Chapters Transcript Video Keynote Presentation: Lipid Management in Pediatrics Thank you so much. I really just so nice of you to invite me. You guys have been so warm and, and it's just been wonderful being here. I really appreciate it and I love this town, the city. It's amazing. I wanna come back and bring my kids very, really great. So, um I'm gonna talk today about dyslipidemia, how we're gonna diagnose and manage in Children and adolescents. Um Our learning objectives today are going to be just to describe the NHL B I screening guidelines. We're gonna talk about some diet and lifestyle treatment options for Children and adolescents with this lipidemia and talk about how to identify which patients would qualify for statin therapy and how to initiate statins and then discuss treatment strategies for patients with high triglycerides. So I wanna first start out um with a couple of clinic clinical cases. So these are representative of patients. These actually are two patients of mine, but very representative of what we see in lipid clinic and probably you might see in your practice. So the first is a 13 year old male who was screened due to a family history of high cholesterol and early heart disease. Dad has high cholesterol, had an M I at 41 had bypass. Dad's, um, mom's maternal grandmother had high cholesterol and coronary stents at age 52. And he has an older brother who's currently treated with a statin. The next patient is an eight year old young lady who was screened due to increasing BM I over the past two years. Dad's overweight. Mom has a history of severe obesity as she is status, post gastric bypass surgery. Uh Maternal uncle is treated for high triglycerides, but there's no early heart disease in the family. Heart disease is the leading cause of death for men, women and people of most racial and ethnic groups in the United States, one person dies every 36 seconds from heart disease. That's about 655,000 Americans every year. Well, you know, why do we care as pediatricians? I mean, we now know it sounds kind of silly like, yeah, we know that heart disease begins in the young, but we didn't used to know that used to be an adult disease which is why actually pediatrics residency, we really don't even talk about this much. You know, we have to actually do additional training. Um So we know that cholesterol is the major component of advanced atherosclerotic plaques. There's a strong positive correlation between serum cholesterol levels and early heart disease as well as the number of cardiovascular risk factors increase. So we're talking uh weight or BM I uh high blood pressure, LDL cholesterol and eight low HDL cholesterol. The number of those risk factors increase. So does the severity of asymptomatic coronary and aortic atherosclerosis in childhood? Importantly, Children with high cholesterol are likely to become adults with high cholesterol unless we do something. So, atherosclerosis begins, as we were saying in childhood from the first decade of life, we have foam cells and fatty streaks. So these can actually be removed. These are not uh do not have calcium yet in them. So we can treat this if we get the LDL cholesterol largely if we can get that down. However, by the third decade of life and onward, you start to develop further atherosclerosis if we don't treat these risk factors. So this goes on to be intermediate lesions, aroma and basically can become a complicated lesion which can either obstruct or rupture and lead to heart attacks or strokes. Pediatric dyslipidemia is very common. At least 1/5 of Children, adolescents aged 6 to 19 years in this one study, um had at least one abnormal lipid level, um including decreased HDL, about 12% high triglycerides and then elevated LDL and elevated non HDL cholesterol. So in 2011, which is hard to believe it's more than a decade ago. NHL B I came out with screening guidelines. So they were these age specific cardiovascular risk reduction guidelines and went over all of these high risk features. Importantly, for lipids and lipoproteins. Universal screening was the big change and the rationale behind this was actually many fold. Um As I was saying, we now know that early atherosclerosis exists in young patients with elevated cholesterol. The previous guidelines had come out in 1992 and things had changed in a couple of decades since we now have early uh effective treatment for youth with uh cardiovascular risk factors including statin therapy. We now know the lipid disorders are common in Children. And since that 1992 guideline, this overweight and obesity epidemic, um that was, you know, increasing. And we also have shown in many different articles that screening with family history alone, which was really the main reason we would get lipid panels if there was a positive