Improving the life span of Cystic Fibrosis patients

The Means Report

AUGUSTA, Ga. (WJBF) – The Means Report tackles the topic of cystic fibrosis, a disease that impacts give or take 200,000 Americans. Not a lot of people. You may not have heard a lot about it. We’ll look at the efforts here at home, at M.C.G. to expand the lives of patients, to extend their time here, and give them a high quality of life. It’ll look at how the dietary implications affect people with cystic fibrosis, and how we might be able to tweak that nutritional component and help those patients do better. Some great research is going on as we speak. Also, cystic fibrosis and the coronavirus. You know the coronavirus is not kind to our lungs and lung function is at the root of this disease. Let’s see what happens when those two intersect.

Brad Means: Doctors, thank you so much for what you do for these patients. And thanks for being here today, I appreciate it.

Dr. Caralee Forseen Thank you.

Thank you.

Brad Means: Dr. Forseen, let’s start with you with our first question. We all have, and this is just to make sure that I understand, and in turn, the audience understands what cystic fibrosis is. My basic grasp of it is that we all have mucus in our systems. And when you have cystic fibrosis, that mucus doesn’t move around like it should. And among other things, makes it difficult to breathe. Is that pretty accurate?

Dr. Caralee Forseen That is correct. There is a cyst, cystic fibrosis is a genetic disease. So it’s inherited. And as a result of a abnormality in a protein, a mutation, the mucus in the airways of individuals with CF is much stickier and thicker. It ends up being dehydrated, so to speak. So it’s much more difficult for them to clear the mucus out of their lungs. It also impacts lots of other organs as well. It impacts the pancreas, et cetera.

Brad Means: Dr. McKie, how soon do you notice the onset in your patients? Is it at birth?

Dr. Kathleen McKie Well, fortunately, most patients are now diagnosed very early by newborn screening and they’re healthy when they’re born. Although they can start to have some nutritional problems pretty quickly. They have trouble digesting fats, and so they may have trouble gaining weight. But usually we don’t see the respiratory symptoms until a little bit later.

Brad Means: When they prick your baby’s heel, Dr. McKie, is that what they’re testing for, cystic fibrosis? Or does that cover a wide range of potential illnesses?

Dr. Kathleen McKie It does, it covers a wide range of potential disorders, but cystic fibrosis is one of them.

Brad Means: Dr. Forseen, what are some of the symptoms? What might we look for in the young people in our lives to raise a red flag?

Dr. Caralee Forseen So I see just adults. But typically for younger children and for those who haven’t been diagnosed, it may be recurrent infections, bronchitis. They may have asthma. They may have difficulty gaining weight. They could have pancreatitis. In older patients, they could have kidney stones, which would be a less common manifestation. Recurrent pneumonias.

Brad Means: And so what kind of test? Well, two questions, Dr. McKie. First of all, how old is your youngest patient?

Dr. Kathleen McKie Well, we get babies as young as a couple weeks old after they’re diagnosed with newborn screening. So we have patients all the way from a couple weeks old to age 21 in the pediatric center. And then after that, they go see Dr. Forseen in the adult center.

Brad Means: Right, and so what kind of testing procedure do you use? If the heel prick didn’t work when they were first born, what do you do with older children to see if they have it?

Dr. Kathleen McKie So the heel prick is a screening test and it just gives us an idea if a patient may or may not have CF. And if the heel prick suggests that they might have CF, then we go on to do something called a sweat chloride test. And that’s really the gold standard of how to diagnose whether someone has CF or not. Most patients also get genetic testing now to see which particular mutations they have, since there are a lot of new therapies that are directed towards specific genotypes.

Brad Means: Dr. Forseen, where do we stand on life expectancy? How old is your oldest patient? When I was growing up, I knew somebody with cystic fibrosis. And if memory serves, they were expected to live until their 40s maybe? Has that gotten better?

Dr. Caralee Forseen It has gotten better. Our oldest patient is in her mid 70s.

Brad Means: Wow.

Dr. Caralee Forseen So.

Brad Means: How did you make that happen? Is it just constant care and treatment and visits to you?

Dr. Caralee Forseen So one of the important things in CF is really the multidisciplinary approach to care. So it’s not just the physicians that are caring for the patients. It’s really a team of experts. So we have a, we’re fortunate to have a dietician, a social worker a respiratory therapist, a physical therapist, a pharmacist, a psychologist, and a gastroenterologist, all with expertise in CF.

