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Presented by the El Paso County Medical Society and hosted by Kathrin Berg.

Cystic fibrosis is an inherited, complex disease affecting the lungs and the digestive system, but thanks to research and innovative treatments, children born with cystic fibrosis today have opportunities that were unimaginable even just a generation ago.

We'll talk to a pediatric pulmonologist and a gastroenterologist working with a dedicated team that's focused on the needs of patients in this borderland region.

Today's program is underwritten by the El Paso Children's Hospital, and we also want to thank the El Paso County Medical Society for bringing this program to you.

For over 29 years.

I'm Kathrin Berg, and this is the El Paso physician.

Neither the El Paso County Medical Society, its members nor PBS El Paso shall be responsible for the views, opinions or facts expressed by the panelists on this television program.

Please consult your doctor.

Thanks again for joining us.

I'm Kathrin Berg and tonight's topic is advances in medical management of cystic fibrosis.

And with us this evening we have a veteran who joined us when we were back in Covid days.

And we were talking about children, how to handle all that.

That is Doctor Endy Dominguez.

And he is the board certified pediatric pulmonologist and chief of pediatric department here at the El Paso Children's Hospital.

And we have also Doctor Denease Francis, who is a board certified pediatric gastroenterologist and Texas Tech physicians of El Paso.

Thank you so much for joining us this evening.

And, this is a unique topic in that there are so many different disciplines that go around with the treatment of cystic fibrosis.

So, Doctor Dominguez, you've been on the program once before and it was great because you're like, hey, I remember it was a different location.

This is what we did.

So you kind of remember the format of that.

But tell the audience who you are.

Yes, but what is it that you do all day, every day in this area of cystic fibrosis?

Yeah.

Well, again, I'm Endy Dominguez I'm born and raised in Ciudad Juarez now working here in El Paso.

Very happy to serve our community, our borderland.

So I'm a pediatric pulmonologist.

I what I do daily is take care of kids with, lung issues, respiratory problems.

When we talk specifically with cystic fibrosis, we work in conjunction with El Paso Children's Hospital, Texas Tech and, multidisciplinary, clinic.

We have Doctor Francis, we have our RTs we have our dietician nutritionist to have a comprehensive care of patients with cystic fibrosis here in the community.

And I got a nice tour I'm going to calling a marketing guy was not in the room.

So it's not fun.

But anyway, I got a nice tour right before we started this.

And the different pods that you have, I got to, meet, Doctor Francis for a second, and she's like, hey, I'm busy.

Let me return a phone call and I'll be right there.

But you do have a lot of varied areas just in this center itself.

So we'll talk about that as time goes on as well.

And, Doctor Francis, how about you?

I know your gastroenterology, all day, every day.

Yeah.

So I'm a pediatric gastroenterologist.

I am from New York, but I've been in El Paso for almost nine years now.

And all day, every day, I tend to see a wide variety of patients with different stomach, gut issues.

And then specifically for cystic fibrosis, as Endy said, we work really closely together.

Seeing kids with, with cystic fibrosis.

And we work with our dietitians, we work with our therapists, counselors.

And our focus is trying to make sure the kids in this region are access and appropriate care, that they don't have to travel outside, to receive the care, which is, I think, one of the most exciting things about our center.

And, and being on this program to promote us, and make sure that the kids know that they're we're here for them.

And I think it's super important to mention, we talked about this a little bit before to El Paso Children's Hospital cystic fibrosis affiliated center.

So this is affiliated center with the Cystic Fibrosis Foundation.

And why is that so important I understand that that was received this year.

Is that correct.

Why why is that such a big deal?

That's that's correct.

Yeah.

It is a milestone for the city for years and years and years, patients with cystic fibrosis were not able to have a multidisciplinary care.

And it's important to mention that the guidance based on the Cystic Fibrosis Foundation mentioned that the patients with this condition, they need to be treated in specialized centers.

So we came a long way.

Now is a big milestone.

Now we have the ability to care for patients with cystic fibrosis here in our borderland.

They don't need to drive ten hours four hours to seek care.

So this is a great, great, great, achievement that we have here in El Paso.

We're very, very proud.

It took us, six years to have these.

Granted, these we started in 2019 with planning, finally in 2000, 20th March.

Well, we have Covid.

We had our first chloride test, which is what we used to diagnose cystic fibrosis here at El Paso Children's Hospital.

And we create a timeline, and we start with only CF clinic in October of 2022.

And working and with the addition of our C cystic fibrosis coordinator.

Now we have a geneticist, we have therapies.

It's a big group.

And finally we were achieve that.

Granted the affiliated center in conjunction with with the help with Dallas.

And so we're very excited, very excited.

Love that.

And I have here and I'm going to mention them here just because it's sometimes easier because I have the list.

We have the therapies.

So the team members consists of a nutritionist, a social worker, a physical therapist, a respiratory therapist, a behavioral.

And I do want to talk about that to a behavioral therapist.

Genetic counselor and a registered nurse.

So that's the team that works with cystic fibrosis.

And then here too, at the multiple multi-specialty center, there's a gamut of other specialists too.

And so the idea is under one roof at one time, one appointment, a lot of things can be seen.