family history, it misses a good proportion of Children with dyslipidemia over 30 to 60% for many reasons. Um You can have a patient who's adopted, you can have a patient who um they don't know the other side of the family. Um You would be surprised how many parents don't know their cholesterol levels. They're bringing their child to me with high cholesterol and you ask them and many have not had their cholesterol levels checked. They don't know, maybe it was done in childhood or the common one is um my spouse often the the husband sorry, doesn't go to talk and so we have no idea. Um And I also always say this too you know, a young parent or someone in their thirties, they might still have early heart disease, but their heart attack is gonna happen at 41. You know. So we just don't know it anyway. So long story screening does not work with family history alone. The other thing that changed for, uh pediatrics was the use of non HDL cholesterol. It's very easy for us to calculate. It doesn't come up on that lipid panel that you get. But it's very easy. It's your total cholesterol minus your HDL. Basically, you're removing the good cholesterol and now you're left with all the bad, the apo lipoprotein B containing lipoproteins. All those bad ones are left in adults, non HDL. Cholesterol is the better predictor of cardiovascular events in LDL and in Children. It's been shown that both non HDL and LDL cholesterol are equally good predictors of adult lipid levels. And I should say importantly, it's accurate in a nonfasting state. So it's very easy if you have a child in your office and it's three in the afternoon, you can give them a slip and say, hey, you can go either you have a uh in your office, you can do it or if they're going to lab core request, you can give them a slip and say it doesn't have to be fasting, go take it. Um, and that's fine. You can use that. You don't have to look at the triglycerides, but you can use the non HDL from that. So let's go over when and how to screen for dyslipidemia. So, less than age two, we don't recommend any lipid screening. Very, very rare cases. So I would just say don't do any lipid screening largely because breast milk formula, whole milk, they're all very high in saturated fat. So kids LDL clusters are going to naturally be high and it does not mean these are gonna be kids with high cholesterol. So I would just, you know, stay away from that between ages 2 to 8. There is no routine screening. However, and I'm gonna just mark off what kind of you might as pediatricians might see. Um, if you get a history. So this is important when you get the history, a parent with high cholesterol premature heart disease in first or second degree relatives, that's less than 55 year old for a male, less than 65 for a female. So grandfather that had a heart attack at 78 that doesn't count great grandmother that had a heart attack at 63. Well, that's interesting. And I do you know you think about it, it, it just doesn't count if no one else in first or second degree. Um Is there the patient has type one or type two diabetes, patient has high blood pressure. Importantly, BM I of 95th percentile or greater, I think that's probably gonna be the bigger reason to screen. Um, and then second hand smoke exposure. The later ones are we should as a subspecialist be taking care of, of those patients. So our first time we're gonna do universal screening is between ages 9 to 11. This age was chosen because it is tends to be a very stable time for LDL cholesterol during puberty, total cholesterol and LDL cholesterol can decrease as much as 10 to 20%. And honestly, I just had this patient this last week whose cholesterol dropped LDL, cholesterol dropped by 90 points in puberty. And now it's actually coming back up. I was, I mean, it was surprising. I actually thought it was an error, but I think it's real. Um So for girls, I would actually aim it on the side of like the nine and even maybe eight, you know, um and I think boys in general probably, you know, 10 or 11 is gonna be fine. But for girls, if you do an 11 year old girl, often you're gonna be catching them in puberty and you can get fooled. I mean, you have a kid who LDL is 128 and you check it again. I've, I've seen this time and time again when they're now 16 or 17 and their LDLs are 100 and 65. So it is important if we can try and capture before puberty, that would be great. Um If we get a nonfasting lipid panel, I'm saying what the guidelines say they say to obtain a fasting lipid panel twice, but at least just once more. Um, if the NON HDL is 100 and 45 or greater or the HDL is less than 40. So if you get a fast, a nonfasting lip panel that has a normal NON HDL, less than 145 with a normal HDL. Don't need to recheck. You don't need to do anything more. But