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Preventing adult heart attacks in early childhood? Don Wilson, M.D., explains why it’s absolutely within REACH. Children with high cholesterol, especially those who have a genetic cause, such as familial hypercholesterolemia, also known as fH are at higher risk for developing premature heart disease, including heart attack or stroke, as they become adults. At the forefront of developing guidelines for lowering this risk, Dr. Wilson details how the Risk Evaluation to Achieve Cardiovascular Health, or REACH, clinic at Cook Children's is putting those guidelines to work, changing the outcomes for children in the future, and saving the lives of parents today.
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Host : Hello, and welcome to Cook Children's Doc Talk. Today we're talking with Dr. Don Wilson about genetic dyslipidemia, and cardiovascular disease. But first, here's just a little about Dr. Wilson. He is board certified in pediatrics, pediatric endocrinology and clinical lipidology. He is the founder and currently the medical director of Cook Children's REACH program, one of the first cardiovascular risk assessment clinics for children and adolescents in the nation. Dr. Wilson is a fellow of the National Lipid Association and an associate editor for The Journal of Clinical Lipidology and a member of the board of directors of the Southwest Lipid Association and the American Board of Clinical Lipidology. Welcome Dr. Wilson.
Dr. Wilson: Thank you.
Host : So children with high cholesterol, especially those who have a genetic cause, such as familial hypercholesterolemia, also known as fH are at higher risk for developing premature heart disease, such as a heart attack or stroke as they become adults. You've been at the forefront of developing guidelines for lowering this risk, launching the REACH clinic at Cook Children's, can you tell us a little bit about the clinic and its goals?
Dr. Wilson: I'd be delighted to. First of all, it might be helpful to understand that what we're talking about really is blood fats. And the words cholesterol, triglycerides tend to be very intimidating, sometimes very frightening. Even worse yet, the word dyslipidemia, which is just a fancy word for the same thing, so blood fats are normal for all of us to stay healthy. It's only until those levels become extremely high that they become problematic. So cholesterol is one form of blood fat. And people who have familial hypercholesterolemia fH, they genetically can't process that fat. So fat comes from two sources. One is what we eat. Most people are familiar with fatty foods, pizza, some of the Mexican foods, whole variety of things can can be high in fat content. But what people may not realize is that fat is also produced primarily in your body. So the liver actually produces cholesterol, about 80% of it, in response to your need, particularly when you're not eating or like at nighttime, for example. So genetically, when you have fH, your levels are going to be extremely high. For example, most children have a cholesterol level, LDL or bad cholesterol level right around 100. But children who have an L level is twice that, say 200-250 have a very high risk of developing heart disease. Not at this age, not even when they're young adult. But usually by the age of 40 or 50 years of age, these people are at very high risk.
Host : So I know you're a proponent of screening children early when they have a parent with fH, or a history of premature cardiovascular disease. But there are a lot of adults out there who may not know they have this risk. Would you like to see mandatory screening of all children? And if so, at what age?
Dr. Wilson: So currently, there are recommendations for mandatory screening, it's just not widely held yet. Those recommendations take quite some time to become standard of practice. But you could say, well, why are pediatricians worried about a disease that's not going to happen until you're 50 years of age, is because we particularly those of us at Cook Children's hospital are very committed to the lifelong health of children and their families. So currently, we actually screen those children at 10 years of age, sometimes sooner if they have an informative family history. So if you come from a family in which many of your family members may have had some kind of heart disease, wind up with a procedure of your heart, may have unfortunately succumbed to heart disease, then those of the kids we want to screen early. You'd be amazed because by two years of age, we pick up a lot of high cholesterol in children. So those, what we call focused screening tests, and so forth, can pick those kids up very early with the idea that we need to start implementing some changes early on in their diet, for example, those kids, we'd actually recommend that small children start on low fat milk, starting at one year of age, for example, and then reduce the fat content in the diet. Long term, that's probably not going to be enough to reduce their cardiovascular risk, they're going to usually require medication to do so, simply because their bodies again can't process that fat. But by doing so we can provide tremendous help to those folks in terms of avoiding heart disease when they become older. Now, let me ask answer another question to ask which is very important. And that is, we not only have a focus on the child, but let's say we pick up a two year old that has familial hypercholesterolemia. So they have high blood cholesterol levels, not because of what they eat, but because of how their body cannot process that fat. Well, we know that it's genetic, which means it's simply transmitted by one or both parents. A two year old has fairly young parents most the time so then we start looking at the family members and we asked them to go get their cholesterol tested. We picked up a number of either fathers, mothers or both who, A, hadn't been tested in the past. B, didn't know that their cholesterol level was high and. C. may to actually be much closer to having an event or a bad outcome than the child would be. So we've actually feel like that we've actually done a great job in terms of looking at the entire family's health and trying to keep everybody healthy.