And Doctor Francis, if you don't mind, talk about how you, throughout your days and weeks work with these different team members here at the center.

Yeah.

So we being here is awesome for me.

I think it's awesome for the patients.

So I am able to coordinate visits, for patients who need to see multiple subspecialists, like we're able to say, hey, like, I can see you on this day because you're seeing this other provider on this day, and it makes it easier for families because they're able to come in one day and see multiple providers if they need be.

And then for me personally, it's helpful, especially if I see kids with like weight issues.

And I know that they're seeing cardiology in three weeks.

I can look at weights at that visit to monitor, we're able to do a multidisciplinary clinic, and then I do, several multidisciplinary clinics together.

Which I think especially for your kids who are have like, complex, health needs, anything that you can do to minimize travel time, or minimize like times that you're sitting in an office is always best.

It's easier for families.

It's easier on the kids.

And I think that's one of the nice things about the multi-specialty center, is that we are able to achieve that.

And, you know, as, as we've all mentioned, anything that makes it easier for our community is something that we are always going to champion.

So just taking this tour here earlier, it's like everything really is built for children and not just like little, little ones, but all the way up through the teen year years.

And it's really nice because there is an anxiety, there is an inherent like, oh, the doctor, oh, the hospital, oh, the medical clinic.

And just looking around and naturally, you want to put people at ease.

And that is that is definitely evident everywhere in every when every one of the pods that we went to, even joking about all the different models that we have.

So you can explain to children and the families what it is that that's going on.

So yay on that.

So I want to, kind of start and it may seem very basic to you all because this is what you do all day, every day, but explain what cystic fibrosis is.

And again, it's an inherited disease.

But you did say earlier too, that sometimes people aren't diagnosed until way later in life.

So explain what it is physiologically.

And then we kind of can take it from there and starting from pediatrics and then going forward.

So cystic fibrosis genetic condition that we call that is a recessive condition, meaning we may carry a gene that will affect the problem.

You will not know.

But when you have this combination of mom and dad give you both, you know, changes in that gene, then you will have cystic fibrosis.

So cystic fibrosis is, a problem that is in a protein that we call Cftr.

That's the name of that protein in particular.

And what it does that that protein is help us to mobilize bicarbonate chloride and sodium.

This is very important for our health because when we have a lack of function of this protein, what is going to happen, for example, in the lungs we will start having thick, very thick mucus and secretion, which will help to the bacteria to overgrow and create infections in the respiratory tract.

But not only that will affect other systems.

And Doctor Francis will talk about it.

It will affect the pancreas and the gut.

So it is a condition that it can be very severe if it is not treated, on time.

And so speaking about on time, it's a genetic.

So when and how is a diagnosis made?

Doctor Francis I'll start with you.

So screenings right after birth I understand.

Right.

So we have multiple times.

Yes.

Okay.

So we have the newborn screen but it's very state specific.

That's one of the things that's really important to know.

So depending on which state you were in they may not be testing for all of those mutations.

And so you might miss, a diagnosis.

And so typically what happens with us, especially now that we're an affiliate center, if there's an abnormal newborn screen, that it gets routed to us and then, Doctor Dominguez usually will start off the process and we'll see the patient in the clinic, get a sweat.

So a chloride test to confirm or and then they may need genetic testing.

And then they'll get genetic testing as well.

And that's kind of how we start the process for, diagnosis.

And then for older patients, definitely, if we have a patient with known cystic fibrosis and the parents are pregnant, and that's something we are aware of.

And so we are hyper vigilant, that there's a risk that that newborn may have cystic fibrosis.

And then with older patients, especially if they're being a naive diagnosis like some older kids, who didn't was missed on the newborn screen initially.

And then came in with certain symptoms and he was able to get sweat chloride testing done and genetic testing done.

And was able to make those diagnosis.

So you hit something perfect there that I like to expand on.

So if a diagnosis was missed, the symptoms, the specific system I know you're talking about lungs, breathing, etc., what would parents be looking for?

And again I'm thinking if it's missed at birth, what's the span of age that you all find is most often they come in when they're a year old, a couple of years old, a couple of months old.

Give me some case studies of when you're like, we should test for cystic fibrosis.

And so there's when we have a would if patient is born with cystic fibrosis and he has the condition, let's say he's missed, for any reason, we came negative and then you were screened.

We were not flagged.

We don't know about it.

We cannot test the patient.

They will not start presenting symptoms until roughly between 4 to 6 months of age.

Oh, wow.

No, this is important to mention.

It depends of the classification or the class of the mutation because they're severities for a cystic fibrosis, when we have severe cases, typically they will present in the first year of life with failure to thrive.

They will have recurrent infections, especially pneumonia.

Sometimes they can have ear infections and they can get dehydrated really, really easily.

So that's or the signs and symptoms that we will trigger right away for us to to think about cystic fibrosis.

And I will tell you that at least in the pulmonary world, if we think about cystic fibrosis, we do that.

We, we we just we don't we don't think about it.

We do this chloride.

Okay.

So this test, say it, say slowly this this sweat chloride, this sweat chloride test.

That's correct.

Okay.

So how is that?

What is that?

How is it performed?

I guess that's more the question.