if the NON HDL is high and or the HDL is low, you can get at least just one more fasting panel. I know like you're not going to go poke this kid three times now. So at least one more is reasonable. If you get a fasting lipid panel for your screening, you're going to repeat. If you have an LDL of 130 or greater, a non HDL of 145 or greater HDL, less than 40 then higher triglycerides, the timing of repeating once again. So the guidelines two weeks to three months, honestly, if the LDL is 100 and 32 I, I would probably wait a year. I, I don't think there's, you know, six months to a year. I don't think there's any rush. If the triglycerides are 800 I think you're gonna, you know, gonna want to recheck those in a few days. So I think you gotta, you know, you can kind of take a look and decide when you want to recheck. But I think especially when there's anything that's very significantly wrong. I would err on rechecking pretty soon to see if it was a lab error versus real. So, between ages 12 to 16, there's not gonna be any routine screening. But if new knowledge of a cardiovascular risk emerges and you are going to get a screening, um, lipid panel, so, you know, they come into the clinic and say, oh yeah, you know, um, dad had a heart attack two months ago. Ok. So now, you know, especially if we miss that first screening. Definitely make sure we get another lipid panel and then we're gonna do our next lipid screening between 17 to 21. So you get your 1st 19 to 11. If that was normal, you're just gonna recheck it 17 to 21. I think a really nice time is kind of before they go off to college. Maybe that junior senior years seems to be a good time to capture them. Um Same um time you're gonna recheck uh same levels at 17 to 19 when you get to 20 to 21 you're now in like the adult category. So we get, you get a little more leeway. So you're nonfasting with the panel. If you do it, you're gonna obtain a fasting the panel again. If the NON HTL is 190 or greater um still HDLs and 40 or you're gonna, if you get a fasting lipid panel, you're gonna repeat if the LDL is 160 or greater, which is pretty high. I think it's pretty high. Um, when you get a little more leeway with your triglycerides as well. So, I just wanted to give you guys this, this is kind of shows our lipid levels in Children and adolescents. Um, I don't care so much about your total cholesterol. Your LDL, you know, 130 is kind of our cut point there. Not HDL 145 triglycerides, which we'll talk about. You know, I give a little bit wider berth than this, but either way they should be somewhere less than 100 or less than 130 then HDL of 40 less than 40 is considered um low and then young adults, um you know, your LDL, you get up to 160 normal though is less than 120. Your non HDL over 190 is considered high. Trigly, starts up to 150. So I, I put this in about lipoprotein little A some of you might start seeing this in your practice families coming to you asking about checking for it. I definitely get some emails about what do I do when, when do I check? Is this? What is this? Um So lipoprotein little A is just a atherogenic LDL like particle. So, lipoprotein is like LDL, low density lipoprotein. It is a fat in a protein just like the LDL or an HDL. But this one is similar to LDL. It's made up of a single molecule of an Apo lipoprotein B and it's covalent bound to this apo A tail. The length of which actually determines erogenic. It has some similarities to LDL but actually is more erogenic and it promotes both inflammation and thrombosis. So, I've seen some families who have stroke with this and some with um early heart attacks, levels of LP A, they're very genetically determined and they're inherited in osm of dominant fashion. Um, so they're about 90% genetically determined, meaning diet and exercise for this isn't going to make any difference. Um, so usually you check it once, if it's high, it's high. If it's normal, it's normal, you don't need to keep rechecking it. Um, and then importantly, you know, there's been more interests over the past decades. You know, several studies have established LP A as an independent and likely causal risk factor for atherosclerotic cardiovascular disease. My issue I have with going around and screening everyone is we don't have treatment for it right now. So, you know, ok, it's high. Well, what are we going to do as of now? We really work on getting all the other risk factors down. So, getting the LDL down and we'll talk about when we would start statins with these patients. Um, and I really use family history to help guide with this. There are a lot of families who have high L high LP A and have no issues. And so family history is very important for, I think with people with high LP A. So this came out in 2021 expert position statement