Host : Cardiovascular heart disease is the leading cause of death in the US. Add to that the fact that every minute in the us a child is born with fH, why do you think early screening in children hasn't gained priority? And what can be done to change this?
Dr. Wilson: Let me just repeat what you said. Because it takes a moment to realize that every minute of the day, there's a child born with familial hypercholesterolemia. So do the numbers. Lots of kids around. So it's about one in 200-250 kids. So if you have a school system of 1000, kids, you have four kids already, but most recommendations that are promoted to prove health take on average around 17 years to find their way into clinical practice. Why is that? Well, it's because the practice of medicine is very complicated. We have to get out and inform physicians, they rightfully so need to ask questions, they need to make sure that they're comfortable with that recommendation. And then they need to figure out how they're going to implement that in their practice. I think Cook Children's actually has one of the better systems in the country for doing so because we have automated systems that we can plug in these recommendations. And we can monitor the outcome of that. So we know how many kids in our system should be tested, we can figure out right away whether we're doing a good job or not. So about 10 years ago, I looked at this at Cook Children's, with budget physicians to see how we were doing with our screening program. Nationwide, only 4% of physicians were actually testing children for cholesterol. Our rates were actually 20%. So the question sort of become for us a we're doing a good job, right? The question is, can how can we do a better job? That's the question we like to ask how can we do better, right? So we want to go from 20 to 40, to 60, to 100% of these kids, so that we can adequately assess risk and keep kids from having heart disease when they get older. But physicians are not hesitant to implement recommendations, it just takes a while for that process to mature. The second thing is we have to we take great pride in trying to educate our families about the importance of doing it. So we don't want to do anything to or for a child or a family without them understanding what the benefits are. And partnering with them in terms of them being the parents agreeing to an understanding what to do with that information. So we encourage people to ask questions, it may take a visit or two sometimes for them to understand kind of what we're trying to do and what to do with the information. But it has important ramifications not only for their child, and the future of their child, but also themselves and even their grandchildren. Because again, a child with fH is potentially going to pass that along to their children.
Host : So can you describe some of the barriers that currently exist in childhood screening? What are the shortcomings in current screening protocols for kids? And why do they fall short in terms of preventing a cardiovascular event in the future?
Dr. Wilson: So we realized that there would be some potential barriers in terms of cost, for example. If you had another test, that means that X number of children are going to be tested. Well, it turns out that the cost for tests are relatively small, we also looked at the mechanism by which kids are tested in the clinic. So for example, if you say 10, that child may not have any other blood tests that are scheduled for that particular visit. They may or may not. But what we do know is that at two years of age, we usually screen the children for other things. So we've talked several of our clinics into screening the two year olds. And so they just don't have the kids don't have to have an extra blood test, they just simply give us another drop of blood. And we test for cholesterol right there in the office. Cook Children's has also been very generous in terms of providing the test equipment for what we call point-of-care, so that those machines are there in the office, and the cholesterol test can be done on the time the visit. And then the pediatricians can have conversation with the families right then. They don't have to wait for a phone call. They don't have to send them information in the mail. And the importance of that is giving timely information to families, but more importantly, being able to look in their eyes and sort of answer their questions as that conversation is being undertaken.
Host : So some argue that the cost of screening is too much at this level. But what is the long term cost of not screening?
Dr. Wilson: Yeah, that's a terrific question. So some of the criticisms, for example, doing anything with children, but in particular, screening is frightening children. We have no intent whatsoever to frighten anybody. This is just an informed conversation. But we and others have actually carefully looked at that impact to ascertain whether we're actually causing any fear or harm about having a genetic condition that's lifelong. Unfortunately, if you've had family members who've had a bad outcome with cardiovascular disease, telling a child that he or she is actually at risk for that could evoke fear. But we've carefully looked at that and we also do a lot of education with the child, making sure that they understand what this is and what it isn't. We make very clear that the child does not have any disease at this time, they clearly have a very strong heart, very normal vasculature blood vessels. And we try to do so in an age appropriate way. What I think we can all take great pride in at Cook Children's is that when those kids come back, if you ask them, you know, why did you come to see us? And what were you telling them? What did you understand? Those kids are terrific, they can give you the answers right away. In fact, we've used many of them to teach other children about cardiovascular disease, and the importance of cholesterol screening. Many of them do so as a school project, for example. So come time for the science fair project, many of the kids will actually do that as a science fair project. So I think there's many benefits to obviously involving kids and the families in this conversation. But if you understand that people who have familial hypercholesterolemia, or fH, approximately 50% of the men and 30% of the women are going to have a heart attack by the age of 50. That's a very early age for people to have problems. We hope that they would never have problems. But those are the statistics. So again, by finding through screening, because the kids otherwise look quite healthy. This is not about being overweight. This is not about sedentary behavior. This is not about eating poorly. This is about a genetic process in which your body can't process cholesterol, or blood fat. And just allowing that fat to accumulate in the bloodstream clogs arteries, most people know that term, right? We call it atherosclerosis, or hardening of the arteries, but the reality is they become plugged up. And when they do so you cannot get blood flow to vital organs, like the heart or the kidneys or the brain. For the heart that's going to translate into having a heart attack. For the brain is going to be a stroke. And I think, unfortunately, most people in America understand the signs and symptoms associated with that, and the outcomes. So we never want to have a screening program where we trigger cardiovascular screening based on symptoms, which is unfortunately what happens in the adult world, right, you don't have insurance, you don't take the time to go to the doctor, they don't do the screening test, then you wind up at 40 or 50 years of age complaining of chest pains when you walk upstairs. That means you have clogged arteries. And yes, there are procedures such as stents, that increased blood flow or some people are familiar with cabbages or coronary artery bypass surgery. But you never want to get there, if you can help it. The key to it, test the kids and offer effective treatments early in life where you can reduce that risk over a lifetime.