So if you're saying this test, it is a matter of a swab.

Is it a matter of what is it that requires this screening or this test.

So that test is basically what it says.

It's a test of the sweat.

So what we thought you were saying?

My thought is sweat chloride.

Yes.

All right.

So what we test is the amount of chloride in the sweat.

Interesting.

There's a certain amount of of chloride that is normal in your sweat.

Okay.

What we check is we grab the babies and, to Children's Hospital, put them on a treadmill for a while.

Well, that's the old ways.

That funny enough, but we don't do that anymore.

Okay?

Yeah, we just yell at them until they're sweaty.

Know what the reality is?

That we do something, that we do something, electrophoresis and pillow capping, which is basically a gel that we wrap around their arms so we can stimulate the sweat glands that we're going to put a little bit of electric stimuli.

It's not painful, not harmful.

Then after the stimulation of the sweat glands we remove it and we put something in there to collect that the sweat.

And then we just put it in a machine.

And the machine tell us sweat, the chloride in the sweat is normal or is elevated.

If it's elevated we have a problem.

And that's what we need to start working yourself into the genetic process.

Okay.

All right.

So now we're looking at someone who's diagnosed.

It's a it's a brand brand new baby.

So we're looking at the first several days of life.

Mom is trying to breastfeed.

Nutritionally.

So what is happening as far as receiving the nutrition that the baby needs?

When are you on alert?

Like, well, maybe they're not thriving and failure to thrive is so vague because I hear that a lot of pediatric type verbiage I will failure to thrive, but that could mean so many things.

I'm glad that you brought that up very specifically.

Because what might that mean with someone who has cystic fibrosis?

Again, we're looking at lungs.

We're looking at not getting the nutrients.

And how do the first few days of this diagnosis, if there are symptoms being shown, how do how do you all deal with that?

How do the parents deal with that?

And Doctor Francis, I'm I'm looking at you just because Doctor Dominguez just had a turn.

But again, you guys can pop with each other on the nutrition perspective.

One of the big things that we.

There is a lot of GI manifestations with cystic fibrosis.

And one of the biggest ones that we look at is, pancreatic insufficiency.

So that Cftr, receptor is in the pancreas and it produces bicarb, same as in the lungs.

But when, it's not functional, the pancreas is still producing digestive enzymes.

And that causes inflammation and destruction of the ducts in the pancreas.

Usually that starts that starts in utero and it progresses a bit.

It progresses after birth.

So typically the thing we're concerned about with is pancreatic insufficiency, which is that those the pancreas is not able to secrete digestive enzymes into the small intestine to help, digest food, primarily fats.

Some patients with cystic fibrosis will have pancreatic insufficiency at birth.

About 50%.

And then some will progress later on.

And it's about 75 to 85% that range of patients with cystic fibrosis who will have pancreatic insufficiency.

So, from a nutrition perspective, in that initial, first couple days of life, if the patient has pancreatic insufficiency, the concern is that they're going to be feeding.

But when you look at the growth chart, you look at the weight.

The weight is not going to be going up at the rate that we would like.

Or you might see that there percentiles are dropping.

And so that's what we would call growth faltering is the new term.

But that's what we're looking for on the growth curve.

The other thing that patients may have is that they may have, oily or like fatty, poop stools, because they're not digesting it.

So they're passing it, and their stools.

And so you might see like oil in the stools.

And that is a concern for pancreatic insufficiency.

And what we will typically do from my perspective is that when they come in, when we see them, I will get what's called a fecal analysis, which is a test to check the pancreatic function.

And if that number is low, then what we have to do start thinking about is giving them those enzymes to replace what the pancreas is not able to do, so that they're able to appropriately, digest fats.

And so from a nutrition perspective, that's what I'm looking for in the first couple days of life, first couple weeks and then even ongoing.

That's what we look for.

The other thing that we do is supplement salt, because that's the issue.

So we give them salt.

With older kids, they're able to get salt in regular foods, but babies aren't able.

They're not eating regular foods.

So we do supplement with salt.

And we also supplement with, some vitamins because we need fat for, absorption of certain vitamins.

Vitamins A, D, and k. And so we give those vitamins, as well, because we know that they're not going to be able to appropriately, absorb those.

And then from a pond perspective, there's a lot that that Endy does as well.

And I'll let him I'll defer to him.

I'm all of those.

I'm going to I'm going to ask you one quick question, because I have on here I did some research on pancreatic enzyme replacement therapy.

So is that what you were talking about.

So pancreatic.

Yeah.

So given the enzyme is pancreatic enzyme replacement therapy okay.

And that specific enzyme that we're given is something called light pieces.

What we use for fat digestion.

Okay.

And it's, it's pretty, it's, pretty cool that we're able to give something that the pancreas is not making.

And then there is actually a newer, several years ago, some kids with, cystic fibrosis, they have a lot of issues with nutrition.

Right?

So they don't grow sometimes they need tube feeds.

And a company came out with this, super cool cartridge that contains, like, paste.

And for kids who are tube fed, the feed is actually able to run through the cartridge.

It's digested by the lipase.

I don't know, pellets in the cartridge.

And then it goes into the patient.

And so that actually helps a lot with kids who are too fed.

