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NACFC 2025 | A Breath of Fresh Age: Redefining Growing Older With Cystic Fibrosis

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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.

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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

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

growing older with CF >> together,

>> together, >> side by side,

>> side by side, >> as one team.

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YouTube Transcript:NACFC 2025 | A Breath of Fresh Age: Redefining Growing Older With Cystic Fibrosis

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NACFC 2025 | A Breath of Fresh Age: Redefining Growing Older With Cystic Fibrosis