Host : That sounds fantastic. And I hadn't even really thought about the kids teaching other kids particularly like out in the community like that, what a fabulous idea.
Dr. Wilson: Children are great resource. First of all, they're inspirational. They're very bright. They talk to other children, they have a credibility with their peers. So we've learned a long time ago that, particularly teens are a great source of information for their peers, but they need to make sure that they have accurate information.
Host : So let's talk a little about LDL, what is a normal LDL level in a child and when should a pediatrician be concerned?
Dr. Wilson: So typically, the LDL, sometimes we talk about l as being lousy, right? Good way to remember it. So the LDL cholesterol level below 130, would be considered normal. Some kids have levels that are 80-90, but typically around 100-110. When those levels exceed 130, we become concerned. If they get to be 160, we look to see if there's other risk factors. So then, are other things going to also contribute to heart disease, such as high blood pressure, diabetes, being overweight, being sedentary... unfortunately, some children start experimenting with smoking, or vaping. Those things all contribute. So we start counting up what we call risk factors. So if you have a couple risk factors plus high cholesterol, 160 and above, then we might suggest that you would benefit from treatment at least until you can reduce those other risk factors. If you have levels of LDL cholesterol, or lousy cholesterol, if I can use that term of 190. and above, then the chances of that coming down simply with diet and exercise are not very good, because that's going to be genetic. And we can prove that we can actually show you the genetic mutation in most of the kids. But that one's going to require medication. Now, nobody likes to think about, physicians or parents either one, lifelong medications or need for medication. But the reality is, if your body does not allow processing that cholesterol it's in your best interest. So do we have a 50 year history of treating kids? No, we have at least 20 years history, but we take the responsibility of safety very seriously. And so we look at every parameter we can, in children, just like adults, it's going to be monitoring things like liver function, kidney function, so forth. Number two, we always want to make sure that the medication is actually doing what we hope it will do, which is reducing the bad cholesterol or LDL cholesterol. And it does it's very effective is simply getting them swallow a pill every day. And the good news is the medication is quite inexpensive. In many people who have insurance, for example, a lot of times they give you the medication at no cost, because the insurance companies have also realized that that's helpful for both parties long term. But in children, we also take very seriously growth and development. We want to make sure that there's nothing there that's going to either slow down or hamper their growth and development. And also remember that we have to be very sensitive about reproductive health for both boys and girls. So those things have been looked at extremely carefully. And you could imagine the process of getting a medication like a statin, for example, FDA approved for children, there are going to be a lot of hurdles, as well, it should be. But I think scientists and clinicians have come through that rigor and then been able to demonstrate to the FDA and more importantly to themselves and to parents that this is safe drug.
Host : So not all high LDL levels in children are genetic, some are acquired, how do you determine the difference in their diagnosis, and is there a difference in treatment protocols?
Dr. Wilson: So we and others would never be quick to assume that this is necessarily genetic, unless we've done things that have not been successful in bringing the cholesterol level down. So when we see a child in our office, for example, we at least have one or two measures of the cholesterol level, specifically the LDL cholesterol levels. And we've usually given them specific recommendations with regard to alterations in diet, physical activity, weight loss, etc. Sometimes medications can also increase the LDL cholesterol level. So if there's an opportunity to alter those medications, or eliminate them, not always possible, but we do everything we can to reduce the LDL cholesterol, including by the way use of some dietary supplements. So many people may be familiar with the American Heart Association symbol on various things like margarine, or milk or whatever, that simply means that that preparation has been altered a little bit to reduce the fat and also use what we call plant fats, or plant sterols or stanols. Those are just fancy terms to mean that plants also make cholesterol, if you will, what we're concerned about is animal cholesterol, or animal fat, but the plant based fats actually reduce cholesterol in your body. So using those either in those type of food preparations, or as a dietary supplement can help. So we try all those things for as long as the parent or child would like to. And then I think by partnering with them, we figure out after a visit or two, that that's just not going to be possible to do by those means. If you have something like diabetes, it's important also to try to improve control to the level that you can. So we try to take every step that we can before making the decision together with the child and the parent, that other steps or other medications might be necessary and beneficial, as well as safe to do.