They don't have to wake up to take, enzymes.

Because we need to give enzymes prior to, to food ingestion.

And so it really helps.

So that's pancreatic enzyme replacement therapy.

So in the opening, like, I know, over the last decade and I know we were talking about since Covid, which is already six years ago, right.

I'm thinking the last decades, not that long ago.

And the talking about this, how long has this been in place, this way of replacing enzymes?

Has this been in place for decades or for decades?

Okay.

For decades for sure.

For sure.

The release arm is new.

That's within the past decade, I would say.

I don't even know how long it's been, but that's a newer one.

But in terms of like the enzyme replacement therapy that's been around for for decades, it's effective.

And so, you know, fix what's not broken.

We can unfortunately give pancreatic function back with the, with the with an asterisk with and I know you looked at me with the little little star and so not fully yet but it's potential.

So it's like on the horizon and we'll talk about modulators but yeah, it's like, oh yeah, potentially modular down here.

Okay.

So I do, I do want to.

And you bounced it perfectly to Doctor Dominguez.

So Doctor Dominguez, if we can take it back to, you know, Doctor Dominguez has all kinds of pulmonary, stuff he can talk about.

So in that area of pulmonology and just lung functions, etc., diagnosis, symptoms, thriving, etc., as they're young, kind of take the same approach of answer but go into lung function and pulmonology.

So in terms of the lung one of the critical problems or what we need to have more in consideration is the ability of the patient to clear up the secretions.

We all normal, we produce certain amount of mucus anymore without any problem because we have a small like hair, like, that we call cilia.

Is hair very small, tiny that let us move all that mucus up and we don't notice.

Well, sometimes we swallow it and then and then we're fine.

We're able to move our mucus.

And that's why we don't get sick.

Because that mucus moves around when we have cystic fibrosis.

One of the main issues again with the bicarbonate, the chloride.

But in this case, especially the lack of control of the sodium, we have, the that cell lining or that lining that we have, it is protected by something that we call airway surface, liquid, meaning imagine that there is in a pool and you have water, and then you have all this helium moving around without any problem moving the mucus around, or if there a bacteria move it up, and then you can swallow it, spit it out, get it out of the way.

When the sodium is, start pushing into the cell more than normal.

That pool dries out.

That's that's a great way to explain it.

So it dries out.

And the cilia or the hairs that we have to move around the mucus die, and then the mucus doesn't go anywhere.

Start having a very thick secretion, and then the bacteria start reproducing without any problem.

And then the body try to compensate that by sending what we call white blood cells, which are or policemen's of their blood, of the blood supply they try to fight they cannot they are overwhelmed.

They die.

And when these white blood cells that we call neutrophils die, they produce, they they all day.

Everything that is inside that cell gets into that area and make more sticky that, that, that, that mucus.

So the main problem with the patient which cystic fibrosis pulmonary wise is that they're not able to clear the secretions.

And at some point they will have pneumonia.

You cannot cough it out anything, even if you cough as hard as you can.

It's almost like rock cement there.

So that's why it's important to do their proper treatment for, for for these patients.

So now that we're there, we have a very bad situation.

You are infected.

There's an infection there.

Everything's dry.

How do you treat that to get back to clear once again.

Like how how is that treatment put together.

They come in they you put them on.

What kind of treatment for them to to how do you how do you get the secrete out.

Yes.

So that's the so we we start with a regimen that we call airway clearance regimen.

Okay.

In patients with cystic fibrosis what we do is we use one of that's something that we called Darkness Alpha.

That is a, inhale or nebulizer treatment that we basically go to that mucus and chop down all the debris.

It's all the white blood cells release, so they chop.

So that way is not too sticky.

That's one thing that we do.

Second thing that we do is we use a medication that we call is a bronchodilator.

That is nebulizer that we call albuterol because have the ability not only to open a little bit the windpipe, but activate a little bit the the cilia and the movements of those hairs.

The other part that is very important when you also inhale we use actually sodium chloride.

In in a high concentration.

So now we have sodium in the, in the airway.

And that sodium are going to pull back the water into the pool to help that mucus to be, again, more loose and easy to cough out.

So what we do is we have in place that we have something that we call as a chest best.

It's a base that vibrate.

Okay.

The fancy warriors, high frequency chest wall oscillator.

But I think the the chest best what it does.

You put it on and start vibrating, and then you're having your nebulizer so we can check things out, like move, move everything around, and then the patient will be able to cough out all that mucus and have relief.

And typically when they're not sick, we do that regimen twice a day, morning and night.

We do 2 or 3 types of mobilizations, and we do the, the, the, you know, the check in with the vest and that there's cause it's a beautiful day.

And and when they're sick we just do that more often every four, every six hours.

But that's how you we you.

Well, we still use that.

And that's what we typically treat patients with cystic fibrosis in the pulmonary, realm.

Now that's only one part because the other problem is the recurrent infections.

And that's when we need to take antibiotics in consideration.

And some of the antibiotics are actually inhaled the sometimes because of that chronicity or that inability of the mucus to move.

We have bacteria.

They stay there forever.

The same time bacteria.

And what we need to do is start to do daily Inhaled antibiotics, to these patients.