for screening. Um and it's very similar to kind of when you would screen for lipid honestly. Um For primary care providers, I, you know, one or both parents to have high cholesterol. I, to be honest, I don't know if II I would screen an LP A but it's ok. I mean, I think you can, but definitely this, I think a parent or sibling known to have elevated LP little A and early heart disease and, and certainly if they're adopted, I, I think it's really important when you get that history of, yeah, you know, my husband or the grandfather had a heart attack at like 38. But yeah, the cholesterol, their cholesterol is pretty normal. And that always in my mind, I always think, have you had your life of protein? Little A checked? And surprisingly, a lot of these adults haven't even yet. So, but that should always kind of ring a bell. Like this might be LP L A and not, you know, some really high cholesterol. All right. Let's go back to the clinical case. So this was that first patient that 13 year old male, um, who had the strong family history of high cholesterol, he had a very normal physical exam, he was just into puberty. Um, he had very high cholesterol this, we definitely got two of um, once again when you find something that's, you know, very abnormal, even though this family history, it's not surprising, you know, I definitely would always want to confirm. Um, his total cholesterol was 444. Um HDL is very normal at 64 member. Over 45 is, is normal. His LDL was 366. I remember normal less than 110. So we're talking about three times normal, triglycerides were very normal. Member. Normal is less than 130. The non HDL is your total minus your HDL was as we would expect. Very high. 380 I always check thyroid. You can, you, I have had a small handful of patients sent to me with hypothyroidism and they can have very significant dyslipidemia like even worse than this. Um And you have to treat the thyroid once you do almost always, their cholesterol is normal. Um I, I don't always check UAs anymore. I used to do it more for nephrotic syndrome. Usually I feel like they have, you can look at them and you can get symptoms. Um, but I always check certainly glucose and I'm not sure why we got an insulin on him. He's not obese. But anyways, we usually check, we might check insulin and hemoglobin A one C on other kids. Um So this patient has FH I mean, clinically has FH by definition his LDL levels and um the family history, you guys will see this in your practice. Absolutely. You probably have several patients in your practice with this. Um, it is about one in 200 to 1 in 250 people in the United States for heterozygote. Homozygote. That's when you be, each parent is a heterozygote. Right? And one in four chance of their child having it. So, the child has two gene mutations. Um, it's about one in 100 and 60,000 worldwide. Um, when I was training, uh homozygote were about one in a million. And heterozygote were felt to be one in 500. But we have largely to through cascade screening and through honestly better screening of Children and adults, we've found that it's actually much more prevalent than we thought. Um Mutations can be found in many genes. Most commonly is the LDLR mutation. I tend to find higher um LDL levels in LDLR mutations. A OB mutations tend to be lower. I tend to find my LDLs in the 160 to 180. It's kind of interesting. Um We do genetically test we use in VT. Um I don't genetically test everyone. I offer it to most people, a lot of families. They say, you know, it's not gonna make a difference. Yeah, I mean, like this one, we did genetically test but you know, would make no difference in treatment. Sometimes I find genetic testing to be very helpful, especially in patients who are on the border or especially adopted patients where we don't have a family history and like you're deciding about starting medicine or not, um, heterozygote what will tip you off. Usually the LDL cholesterol are going to be over 160. I have a small handful that are in the one fifties and one forties, but generally 160 usually even much higher than that. The reason why we care and it's super important for us as pediatricians to identify and treat these patients. If we don't treat this men by age 60 nearly everybody with, with heterozygos FH will have had 80% will have had some sort of heart disease and women about half by age 60. So this is a comparative on us to start treatment um during childhood homo zygotes. Um this is a, it's basically a different disease. Um It is, it's treated, it's, it's treated quite differently and much more aggressively. Um These patients get treated on diagnosis as young as we can to whenever, I mean, you know, 23, whenever they're diagnosed, um they're on statin, they're on multiple medications in, in injections, IV infusions