Host : Certain traditional thinking is that cholesterol levels are the direct result of a poor diet. And we know there is some truth to this, but it can also be inherited. And that's where the importance of screening children at an early age becomes really important, correct?
Dr. Wilson: That's correct. And that's why we've actually started focusing on two year olds not only because of what I said earlier, that is we can incorporate that into the standard blood tests that they're getting so we don't have to have another blood test for the children. But also, because at that age, very few of the kids have weight related problems or have developed other dietary habits that may actually increase the cholesterol, as children get older, and certainly as they become adults, it gets a little bit more confusing, right? Many people have gained weight, they're sedentary now as an adult, because they have jobs or a whole variety of life related issues. But the discrimination, if you look at the LDL cholesterol levels, for example, is much better at a two year old than it is at a 20 year old. So doing early screening really helps us separate the people who have genetic problems from the ones who have acquired problems. Now, in fairness, that you can have both. So we also do screening for children who have weight related problems or thyroid problems or diabetes, but it's a separate distinct conversation from the ones who have genetic mutations.
Host : There is also a perception that children at risk are predominantly those from lower income families as well as kids who might be part of the drive thru diet generation, if you will. But this isn't necessarily so. Can you give us a view of what populations are actually at risk?
Dr. Wilson: Yeah, so thank you for bringing that question because if that perception is out there, it's false. fH or familial hypercholesterolemia, pretty much cuts across every race, ethnicity age group. It's something that children are born with just because that's the way their genetic makeup is. So it has nothing to do with selecting a group of patients. We do know that there are certain populations, for example, in African American children, we would look for more hypertension or high blood pressure. And our Mexican American kids, we know that they're much more likely to have insulin resistance or diabetes. But with fH or high cholesterol, it's pretty much all kids. So the nice thing about uniform or universal screening is that there are no criteria. If you have a 10 year old in your office, as a pediatrician, you should realize that that child needs to be screened. It doesn't matter what their family history it is, it doesn't matter what their personal history is, you just go ahead and screen them. And that's the beauty of it.
Host : So once a child is diagnosed, what are the treatment options, diet medication, a combination? And if medication is chosen, what are the risks short and long term?
Dr. Wilson: So we always start with trying to understand the problem, because the best fix for something is always understanding what's broken, right? These kids are suspected of having a genetic mutation, but that may or may not be proven. And as I said, we always start with trying to understand the family's eating habits, understanding your physical activity, taking a critical look at the child, we always eliminate secondary factors. So for example, if a child has a problem with their kidneys, or they have problem with their liver, or they have problem with her thyroid function, like hypothyroidism, or an underactive thyroid, those can also affect the cholesterol levels. We take a careful medication history, not only on prescribed medications, but also over the counter preparations as well as dietary herbal preparations and that sort of thing. So understanding all that we start off with lifestyle modification. What can we do as a family to try to improve everyone's cholesterol, not just this child's cholesterol, and it's important not to single the child out because these are usually family issues, right? And they're family solutions, we want people to be supportive of each other. So whatever period of time that takes, but we usually give it six months to a year to see if we can make any improvements with frequent conversations or additional education is necessary, then we'll sit down and say, Okay, this continues, the high LDL cholesterol has not really significantly changed in the parents usually come to the right conclusion, which is this seems to be part of my child, it may be part of our family. We actually screen the other kids, because high cholesterol levels in other family members sort of suggest again, that it's going to be genetic. And then for many of our families, we actually offer genetic testing, so we can actually pinpoint, it's not absolutely necessary for treatment, some people opt not to for a variety of reasons. Some people really like to know whether their child has a genetic mutation, because then that information can be used to tailor the treatment. It can also be used to pinpoint cholesterol problems in other family members because it cholesterol levels can be high, or maybe in midrange. But it'll definitively tell you whether you have that condition or not. And whether young children, for example, might be at risk of passing that on to their future children. But after all that conversation's over the standard of treatment would be use of some type of cholesterol lowering medication. Typically, we use something like a statin, many people are familiar with these terms. There are a variety of them, there's about 12 different statins that are FDA approved for use in children down to at least at age eight. In Europe, it goes down to age six. So beginning at that age, we would start using medications to lower the cholesterol. The good news is even though in the adult world side effects are usually primarily with muscle aches and pains, I'll tell you right away that adults have a lot of aches and pains so we don't want to add to them. But some people do seem to have difficulties as an adult with taking statin medications. So we always ask about that, because sometimes the family members are already being treated. But in children, we actually don't have to use as potent a medication. And we don't have to use very high doses, because what we're trying to achieve is cholesterol lowering, but over a lifetime. In adults, they may start out with an individual who's 42 years of age and already had a heart attack. So you have to jump in really with both feet there and make sure you get the cholesterol levels down to keep them from having any more problems. But in showing we can actually start with less potent medication, we can start with lesser doses. And as a consequence of that we and others really have not seen very many of any concerns in children. Now we always monitor them, we always look at them, we always talk to them about their school performance, their activities and whatever, but they don't seem to have any changes whatsoever. And then we also look at some screening tests. For example, we may look at liver function studies, we've not seen any changes whatsoever. As a consequence, the national recommendations is not to do any more screening tests. Now we tend to kind of hang on to those for a little bit. But most people will just look at the LDL cholesterol response to treat it. And then titrate the dose. You know, the biggest problem with children in terms of taking a medication like a statin?