So that's typically what we do.

and I was thinking antibiotics.

And this is just me and everything everyday life, it's going to mess up your gut.

So for sure that all the yeah for oral antibiotics.

Definitely.

And then the inhaled antibiotics less.

But for if they're doing oral antibiotics, they're definitely going to mess up the gut.

So patients with cystic fibrosis are at risk of, like higher risk of which is usually because you've wiped out other bacteria and that is allowed to proliferate.

We manage it, right, because you need the antibiotics for the lung component.

And so if we get CNS, we can treat it.

If there's some gut overgrowth, we can do some probiotics and rebalance thing depending on like what the patient's status is.

So it's one of those things where it's the gut is he's going to love.

The gut is not as important in this single scenario and every other scenario.

The gut is definitely very important.

And I get that.

I love kind of the I'd love to just spend a little bit of time on this for people who don't know antibiotics and you've got prebiotics, you got post by Odyssey, you have all this.

So it is it's something you hear about all the time.

So for people who are not familiar with that, why is it that antibiotics might cause issues in the gut?

Why is it that you're trying to do with that?

And and what are probiotics?

So and I know I really do loaded question without any great answer because these are terms we hear all the time.

But we don't understand how they work together.

So antibiotics will they're designed to kill off and a bacterial infection.

But depending on how broad spectrum of an antibiotic you're using, some broad spectrum means it kills a lot of different bacteria.

You're probably killing off some helpful bacteria in the GI track.

And when you kill off helpful bacteria.

I don't want to say bad, but things like, see, this is they kind of grow, right?

So they have lots of.

They go a little crazy.

And that can give you, diarrhea, can give you issues.

And we then use another antibiotic to kill that bacteria.

And then probiotic is probiotic.

Probiotic.

That's a very loaded question because there is 1,000,001 formulations of probiotics in probiotics.

And there is some research on some kinds, no research and other kinds, for like, infectious diarrhea.

There's research on one specific kind called Saccharomyces baladi that shows that it's helpful.

And so I would recommend when kids have infection, I recommend that.

But a lot of it is well, you know, it's, it's, it's that it's, it's not an exact science.

It's not an exact science, but it is helpful in certain situations.

But in terms of to tell you like one formulation versus another formulation, should you take probiotics versus probiotics?

Do you need a combination of both?

It's really patient specific.

And there is no great answer in terms of what combination you need.

Right.

And I'm just thinking too.

And we'll get to that question later.

But later in life when people with cystic fibrosis are treating themselves now, you know, again, there's cost to medications.

There's over-the-counter we'll talk about in a little bit.

But I kind of want a floating in your brain for a while.

We talked about, cystic fibrosis tr modular medications.

So I like to kind of go into that for a moment.

And what are they and what do they do.

So this is a game changer for patients with cystic fibrosis, no doubt about it.

And talking about the last 1015 years he has been a revolution.

And the way that we treat cystic fibrosis.

So the series of TR modulators are medications.

They have the ability to help us to potentiate, okay, the function of the protein that we still have there, and also to make a correction so we can have more proteins in the membrane of the cell.

Meaning let me explain that right.

The way that the proteins, are manufactured is basically are in the nucleus of a cell.

So they are produced, they go up into the membrane of the cell in they start, allowing the chloride, the sodium, the bicarbonate to move freely in or out of the cell.

When we have cystic fibrosis there's many things that it can happen.

Number one, it may not go out from the nucleus.

That's bad because we will not have any protein in those patients will have not too good of a prognosis.

Then you have other type of classification that they are produce.

But they don't reach the membrane, then have other type of problems where they reach the membrane.

But they don't that that protein, they don't open, they don't open the gate.

So the the chloride and the bicarb, they're stuck there and they don't go where they need to go.

So these medications, what they do one potentiate that protein, that channel.

And we'll tell him, yes, we can open the, the the gate.

And then you open the gate.

And the other medication is a corrector.

So as soon as we go out from the nucleus, go fix like a Lego or you don't have this piece, you put the lego then is able to reach that area.

And that's where we have this type of medication that basically potentiate and correct the malfunction of that protein.

And now we have patients that basically they don't get into the hospital anymore because when I train, I used to have my patients daily rounds with, you know, asthma or other stuff or cystic fibrosis there.

I don't I don't have patients admitted in the hospital once a year, probably one not everywhere like one, probably one patient is, and maybe more because with this new medication, we basically are fixing the main issue with cystic fibrosis.

Okay.

And how often do they take the medication daily.

So it's a daily medication administered some meds is oral medication.

So for example for babies there's granules.

And it's very easy.

You do it with for example applesauce or peanut butter.

Different types of fatty foods.

It has to be fatty foods because they absorb better okay.

And and that's how we do in a little bit bigger older patients.

You can eat tablets, you swallow, one tablet in the morning and then one tablet at night time in.

One of the beauty of these medications is when we talk about pulmonary function, we we have the ability to measure that with something that we call, pulmonary function tests.

And we, we measure something that we call a force exhale, capacity in one second.

How how good you are to blow out your air.

Yeah.

So typical in patients they're affected with cystic fibrosis.

That number is going to be low.

But we have seen and and this is important because when we reach a certain percentage how low it is.

