. By this
take a moment for a breather. By this point, hopefully you've seen the
point, hopefully you've seen the importance of a healthy, active
importance of a healthy, active lifestyle. So, how about we all get
lifestyle. So, how about we all get moving together. To those of you
moving together. To those of you watching at home, please join in. I'd
watching at home, please join in. I'd like everyone to come forward to the
like everyone to come forward to the front edge of your seat and put both
front edge of your seat and put both feet flat on the floor.
>> I can use the word posture. Okay,
Okay, >> think back to that breath you took
>> think back to that breath you took earlier with slumped posture and
earlier with slumped posture and remember how it felt. Now I'd like
remember how it felt. Now I'd like everyone to lift their chest. Roll your
everyone to lift their chest. Roll your shoulders back and down and take a nice
shoulders back and down and take a nice big slow breath in
big slow breath in and out.
and out. Easier than with slumped posture.
Easier than with slumped posture. Now let's add some movement. With elbows
Now let's add some movement. With elbows at your sides and palms facing up, take
at your sides and palms facing up, take a breath in as you rotate your hands out
a breath in as you rotate your hands out and squeeze those shoulder blades
and squeeze those shoulder blades together.
together. We're all family here. It's okay. And
We're all family here. It's okay. And then bring your hands back in as you
then bring your hands back in as you blow out.
blow out. >> Hopefully they're
>> Hopefully they're >> Let's do one more. Okay. So, elbows at
>> Let's do one more. Okay. So, elbows at your sides, chest is lifted. Rotate
your sides, chest is lifted. Rotate hands out as you breathe in
hands out as you breathe in and then out.
and then out. Feels good to move, right? It's been a
Feels good to move, right? It's been a long day.
long day. >> Yeah, that feels good.
>> We're not done yet. >> Yeah, I also thought we were done. So to
>> Yeah, I also thought we were done. So to to recap, we briefly mentioned those
to recap, we briefly mentioned those four themes that we talked about earlier
four themes that we talked about earlier for today and how to reduce inflammation
for today and how to reduce inflammation to decelerate aging and prevent or delay
to decelerate aging and prevent or delay complications related to growing older.
complications related to growing older. And there is lifestyle interventions,
And there is lifestyle interventions, cells and immunity, genes and proteins,
cells and immunity, genes and proteins, medicines and vaccines. And a message
medicines and vaccines. And a message for everyone, providers, scientists,
for everyone, providers, scientists, people with CF, make sure to exercise,
people with CF, make sure to exercise, reduce stress, and get plenty of sleep
reduce stress, and get plenty of sleep to keep those telomeirs fresh.
to keep those telomeirs fresh. >> We encourage you, we're not done. Wait,
>> We encourage you, we're not done. Wait, >> we're close. We're close. I promise.
>> we're close. We're close. I promise. >> We're teasing you. We're teasing you. We
>> We're teasing you. We're teasing you. We encourage you to form relationships with
encourage you to form relationships with local specialists um such as menopause
local specialists um such as menopause experts and welcome them to become
experts and welcome them to become authorities in their field for people
authorities in their field for people with CF. Once they join the CF
with CF. Once they join the CF community, they will likely find it
community, they will likely find it deeply rewarding and quickly grow to
deeply rewarding and quickly grow to love working with this population like
love working with this population like we all do.
we all do. >> And that I've seen that happen. We need
>> And that I've seen that happen. We need to expand that next generation of
to expand that next generation of providers including with amazing
providers including with amazing programs sponsored by the cystic
programs sponsored by the cystic fibrosis foundation like digest and
fibrosis foundation like digest and vision and pace just to name a few. In
vision and pace just to name a few. In addition to initiatives sponsored by the
addition to initiatives sponsored by the CF foundation, there are other
CF foundation, there are other organizations are also doing their part
organizations are also doing their part like CFRI and the Nash family foundation
like CFRI and the Nash family foundation sponsored Pat Nash Fellows program.
sponsored Pat Nash Fellows program. >> Yeah. And um I've been listening to this
>> Yeah. And um I've been listening to this podcast about aging with CF. Can you
podcast about aging with CF. Can you tell us more about that?
tell us more about that? You listen?
You listen? >> I listen. Don't leave me in.
>> I listen. Don't leave me in. >> That's so great.
>> That's so great. >> Well, there's a grassroots podcast
>> Well, there's a grassroots podcast called It's a Long Story and it's
called It's a Long Story and it's dedicated to people and topics about
dedicated to people and topics about growing older and it is produced and
growing older and it is produced and co-hosted by people with CF. So visit
co-hosted by people with CF. So visit it's a long story.com and we welcome
it's a long story.com and we welcome your ideas and maybe we'll have you on
your ideas and maybe we'll have you on as a as a guest.
as a as a guest. >> It's pretty good. It's worth listening
>> It's pretty good. It's worth listening to. While trends and data
to. While trends and data >> Thank you for allowing me.
>> Thank you for allowing me. While trends and data from the larger CF
While trends and data from the larger CF community are extremely beneficial, it
community are extremely beneficial, it is still about the care team partnering
is still about the care team partnering with each individual.
with each individual. Practicing good communication to build
Practicing good communication to build and maintain relationships is of the
and maintain relationships is of the utmost importance and respecting and
utmost importance and respecting and trusting each other allows for those
trusting each other allows for those gear gears to keep turning. Trust does
gear gears to keep turning. Trust does not mean we have to think the same way
not mean we have to think the same way or have the same opinions. But trust is
or have the same opinions. But trust is honoring the person with CF as a
honoring the person with CF as a decision maker. It's listening without
decision maker. It's listening without judgment and respecting them as an
judgment and respecting them as an expert.
expert. >> Yeah. A quote from one of the experts
>> Yeah. A quote from one of the experts about aging. The question with me is,
about aging. The question with me is, you know, at at 77 years old, are we
you know, at at 77 years old, are we dealing with CF? Are we dealing with
dealing with CF? Are we dealing with age? Are we dealing with both of them?
age? Are we dealing with both of them? Or does CF make the age process worse?
Or does CF make the age process worse? We need to work with each individual to
We need to work with each individual to learn about their unique experience with
learn about their unique experience with aging. So ask your patients what's
aging. So ask your patients what's changing in their lives. They know their
changing in their lives. They know their bodies better than anyone else. Ask
bodies better than anyone else. Ask about what new symptoms they're
about what new symptoms they're experiencing and what do they think is
experiencing and what do they think is the cause. Ask about their expectations
the cause. Ask about their expectations for care. And most importantly, ask how
for care. And most importantly, ask how the changes are making them feel.
the changes are making them feel. >> We need to stay curious and
>> We need to stay curious and non-judgmental. We need to ask about
non-judgmental. We need to ask about what it's like growing older and truly
what it's like growing older and truly listen like Brandon said. Finally,
listen like Brandon said. Finally, we need to continue to do what we do
we need to continue to do what we do best as a CF community. We need to stay
best as a CF community. We need to stay ahead of the curve, which again involves
ahead of the curve, which again involves forming relationships with experts
forming relationships with experts beyond that core team.
beyond that core team. >> And to always remember the words of our
>> And to always remember the words of our dear friend of the CF community, Francis
dear friend of the CF community, Francis Collins. We have within our grasp the
Collins. We have within our grasp the ability to alleviate suffering and
ability to alleviate suffering and improve quality of life. That must be
improve quality of life. That must be our mission. Not just adding years, but
our mission. Not just adding years, but adding meaning to yours.
adding meaning to yours. So, let's keep the gears turning on
So, let's keep the gears turning on growing older with CF