Host : Parents?
Dr. Wilson: No. The biggest problem is actually remembering to take it. For parents that shouldn't surprise you, right. So the biggest problem when when when the kids come back and they've been on medication for a short time mom says He never remembers, She never remembers, I have to tell him all the time. I said Mom, that's basically going to be your responsibility until they get old enough. And we take the personal responsibility of making sure that they're educated about why they're taking this medication, why it's important, what the benefits are to them, and if this is going to be a lifelong endeavor.
Host : Interesting. I'm one of those terrible kids who forgets to take her medication. So when I said parents.
Dr. Wilson: Well, you know I think it's important, I usually tell families Look, you're not gonna be perfect, your child's not going to be perfect doing this. He or she may be perfect in other ways, but they're not going to be perfect in terms of taking their medication. As long as it's an occasional missed pill is not going to be a big problem. Okay, so we just do the best we can.
Host : So with some of these kids, we've been doing this here at Cook Children's for about 20 years now, have you followed any of the kids into adulthood to see how their outcomes are thus far?
Dr. Wilson: So most of our kids are too young to be of an age where you think that they might start having symptoms. The other thing that I can't tell you is that once they leave Cook Children's into the adult world, whether they continue with that or not, hopefully they will, we again, try to provide them enough information. And we even provide documentation for their adult physicians as to why we think that this is important and what's been offered to them to that point, and the results of that. But there was recently an article published in the New England Journal, which is the premier journal for medicine, right? So you have to be pretty good to be able to publish in that journal. Wasn't our publication, but it was a group that actually has been treating children starting in childhood with statins for about 20 years. So it was very interesting, because these kids all had genetic definition of their hypercholesterolemia, so they had mutations, we knew exactly what was wrong with them. So the recommendation was appropriate. And these kids were treated consistently with a statin drug for 20 years, the researchers compare them to two groups. One is their parent who has fH, because it's usually inherited by one parent or the other, called autosomal dominant inheritance, that parent has usually had some sort of event. So they have clogged arteries, and they're either having angina, which is heart pain, or they've had a heart attack, or unfortunately, sometimes they've had a fatal outcome. So if you followed those adult patients out about 40-45 years of age, about 15% of them have had some type of event. If you follow these kids, their children who also have exactly the same disease because they have the mutation, and you've treated them and lowered their cholesterol by the time they get to a comparable age. So these kids are now 40 to 45, right? They have zero, they've experienced zero, this is 200, plus kids, zero events. Equally important, because what cholesterol does is accumulates in the blood vessel wall of arteries, it's kind of like ring on your bathtub, right, it just kind of keeps the, it's a bad analogy, but it's something everybody can relate to, right. So you just kind of get this cholesterol build up and the lumen or the way the blood flows through the blood vessel becomes smaller and smaller until it finally clogs up, right. But when you look at that, you can do it through like ultrasound. If you look at that in these children in this study, and you look at their sibling, their brother or sister who doesn't have fH, by the age of 40, or 45, they all look the same. They have no evidence of build up of cholesterol on their blood vessel. So that's very reassuring for all of us, in terms of what we're doing, I think it's very effective in keeping these kids from having problems when they become adults.
Host : For children and adults who don't get screened, so they don't know they have this issue, what are their risks? And what are the possible outcomes if left untreated?