This means that the lung is very bad.

And then in the old days they need to go and have a transplant.

Now we are seeing as soon as we start these Cftr modulators, we give it and then our, our, our pulmonary function skyrocket to normalization to normal.

And Doctor Francis was laughing a little bit about the pancreas because we cannot do that yet with pancreas we we always joke about, in our clinic that we say that at some point is going to be more, GI condition because pulmonary wise, we're we're being very lucky about it, but not yet with the pancreas because now it looks like one of the medications may will help if we do it a little bit soon.

Yeah.

She's so cute.

She's like yeah.

So I always the gut is very important.

More important, not more important.

Not at all.

Because yeah.

And I'm thinking too.

So there's lung functions but I think often in my head I've always said it's mainly a lung function.

And then digestive is always comes second in my head.

That's not the case.

But that's why I'm learning today.

Right.

So we're looking that even with these TR modular medications lung lung lung.

But you're still looking at the condition of cystic fibrosis.

That is affecting the digestive system.

So perfect transition there is taking it now to the pancreas even with these medications there.

Do you see number one has it been beneficial in the in the GI tract for sure.

Right.

Do you see something in the future that's coming in that might help out with the pancreatic enzymes?

So for the modulators it is it's awesome.

Right.

So these kids are and their life is better.

And I definitely notice with kids on modulators, weight gain is significantly, better.

When your lungs aren't working, you're spending a ton of energy.

And when your lungs are better nutritionally, you need less.

And so that definitely helps from a nutrition perspective.

From a pancreas, pancreatic function perspective.

Potentially.

Right.

Like it's not quite there.

I feel like every year when we go to the, cystic fibrosis conference, there is some, discussion of looking at, fecal elastics with that measure of pancreatic function and checking it on modulators and potentially maybe seeing some changes.

But there's nothing very conclusive as of yet.

But one of the great things about cystic fibrosis is that it has this very dedicated group of parents, researchers, providers, dietitians, counselors.

There's this huge group of people whose sole focus is on improving this disease and, the lives of patients with this disease.

So right now, the lungs is the main focus because it was the there is a GI component, but the lungs was always the big issue.

I think as that is health more and more, there's definitely going to be more research done on looking at things that will improve, pancreatic function.

So I think it's down the line and it's, it's definitely I think it's, it's, it's a thought in the back of everyone's mind, because we want their nutrition to be just as good as their lungs.

Yeah.

I sir, and speaking of lungs and lung function, I always think about exercise and, you know, once kids get a certain age, they're just they're all over the place.

And you were talking about how belabored breathing can be and how much energy that does take in general.

What is the advice that you give to parents you give to families?

Either one of you, but I'm thinking too, with lungs.

Go out, run a little bit, exercise, participate in team sports.

What is it that you look after or for?

Before giving that advice to parents?

Because everybody's on a different level of where they are in their treatment and in their disease, I'm imagining.

So just in general, how did those conversations occur?

I think you you pointed out really nicely.

Depends on the patient.

Right.

If I have a patient who has that is lucky enough to his mutations and is candidate to have these Cftr modulators, it will be easier to have, a plan for them to exercise.

One of our patients actually here in El Paso, the he runs, he he just runs, he plays football.

He's a high level athlete.

And he's great.

I think I think it is it is important to understand that these medications are, you know, game changing.

However, when we have patients that they don't have the ability to have these therapies because it doesn't work for and for, not for everyone, depending on your genetic composition, then you have to create a certain environment.

Make sure, for example, if he's out in the open, it's easier versus if you go to the physical activity, in a concealed the environment.

When you have a lot of kids that will be sick.

Why?

Because it's normal in in an in toddler years, you have kids that will have a viral infection that's breathing better.

That's common.

That's how we get our immunity system running.

So those then with those parts, we need to tailor what we need to do because they need to do physical activity.

They need to exercise because the exercise is not only for the lungs, it's also for the bone structure be able to to maintain muscle.

So so exercise home physical activity is extremely important not only in cystic fibrosis for mainly any condition.

Yeah.

Yeah.

And so you hit on something that was interesting too.

Depends on their genetic composition.

With the cystic fibrosis and beginning of program.

This is an inherited disease.

That's correct.

So on that note and again it's different with every patient.

I completely understand that.

How do I so I know we have now in town a geneticist.

How is that test done on different patients on what the genetic composition is?

And then with the knowledge of how you treat cystic fibrosis with their particular composition.

So we typically we, we ask, Michelle Kosar, genetic counselor, and then doctor who's our geneticist.

We ask them when we need to do the test.

They come and they do a simple swab and they send it.

And then we have the result and we see what type of mutations this patient have.

So we have to understand that although it's one gene who costs 60 fibros That gene can have roughly around 2000 mutations that they will cause malfunction of that protein or that channel.

So the medications that we have, not will not correct.

They will not have the pieces of Lego to correct all the genetic mutation, and they will not have the ability to potentially do, help them to open that gate to all that.

It depends how that Lego was formed.

So we're not yet in that regard.

But, this year or last year, we have a new medication.

So we start with one protein share.

Then we have with a protein sharing or for one of the most common, mutation, which is the delta 508 Then after that we start having another medication for we with one correct or one protein share.