Brad Means: Dr. McKie, it’s sort of your turn as we bounce back and forth. But if you’re not comfortable answering this question, you can ask Dr. Forseen to answer it. But it’s about the coronavirus. And I know a lot of young people either don’t have it or they’re not as symptomatic as perhaps Dr. Forseen’s patients, but what has happened to cystic fibrosis patients, if you’ve seen this, who have gotten the coronavirus? Because it’s so hard on your respiratory system.

Dr. Kathleen McKie Well, we’ve been fortunate. We haven’t had a whole lot of pediatric patients that have had CF that have gotten the coronavirus. And those that have, they have had a flare up of their CF, but fortunately hasn’t been anything that we weren’t able to handle. Dr. Forseen probably has had more experience with that.

Brad Means: Yeah, have you Dr. Forseen? Go ahead and talk about that. What have you seen firsthand in your office?

Dr. Caralee Forseen So we have had probably 15 to 20 individuals who have had the virus. We are fortunate that actually none of them have had to be hospitalized as a result of the virus. They’ve been able to be managed at home. And part of that I think certainly has to do with the new medication that many of them are on.

Brad Means: Dr. Forseen, you mentioned this multi-disciplinary, multi-discipline approach to cystic fibrosis, how it takes a lot of docs, a lot of experts to help these patients get through this illness and maintain a high quality of life. When they’re away from you, when they’re at their homes, are there treatments or things that they can do to make their days brighter and better?

Dr. Caralee Forseen Absolutely, most of them are doing several medicines, typically nebulize to help with their lung disease, the lung manifestations. And they may be using something called the vest or other ways to help mobilize mucus. Many are also taking enzymes, pancreatic enzymes to help absorb the fats and foods that they eat, since that is a typical abnormality for individuals with CF.

Brad Means: Dr. McKie, what’s it like for a child with cystic fibrosis because of the dietary, I don’t know if limitations is the right word, because I thought that you were supposed to eat as much as you could because your body is so incapable of absorbing nutrients. What does a day look like for a child when all his or her friends are eating regular food? What do they have to do?

Dr. Kathleen McKie Well, you’re right. We do encourage our patients with CF to pretty much eat whatever they want. And in fact, we kind of tell them to eat all the bad stuff. We tell them to eat all the pizza and calories that they could possibly fit in. They just have to take, most of them have to take pancreatic enzymes to help them digest the fat in the food that they eat as Dr. Forseen mentioned. And so for some of the kids it can be a little bit embarrassing if they have to take medicine every time they eat at school. But, and the other thing that can be hard for them is sometimes no matter how much they eat, they really can’t fit in enough calories to maintain their weight. And then some of those children have to have extra feedings, either through dietary supplements or sometimes through a G-tube.

Brad Means: Why can’t we just give all these patients a lung transplant and fix it? Would new lungs make everything go away? And either one of y’all can answer that.

Dr. Caralee Forseen So lung transplant will help the lung manifestations of the disease, but it certainly is by no means a cure because there are a lot of complications that can occur associated with transplants. So certainly the goal is to delay that as a possibility. So if they get two new lungs from a donor, they won’t have CF in their lungs anymore, but they still have CF in all their other organ systems.

Brad Means: Dr. McKie, you talked about diet, both of you all have talked about treatments that can be done both in the clinical setting and at home. If all of those things are working at their optimum, can these people with cystic fibrosis lead what most of us would call a normal life? And by that, I mean, can they exercise? Can they play sports?

Dr. Kathleen McKie Absolutely, we really encourage our patients to exercise. Exercise is a great form of airway clearance and actually helps them get more mucus out of their lungs. So we love for our patients to play sports, play with their siblings, you know, run. We’ve got diverse. We’ve got baseball players. We’ve got kids that do all kinds of sports.

Brad Means: Dr. Forseen, you mentioned that it impacts so many other organs, so many other parts of the body. Does that typically happen after Dr. McKie hands the patients off to you and they age? Or can we see this kind of impact on young people too?

Dr. Caralee Forseen Definitely can see in young individuals as well. Certainly we see some of the other complications later in life as they get older. But yes, even the pediatric population may be diabetic. They certainly oftentimes are pancreatic insufficient, so they are taking pancreatic enzymes. They may have sinus disease. So some of those things can certainly be seen earlier in life. And then there are other things that we may see later in life.