Dr. Wilson: So if you have this condition, which genetically causes your bad cholesterol or LDL cholesterol level to be high, over a lifetime, there is a phenomenal increase in risk of having a heart attack prematurely. What do we mean by that? We mean men are going to have a heart attack by prior to age 55. And women by prior to age 60. Plus, they may actually have ill health or symptoms related to reduced blood flow to the brain or the heart. Now remember, people who are less than 50 or 60 years of age are usually still in a prime, right? They're still working or enjoying their families or whatever they enjoy doing. But most people are very functional at that age, physically and cognitively. So we want to try to maintain their good health so that they can enjoy life, enjoy their families, enjoy long longevity. But unfortunately, we do now see, we still see a number of children brought to us whose parent, sometimes the father, sometimes the mother have had a fatal outcome. So if your spouse has just had a fatal heart attack at 42 years of age, you want to know why. And then the surviving parent then asks the questions, What about my children? Are they susceptible to the same thing? And lo and behold, those circumstances sometimes the kids are tested for a known risk factors like high blood pressure, diabetes, cholesterol, low and behold, they have high cholesterol. So in kind of a reverse way, you figure out that that spouse who died at 42 years of age actually had hypercholesterolemia, or fH, right? Which is tragic, because if we'd known that, like in our screening program at age two, we would have done things to prevent them from having symptoms, much less a fatal outcome. So those parents are usually very keen on getting kids treated. They don't want the same outcome for their child that they experienced with their spouse. And that doesn't have to happen. But we have to get really good about screening all children and giving appropriate advice about those who have lifelong elevations of cholesterol. We're getting better. We're doing reasonably well as an institution at Cook Children's. Nationally, we're not doing so well. So if you looked at everybody in the United States who who has fH, we've only identified 1%. That's tragic, we need to do a much, much better job. But in order to do that clinicians, physicians need to get busy about incorporating that into their screening program. But I think we also need to do a better job in terms of informing family members so that they can also be advocates. We tell families, you know, all the families we meet, if your pediatrician hasn't screened your child, and it's time to do so, bring it up to them. They're not reticent to do it. They just simply need a reminder sometimes, because there's a lot of things on that pediatrician's plate these days, you know, gun safety, avoidance of smoking. a whole variety of things. So don't be hesitant to partner with them in terms of saying, Hey, is it time for my child's cholesterol test to be performed?
Host : So let's say someone is born with this, and they don't know it, and then they find out because their child has it, and when they go get tested, of course, they they have the issue, can you reverse the damage at all? Or is it like once they start taking medication, or are they just kind of at this point, they may have to have surgeries or other things to help them along.
Dr. Wilson: So it depends upon how far they are into this process. Okay, but here's an analogy that I try to use with some of the families, let's take a rubber band, you can stretch that rubber band so far and it'll break, okay, so if you don't pick this up, someone could have a fatal outcome from a heart attack or stroke, right. But you can also take that rubber band and stretch it to where when you release it, he won't go back to its elasticity. So if you allow this condition to go on, and don't detect it, say until late 30s, or 40s, you may stabilize that, but the rubber band will never go back to its elasticity. However, there is a period of time below which you can actually reverse the whole process. So for example, there are a lot of studies of kids who are 12 to 18 years of age where you can see a buildup of cholesterol in their arteries, and you start them on a statin and reduce their LDL cholesterol levels, it goes right back to normal, just like the study I quoted from the New England Journal. So that's very reassuring. But that also sort of says we need to be screening people early.
Host : So in presentations, and in your writings, you talk about cascade screening, what does that mean?
Dr. Wilson: So cascade screening is just a fancy term that says we need to go find everybody in this family who has this condition, because it's, again, it's an autosomal dominant condition. So it means this is the kind of genetic problem that's passed from one parent, could be both. But it only takes one parent having this condition that passes it down to 50% of their children. So if you think about the math, there's a huge case detection. So when you find a child, you know that half their siblings and one of their parents is also going to be affected. So whether those kids or the parent had been tested, we need to at least suggest to those parents that both the children and the parent, go get tested. We actually offer the testing to the siblings if the parents are willing to do so. So we try to take that responsibility and follow up on it. We don't take care of adults, so we have to encourage them to do so. But we've also identified several places around town where testing can be done at low cost. And they can be given the information to take to their primary care physician, for example. Or, if they have a primary physician, we usually suggest that they bring it up at their next scheduled visit. It also helps us in reverse in terms of understanding the potential gravity of their child's condition.
Host : So mandatory screening could have a kind of reverse cascade because a parent who may have never been screened, because they might be unaware of their own condition or family history, they could then be referred for screening, ultimately saving lives and certainly lightening the burden on the health care system overall. Correct?
