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Welcome everyone to the Becker's Healthcare podcast series.

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I'm Mariah Muhammad, writer and moderator with Becker's

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Healthcare. I'm thrilled to have with me today

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doctor Michael j Welsh, professor of internal medicine

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at the University of Iowa and Grace Lidget,

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a patient at University of Iowa Health Care.

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Thank you both for speaking with with me

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today, and welcome to the podcast. We're very

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excited to have you join us. Today, we'll

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be discussing a fascinating fascinating story about the

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treatment

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of cystic fibrosis,

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between doctor and patient.

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To get us started, doctor Welsh,

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I'll get started with you first. For those

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who are not familiar with this, can you

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explain what cystic fibrosis is and how it

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affects patients' lives?

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So cystic fibrosis

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is a genetic disease. It's inherited in autosomal

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recessive fashion.

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It's the most common,

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severe,

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life threatening disease in Caucasians, particularly of Northern

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European

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ancestry.

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It affects multiple different organs.

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It affects the pancreas.

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Destruction of the pancreas and intestinal problems

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used to

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be the major

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early

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claim on babies born with cystic fibrosis.

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Then,

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physicians and scientists developed

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pig,

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pancreas

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enzymes, and that helped with nutrition.

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But but for a long time, the lung

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disease is the major cause

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of suffering for people with cystic fibrosis.

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And there, the problem was

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infections that were really recalcitrant

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to treatment.

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Infections with many different organs. Pseudomonas aeruginosa

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is a common widely recognized

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organism as well as staph aureus.

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And that

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treatments had progressed

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for cystic fibrosis,

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and

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those consisted of

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antibiotics,

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consisted of attempts to dislodge the mucus with

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mists and saline

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administration

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and,

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pounding on people's backs.

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And that's where

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the situation was prior to,

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the recent developments,

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recent developments beginning

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a long time ago, but accelerating and it's

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a turning point with the discovery of the

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CF gene.

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Wonderful. Thank you so much for giving us

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that background.

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Grace, going to you, you were diagnosed

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with CF as a baby. What were some

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of the biggest challenges,

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you faced growing up with the disease before

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the therapies were available?

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When I was born, it was about it

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was 02/2004.

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And so

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I don't ever remember not having the therapies

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because as soon as I was actually old

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enough to know what was happening around me,

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I was

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doing two nebulizers a day, and I didn't

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have a vest yet because I wasn't old

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enough. It straps to you like a vest

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that you'd normally wear except for it inflates

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with air, and it shakes you

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very violently to try to break up that

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mucus so that way you can cough it

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up.

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So once I reached that age, I I

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had a vest readily available. I was born

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during a very fortunate time where I didn't

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have to live without these,

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specific therapies,

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like that.

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However, these modulator therapies,

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before those were avail available,

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I got sick quite often.

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I

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I remember having Pseudomonas

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three times in my life right when I

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was first diagnosed

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at 14 old,

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or yeah.

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Right when I was diagnosed at 14 old,

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once in fifth grade, and then once again

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in eighth grade.

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I was lucky enough that these didn't severely

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damage my lungs, but it was a consistent

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enough

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issue that I was at risk to become

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chronic with it.

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But it was only a couple years after

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my last diagnosis with Pseudomonas that these modulators

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came out. My lung function

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skyrocketed,

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which was such a blessing because I am

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a long distance runner, and so

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it really helped me out to pursue that

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passion, something that I was already actively pursuing,

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but now could comfortably

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continue to do so with a little bit

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of support.

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Yeah. Yeah. Absolutely. Well, that's that's very good

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to hear. And,

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doctor Walsh, you've been studying CF for more

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than forty years.

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What drew you to the this disease early

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in your career, and, also, what kept you

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focused on it for so long?

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So my first exposure to cystic fibrosis was

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when I was

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a medical student, junior medical student here at

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the University of Iowa.

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And, at that time, I I was in

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the pediatric clinic, and I met a young

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girl, probably seven or eight years old.

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And,

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she was struggling to breathe. I could see

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her using her accessory muscles, speaking in short

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sentences,

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just obviously having difficulty breathing, frequently coughing, a

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really harsh cough.

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And I learned about all the things she

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couldn't do, and I learned

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how short her life would be. That made

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a made an indelible impression.