Brad Means: And probably my last question, and I always ask this. Well, I do most of the time with doctors. Are insurance companies good about working with you? Some families watching right now may be overwhelmed by everything that’s involved in treating a cystic fibrosis patient and might be leery about coming in to get checked.

Dr. Kathleen McKie It is, insurance companies usually are very good about covering the therapies that we need. But it can be very complicated because there’s just a lot of burden of care for these patients. So that’s one reason why we have a social worker on our team that can help us with negotiating with insurance companies and other programs that can help our patients get what they need.

Brad Means: Wow, y’all do such an incredible job helping these patients young and old live their very best lives. And for that we are all grateful to you. Doctors McKie and Forseen, thank you for taking the time to be with me today.

Dr. Caralee Forseen Thank you.

Dr. Kathleen McKie Thank you.

Brad Means: I appreciate it so much. When “The Means Report” continues, we will continue our look at cystic fibrosis with an incredible in-depth study. Doctors Forseen and McKie a part of this as well. A study that could really help doctors and patients make great strides forward, on “The Means Report.”

Part 2

Brad Means: Welcome back to “The Means Report.” Cystic fibrosis is our focus today. Learned a ton about it in our first segment. And we are going to learn about a research project that hopes to learn more about it as well. It’s going on at M.C.G. at A.U. And Dr. Ryan Harris is the principal investigator in this five-year study. Dr. Harris, thank you for what you’re doing for these patients. And thank you for being here today. We appreciate it.

Dr. Ryan Harris: I’m glad to be here and thanks for having me.

Brad Means: So, first of all, let me just make sure that I’m correct. This study, if I had to sum it up briefly, would be to figure out how we can improve the length and quality of cystic fibrosis patients’ lives. Is that accurate?

Dr. Ryan Harris: Yeah, I think so. I mean, the ultimate goal of all the research that we’re doing is to improve quality of life in our people with CF.

Brad Means: You know, the doctors in our first segment certainly touched on this, but I just want to get your take. So many people think of it as a breathing disease. But it is so much more, especially in the digestive area I’ve learned so far today. That’s true, isn’t it?

Dr. Ryan Harris: Absolutely, there’s so many different factors related with CF that we’re interested in. And in fact the grant that we just got is really looking at all the other factors, other than the lungs. So we’re really not focused on lung health in this grant. We’re just focused on everything else related to CF.

Brad Means: So your study’s gonna look at three components, nutrition, body composition, and exercise, and see how those things impact CF patients. Let’s start with nutrition. Our doctors in our first segment confirmed that the goal is to try to get CF patients to eat as much as possible because it’s so difficult for their bodies to absorb the nutrients. So are you looking to perhaps tweak the food here? Or the way the body handles it?

Dr. Ryan Harris: I think a little of everything, Brad. You know as the original thought was to just feed our patients as much and as anything that they can eat just to maintain their body weight. But now there’s different treatments that are helping with nutrition status, absorption. And so now we really gotta focus on the quality of the diets in our patients and ensure that these patients that once were thought to, or once were told to be, to eat anything they can, you know we really gotta focus on exactly what they’re putting in their mouth because it can impact their health later.

Brad Means: So different diets for different study, excuse me, different study participants, is that how it’ll work?

Dr. Ryan Harris: So one of the big focuses is on fat intake and sugar intake. So we’re really trying to understand how these different macro nutrients affect the progression to CF related diabetes.

Brad Means: Are there foods, even before the study gets underway, are there foods that you know right now that are easier on a compromised digestive system that you recommend more for your patients?

Dr. Ryan Harris: That’s hard to say, Brad. There’s no real research really that could say what’s easier on digestion. The enzymes that patients take definitely help the fat absorption, which generally is the key component which can increase their body weight.

Brad Means: Let’s look at the body composition part of your study. Does that just mean you want to look at cystic fibrosis patients of all shapes and sizes?

Dr. Ryan Harris: Kind of. The body composition is made up of two main components. You have your fat and you have your muscle. And what we’re trying to understand is the relationships between these two different types of components. You know, whether those patients with a higher muscle component actually have better outcomes.

Brad Means: And so are there ways to perhaps, we hope, beef up the muscular structure of patients who don’t have it so that they can fight this disease better?