And now we have one with two correct or one potential.

And then a new one.

So things are moving so fast.

However what is coming in the future is gene therapy.

Yes.

And that's where we're waiting, to see how because when we have gene therapy, that's going to be another level of, of of therapy and hopefully look for the cure.

So I understand gene therapy is in Europe there's quite a bit going on with gene therapies.

And I know in America too, it's it's FDA, it's and I, it's, it's the way our system works.

And I think it's great.

But also it's almost like experimentations are happening.

So I love that you brought that up with gene therapies.

Where are we where are we now?

And let's let's just use that decade.

I like using the word decade.

So where are we now with gene therapy versus a decade ago.

And now in both of your heads, just jump up five years from now.

Let's not wait a whole decade to go further.

Five years from now, what do you think?

Gene therapy is going to produce in the world of cystic fibrosis?

And how are you both want to answer that?

Feel free.

But I'm going to say I think sickle cell has really helped with gene therapy.

And then I'll pivot to you.

I think that has really been they've achieved a lot with gene therapy with sickle cell disease.

Like it is super cool, very fascinating.

And I think that is helping a lot in terms of what other diseases are.

All right.

Diseases are able to do, with, with gene therapy and I would say from ten years ago to now, you're in a very different place with gene therapy.

Because now they're, they're treating kids with sickle cell, and they are they're like, curing them.

By truly using the word cure.

I felt like that wasn't a where you could use the sickle cell use in the word cure.

There has been some really cool, out of chop, like, some really cool articles that have come out and they're curing kids with sickle cell with gene therapy.

And so in cystic fibrosis ten years ago to where we are now, it's hugely different in terms of where we're going to be in five years.

It is difficult to say that because it all depends.

So you can't get sued because yeah, there's there's a disclaimer I know there.

So this is best guess, best educated My best guess probably is that we'll have good answer.

Like first of all is going to work or not.

I think we will know sooner.

And then we just need to tweak how and when.

But I think it's a very bright future.

But to go back and answer where we were and ten years ago or that decade ago, I will tell you this, yesterday we had we run our clinic, yesterday we have our, our residents and and when they rotate, I like to show them when you go this year foundation website, in the medical bar when you see something that we call drug pipeline, when you see exactly where are we, where is the pipeline, what are we doing right now?

So when I was a fellow, when you go to that website, you see pipeline of the anti-inflammatories, the pancreatic cancer times, you start seeing 1 or 2 approved modulators and a lot of them that were coming to start treating you.

Can I use them?

There is and it is, you know, depend on the stage that you are you can use or not the medication.

Now you go to that website, you have you when you go to a Cftr pipeline, you have a bunch of them.

But not only that, now I have a pipeline of different laboratories working on gene therapy that we didn't have before, and this is in the last 2 or 3 years that we have now that pipeline and is amazing.

And and I like it because it is so open.

And when there's a way that you can click a button and see the discontinue, treatments or, or, or studies, any open and show you how much we have.

Also if people say failed but I don't think failed like we get more close to the answer.

Right.

And and it's amazing to see the progress of that.

And the residents like to to see that and yeah.

So and this is one of those things too, we're in about ten minutes before the show wraps up, and I've got my questions here.

I want to know, because this is always, to me, the sweet spot of the program.

Right.

We're into it now.

You're thinking about things that I want to tell the audiences.

I want to tell the audience that Doctor Francis, is there something that we haven't covered yet in the world of gastroenterology or anything else that is related to what we're talking about, that you're excited about, that?

You know, that these things are going to be better in the future and or what we've been able to do in the most recent past.

And it could be related.

It could not be related what we've been talking about.

But in general, and I'm going to ask you the exact same question, because this is where the excitement happens, right?

You see, kids all day, every day.

You see the families, you interact.

There are things that you learn.

There are case studies.

Sometimes these are stories.

And this is this is the golden part.

I think I'm excited about I think I'm really excited about what we've achieved.

I think that's the first thing I'm really excited about.

And, and this is a credit to Endy, who really worked very, very hard, to get us to where we are now.

I, I would say Doctor Trumper had a dream to start, and, Endy came with that same dream and was able to, like, realize it, which I think is really important.

And so I'm excited that from where we started, that this is where we are now, where like, like we like, did all of this work and it there was a reward, which I'm excited about.

I'm excited for our community.

I'm excited for what we're going to be able to do, for the patients in this community from a treatment perspective, but also from a research perspective.

We live in a community where we're not as well represented in research, across all disease spectrum.

And I think having this affiliate center here really opens up what we're able to learn about cystic fibrosis in Hispanic patients and what we're able to achieve in terms of like treating them and I think that's really exciting.

I'm excited about our team.

We have a really awesome group of people.

That we work with.

And while it's the two of us, here today, definitely we don't do this, without the mass support of the people we work with.

And I'm excited for for our team and what we're going to be able to do.

And I'm excited for me because this is awesome for me.

And it's fun.

And I'm learning a lot.

Every day I learn more.

And so, yeah, I'm excited for for all of this.

You know, I think it's, I think it's been really amazing and I think it's cool.