Dr. Wilson: Correct. And let me go back to what you said at the first of this podcast. cardiovascular disease is the number one health condition that causes people significant morbidity, meaning they're suffering from the condition and death. I mean, we think about other conditions that are more dramatic, perhaps not any less important, but if you just looked at numbers-wise, heart disease is what's going to get most people in the United States into trouble. But with early screening and effective intervention, sometimes requiring medication, not always, but many times for these genetic conditions requiring lifelong lowering of cholesterol levels, that dynamic, at least in the United States can change. So in the future cardiovascular disease as far as an outcome for adults would take a backseat. I mean, it would be we could almost eliminate acquired cardiovascular disease that way. Just think about it, that's phenomena that needs to happen.
Host : It's huge. So what role should both pediatricians and adult primary care doctors play in screening and prevention for their patients?
Dr. Wilson: Well, people who are in primary care, first of all, they have my gratitude because they have a difficult job. They see lots and lots of people but they're also the forefront for prevention. They know this very well. What they have to consider is the recommendation and make sure first of all that they agree with it. If they don't ask questions, let's debate it. But I think that conversation has been debated pretty effectively on a national international basis. So all the all the healthcare organizations such as the American Academy of Pediatrics, the American Heart Association, the National Lipid Association, they've all pretty much endorsed routine screening. But how one does that in your office that conversation needs to be had. And then if family physicians or pediatricians need help in terms of how to guide folks, or what to do with that information, that's why we're here. That's why we created the REACH clinic, we want to be a resource to the community. So we do actually see children from all over North Texas and several states outside of Texas. We also do consultation for many countries outside the United States, where that expertise may not be known.
Host : And that's great. What are some of the new medicines and technologies on the horizon for treating kids with fH.
Dr. Wilson: So currently, for both adults and children, the first line drug is always going to be a statin. And as I said, there's about 12, or 14 of them now. So people have a variety of medications that they can choose from. So for example, if an adult can't tolerate one drug, they could try another one. But some people can't tolerate any of the statin drugs, adults, we've never seen this in children. But if an adult can we now have several other medications, one's actually an injectable drug that can be taken once or twice a month, that will lower their cholesterol level. Sometimes these are used in combination, sometimes they're used just by themselves. But it's nice that we're developing other medications that will give people alternatives. So if they're having difficulties with one or they can't afford one, then perhaps can afford another one. So there should be no no reason that people couldn't be offered affordable, available help. Which one works best for you? I think in the future, we're actually going to be able to, the term's called precision medicine, where we actually take some genetic information from you, and then look at what would be the most effective and safest drug out there. That's kind of an exciting topic in itself. But that's what's coming in medicine. But for today's discussion, what we typically do is to use a medication that's been around the longest, and also the safest, at least the information that we have, and also affordable. So there's no reason to use a more expensive medication or make it more complicated if we don't need to, because again, we want to make sure that young people, once they graduate high school, enter the military go to work, whatever they're going to do in life can still afford that medication, because it's lifelong.
Host : So what do you see for the REACH Clinic in the future?
Dr. Wilson: Well, first of all, I'm very grateful that Cook Children's, not surprised by but very grateful, that Cook Children's sees the wisdom in providing this type of a clinic for children for conditions that are not going to happen on our watch, right? These are things that happen when people become 40, or 50, as I alluded to. But I think the organization and certainly the people who work here are very committed to the lifelong health of children and their families. So I do appreciate the support that we've gotten for the clinic. As in any clinical operations, we had to do a lot of education with our community, with our community physicians, we had to set up some screening programs, we put together some educational materials for families, and so forth. And then quite honestly, our families have partnered with us to become advocates. So many of them work on the national level, some of them work locally, to try to inform other family members, for example, they may go to parent teacher organizations and talk about this cholesterol problem with parents who try to answer questions about their child being treated or screened. So that's tremendously helpful. Because there's another mother there who's concerned about her child and doing something about it, Perhaps as a mother, I need to think about my children, have them screened. So it's been a great experience in terms of a community who sees the wisdom of this, have supported us. And I guess our only problem is that we keep growing. So we've identified probably in our six county region for Cook Children's, about 1,500 kids with fH. Wow. That's the estimated population affected. We've actually identified about 350 of them. So we still got a ways to go. But that keeps growing exponentially.
Host : And that's the six counties?