Dr. Ryan Harris: Absolutely, exercise, exercise, exercise. We really want our patients to engage in more exercise and physical activity. And this doesn’t mean going to the gym and pumping iron, lifting weights. We really just want them to be active in general. And we know that physical activity can improve the quality of life, reduce hospitalizations. It could actually increase survival in our patients. So we really want them to exercise.

Brad Means: You’re right, Doctor, it’s so good for you. So will all of the participants, and I think y’all have 50, will all of them to follow the same exercise regimen? You know, okay, everybody time for you all to walk a mile, time for you all to do two sit ups? How will that work with that many people?

Dr. Ryan Harris: We’re so at the beginning right now, Brad, that this study really isn’t looking at an exercise program or an exercise prescription. We have a really cool technique in the lab to measure the capacity of one’s ability to exercise. So right now we’re just focusing on the exercise capacity and the relationship with all of their outcomes. Once we get all this data and learn exactly what this means, then we could really develop the individualized exercise prescription for our patients.

Brad Means: Dr. Harris, is there a typical cystic fibrosis patient? You know, the way that they look or function, what their limitations are? Or are they all different?

Dr. Ryan Harris: That’s an interesting point. A few years back, we would all say that most patients with CF were skinny, lean. And now we just, we have patients of all different types of body types. And some are gaining weight because of the therapies, you know, unstoppable. Some are still lean. I don’t think you can really pinpoint someone with CF out of a big crowd.

Brad Means: You mentioned diabetes a moment ago. How early do you see the onset of that illness in CF patients? Is it from childhood on?

Dr. Ryan Harris: I think it takes a few years to progress into a CF related diabetes. Generally, it’s about 50% of adults will acquire the CF related diabetes. But we are seeing some pediatric patients early on that have the disease.

Brad Means: Certainly our goal is to help these patients live longer, better lives. But as they get older, just like with the rest of us, doesn’t that make them even more susceptible to illnesses that show up later in age? And since they would be more susceptible to it, wouldn’t their bodies be more ill prepared for that and it would have a more negative outcome for those patients?

Dr. Ryan Harris: Absolutely, and that’s I think what really got my interest in this patient population about 11 years ago. Patients are living longer. They are taking these really cool medicines to help the protein, you know, help them live longer. And with that living longer, they’re gonna start developing what we associate with age related diseases and disorders. Heart disease is one of the big ones. So we’re really trying to look at this as a holistic approach, and saying these patients are living longer. Let’s try to learn a little bit more about these other conditions and help prevent them.

Brad Means: Who is funding this study? Where did y’all get that?

Dr. Ryan Harris: This study in particular is a grant from the National Institute of Health. And in particular the NIDDK, which is focused on digestive and kidney disease.

Brad Means: I forgot a question that I wanted to ask you a moment ago when we were talking about the digestive part of it. And fortunately we have time to get it in. Just to help put more of a face on this disease, so when the body is not absorbing all of the nutrients it needs to, and you’re eating as many calories as possible, what happens? Does the body void most of that out, leaving you with very little to process? Is that how the body handles it, handles food?

Dr. Ryan Harris: Right, it just goes through and out the other end.

Brad Means: Wow, that doesn’t sound like a very pleasant existence. What can you do to improve that part of their lives? Are there medications they can take that can stave off that symptom?

Dr. Ryan Harris: Yeah, part of the treatment regimen is to take enzymes to help digest the food a little bit more so that we could absorb it in the gut. The new modulator therapies actually affect the transporters within the gut to help them absorb more. So I think there’s multiple ways that we could do that.

Brad Means: Well, I’ll tell you what. For a disease that does not get in the spotlight as much as it should, you have done an incredible job of helping us all understand it, Doctor. I wish you the very, very best on your study. And I look forward to following your progress along the way. I hope it yields great results.

Dr. Ryan Harris: Great, thank you.

Brad Means: Absolutely, we appreciate you so much.

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The Means Report first aired in January of 2009 offering coverage that you cannot get from a daily newscast. Forget about quick soundbytes -- we deliver an in-depth perspective on the biggest stories. If they are making news on the local or national level, you will find them on the set of The Means Report. Hosted by WJBF NewsChannel 6 anchor, Brad Means, The Means Report covers the topics impacting your life, your town, your state, and your future.