And I love I'm going to talk about the team again because with with working with you, team members consist of the nutritionist, social worker, physical therapist, respiratory therapist, behavioral therapist, genetic counselor and registered nurse.

And this center in and of itself, we've got neurology, nephrology.

I mean, there's there's all kinds of things, again, multidisciplinary specialties, that all kind of intertwine with each other.

And I love the excitement that you have in there, too.

And I remember interviewing Doctor Trumper.

I remember like, he was an excitable guy, too, like he would get on since like, oh, oh, let me just tell you, it's been a while though, but I when you brought up that, I mean, like, I remember that guy, I remember him.

So, doctor, Dominguez, let's talk about, what you may have been thinking about prior to actually going on the air tonight.

Of talking about this evening when they asked you to be on this program, you're like, think.

Okay, I'm going to make sure that the world knows blank.

I really want the borderland to know that we have, accredited Affiliate Center.

I think that's that's the that's the the dream that a lot of people and patients and parents, they had, wish not to drive long hours, have the ability to connect with our physician right here at home.

If their daughter or son were sick.

I think that's that's that's the message that that that I really want the community to to know.

We're doing several multidisciplinary clinics, but this one specifically, obviously, is the in our heart because that's one of our conditions that we treat.

But as I, Denease mentioned, the support that we have from from El Paso Children's Hospital, we, ourselves as physicians from Texas Tec Their support of our coordinator, Mitch the team, you know, is, is, we're very grateful to have the ability to give, not only a standard of care above a standard of care for patients with cystic fibrosis.

What they deserve, they don't need to go to any other place to look for it.

And, and again I'm very happy.

When we were talking, before we started the show, when I came here in 2019, we, I create in my head a pipeline of service that we need here in the community were five pipelines.

One of them was the cystic Fibrosis clinic.

And when I talked to Denease, she never hesitated.

She say yes.

And then we start running.

And I think without the support of Denease and the team we have, it will be extremely difficult, probably will not be able to achieve this type of, success that we have now with a cystic fibrosis affiliate center.

There's something I kind of want to to end on.

On a very positive note, both of you have pediatric, etc., etc.

as the beginning of your title.

Now we're looking at and I sheepishly asked right before the program, like literally before the camera went on, it's like, what is the lifespan expectancy?

Is it any different then and then you're like, now?

Well, it's it's normal, right.

And normal is again a loaded word.

So what I'd like to do is ask both of y'all the question of parents.

We're taking care of cystic fibrosis patients until a certain age where they were not thriving anymore and ceased to exist.

Terrible way of saying it.

But now we're looking at patients be coming of age to where they're taking care of themselves.

So as that transition happens with both the all being pediatric pediatrician, specialists, what is the advice to families and or directly to patients as they're aging out as mom and dad's care?

Does that make sense?

Yes.

And I think what a beautiful thing that is.

Yeah.

So first of all, we have to one of the recommendations with our to be accurate data center is that you should be able to take care of an adult with CF.

So we are patients is not already comprised of pediatric patients.

We have patients.

They're adults 30 years 20 and about.

And this is important because what happened is before they use they they didn't survive enough.

So as a pediatricians taking care of patients is it's a favor.

Also we used to take care of adults would at that time.

They used to survive.

But not only that here nowadays we have the ability to run our doctors.

Doctor who's, pediatric and internal medicine physician will help us to do the transition for those patients.

So it helps because he has the ability to teach them.

This is what you need to do now on you will not have mommy and Daddy behind you.

You have to remind yourself of the medication.

And and it is for learning process that that is that is there.

So I think, I think that, that that I don't know if answer.

No it does.

It actually makes the answer.

And I would think that the condition would not get worse just physically unless medications and or things are not adhered to any more.

Would you.

Yeah.

No, I like to add anything on to that.

No, I, I agree, I think we're lucky because we do have, someone who is able to bridge that pediatric adult world, and really help with transition in transitioning those patients from, you know, having their parents there with them for every visit to being able to attend, the visits by themselves.

And I think one of the things that, we really stress is taking ownership of your disease when you have a chronic disease.

I think it's important when you're seeing the patient.

And even though the parents are there, you really need to talk to the the kid at the same time, right?

It doesn't really help if the parents are answering all the questions.

And I think if you really push kids to take ownership of their disease, it makes the transition, that much easier.

And I think we both do that.

We really try to stress, having kids like, no, their medications know what they're doing.

And I think that helps a lot with that transition.

That's a perfect note to end on.

Thank you so much.

Again, this program has been called Advances in Medical Management of Cystic Fibrosis.

It's been very exciting to see everything that's been going on.

And if you just joined us and are curious about this, there's a couple of places that you can watch this program again, in any program that the opposition has put on.

The first one is PBS El paso.org.

You will go to that website and you will automatically see the El Paso position pop up.

And also the El Paso County Medical Society website which is EPK, cms.com.

And then you also have YouTube.com.

And when you're doing the search engine, just put in there the El Paso physician, using the last program that has been aired will pop up.

But you can also go in there.

And for example, we can talk about the geneticist that was on about two years ago.

Great program as well.

But you can always watch the programs back there.

Thank you so much for joining us.

I'm Kathrin Berg and this has been the El Paso physician.