Dr. Wilson: That's just in six counties, right. So that's not the state of Texas. But I think at the end of the day, we want to try to reach every one of those children, and at least have the conversation about what your risk is. I mean, we realize that we give people information and advice all the time, whether they choose to take it or not, is up to them. But it's kind of like education of a child in general, right. The investment that you make today has the potential of making huge impacts in their lives. But it may take a few years for that information to sink in. And it may take a level of maturity before they're able to act on it. So we never think that that's a waste of time. But I'll tell you, after one or two visits, those kids could stand up and give a lecture on cholesterol. They are phenomenal kids. They're just a joy. They are great kids, great families. We're just delighted that they have allowed us to be a part of their families. So the other thing that Cook Children's has done is to allow us to have a variety of people in our REACH Clinic who provide other services. So we have a full time dietitian, for example, we have a social worker, we have clinical psychologists. So we offer all those services to our family members, because sometimes there's issues about what should I be feeding my child at various ages? Is this safe to do? You know, fad diets come and go? Are dietary supplements appropriate? So our dietitian does a great job in terms of answering those questions and being a support. There are also issues with regard to finances, so our social workers are very helpful. And then our clinical therapists where it says, Okay, you've been given all this advice, how do you get the kid to do it? How do you get your kid to understand it? Right. So as parents, I think we know that too well, right. We know our kids should be doing, we've told them, now getting them to do it. understanding why that's beneficial. That's a little bit tricky sometimes. So all those people are extremely important in terms of our effort.
Host : So I like that sort of like not just the child or just even the whole family that in some senses, even the community that the REACH Clinic literally reaches out to so
Dr. Wilson: Well this has been said before, but we look at every clinical encounter, particularly new patients, we're starting a journey together, right, so let's kind of get used to each other kind of know each other, develop trust, we, we feel that that is extremely important. Learn to effectively communicate. And then as we grow together, let's talk about how we can help.
Host : So for pediatricians or other primary care providers, as well, as specialists who have patients, they think may be candidates for the REACH Clinic at Cook Children's, how should they refer them, at what ages, and how early in their care?
Dr. Wilson: Well, we encourage primary care physicians to first of all, embrace screening, because if you don't know the problem exists, you can't do anything about it. Then some physicians are actually quite conversant with this. And if they have the resources and time that they want to counsel their own patients they're welcome to do so. Even treat them if they want. At this stage of the game, most physicians are not likely to treat them, but they're welcome to do so. And I'm more than happy to help them if I can. What they are capable of doing, though, is taking the first responsibility of making sure that they've been screened, that those who need the services are provided dietary guidance, that they're encouraged to lose weight if they're overweight, that they increase their level of physical activity, that they avoid smoking, all those things that we know that contribute to high blood pressure, diabetes, cholesterol problems, and ultimately heart attacks and stroke. And at some point, if the numbers are high enough, or they're persistently elevated, and they've done all those sort of things that I'm more than happy to see the kids. If they actually screen the kids, and they have extremely high levels, but let's say an LDL or bad cholesterol level of 190 and above, we probably should see those kids early on, because we're going to do all those things that we talked about. But in the end, that's almost always going to be a genetic problem. And so we need to talk to families about genetic testing, we can interpret that for them and then kind of guide them along that treatment path. But the pediatricians, family physicians, are extremely helpful in the process. They also play an important role when kids come back to their practices. And we've provided them information about the diagnosis and our recommendations. We encourage the family members because they have a very nice relationship with their primary care physicians, talk to your doctor, your child's doctor about this, make sure that he or she agrees, ask them any questions you want, make sure that they are in agreement with our treatment plan. But generally the pediatricians, family physicians, are very supportive, and they can be a very important network for the families.
Host : Is there anything that we've missed during this conversation that you feel is important for our audience or anything you'd like to add?
Dr. Wilson: Well, this whole business of cholesterol because you hear a lot of conversations about it, but pro and con is just like any other topic, right? So there's a lot of misinformation there. And so we would certainly encourage people to ask reputable professionals such as their pediatrician, family physician, ourselves. There are some websites that are good information, sound information based on science. For example, the fH has a foundation where they have a website, Cook Children's has a lot of information available on their website. So go to reputable sources. Realize that some people are confused about this and provide misinformation. So we don't want to use scare tactics. We want people to use informed decision. But realize that if you come to see us or if you go to see your primary care physicians, what we're trying to establish as a personal relationship with you and your family, and then we want to try to help together make the best decision about your children and their future. So that's what we're really all about is trying to keep kids and families healthy and happy and enjoying life.
Dr. Wilson: I know you're super busy. So I really appreciate your taking the time to talk about this fantastic program. Thanks for all you do and for making a difference in the lives of kids not only here at Cook Children's but kids everywhere.
Dr. Wilson: Thank you for the opportunity. And let me first and foremost thank the families that have invested the care of their children in our clinic. It's an overwhelming responsibility sometimes but when we really enjoy you know love to see the smiles on kids faces as our members improve. This is not the kind of condition where you feel better or feel any different but just the smile on their face when they see their numbers reduced is a joy. So thank all of our families for the opportunity and thank cook shoulders for the support that they give us.
Host : It's been a pleasure. We're so glad you could join us today. If you'd like to learn more about this program or any program at Cook Children's, please visit us at Cook Children's dot org.