and um starting to not effuse apheresis as much due to new meds. But anyways, it's a whole different disease if you guys. Um and I'll show you actually in a minute, I'll show you if you ever see these to send to us. So signs that might raise the suspicion for FH Now the, the trick is in kids, you're really not gonna see this much, you might, so a parent who's been untreated, who has heart disease, you can definitely feel, um, their tendons, like go, go down and feel their achilles. Tendon, feel yours unless you have FH, but you could feel yours and feel theirs and you'll notice a big difference in kids. A tip off. Yes, they might have tendon Xanthoma. But if you see a kid who has xanthoma, so they're on pressure points. So you'll see them on elbows. So if you have a homozygous, see them on elbows, knees and buttocks. Um there are some other rare diseases, not just homozygous FH where they do get um xanthoma such as Cyto. So if you ever see something that you think is a xanthoma, please get a lipid panel. A couple of my homozygos end up going to dermatology to get biopsied. And that was a wait, it was like a six month delay. And then finally, you know, the biopsy showed its cholesterol and so they end up getting lipid panel. So if you see it, please just grab a lipid panel first. Um Xanthe Asmaa, I, I, I've only seen it in an adult with low HDL cholesterol. I think, I agree. It's very low specificity. Corneal arcus is super interesting. I've seen it in a couple of, of the adult patients. Once again, I've not seen any of my homozygos. You're not going to see it, you know, in pediatrics, but once again, the parent you might be able to see. And it's kind of a neat physical finding that I feel like we as pediatricians don't, you know, don't see so much. So, how do we treat? So, in pediatrics, the nice thing is, um, I feel like it's great. We don't always just throw medicine on, on them as, you know, in adult medicine, it's often, you know, here's a little pill for this, a pill for that, you know, in pediatrics. I think we all try to stay away from medicating the Children unless they certainly need it. So we always are gonna do, I call them the three Ds or a new diet doing and then we have to talk about drugs in some people. So shit is that we're not, maybe we don't have any, maybe no noise. Ok. Well, Melissa mccarthy telling him that she's going to take away the barbecue sauce because he has high cholesterol anyways. Um It was very cute. I found it. I was like, this is great. Um ok, so um the guidelines say so for Children with dyslipidemia overweight or who are at risk, I, I put the guidelines up but I kind of dislike them because I do not want my parents, I do not want my child, the Children, especially the adolescents. I don't want people like counting grams and like, you know, percentages. I feel like it starts to lead to a lot of, you know, too much focus So we have, I, I, you know, these are my things. We're gonna add one fruit or vegetable to every meal and snack. So great. You eat cereal for breakfast. Let's add some half a sliced banana on top or you know, throw some blueberries in or whatever we kind of talk about like practical ways of getting like fruits and vegetables into the diet without being like, you know, you need uh age plus 5 g a day of, of fiber. Another thing that we do for fiber, 2 to 3 g of fiber per serving or slice. That's a very easy way. It's like when you're looking on the nutrition label, OK. This has got a little more fiber than that one. I'm gonna choose this one and then saturated fat, we're gonna aim for less than 2 g of saturated fat per serving or slice. So it is not a low fat diet. I have some families, they come in, they like, you know, cut everything out and the kids are literally eating apples and you know, rice cakes all day and the kids already now lost £8 and you know, it's like, no, we don't like that's not what we want and they need fat. Um So we encourage, you know, healthy fats and just kind of lowering the saturated fats. Now, if they have um continue to have high LDL cholesterol, um I think, you know, meeting with a registered dietitian is super helpful if you're able to, I know it's often hard. We're very fortunate we have one with our clinic. So they meet at the first visit and if needed after I know it's, it's not always, but it is very helpful. Um, especially I think the families that have like, really kind of overly restricted the kids. I think it's very helpful to hear what they really need. Um And, and additionally, especially in my athletes um who come in with high cholesterol and like figuring everything out and making sure that they're getting enough nutrition. Um But soluble fiber is a great thing to lower LDL cholesterol. So our friend Metamucil psyllium fiber, um up to 6 g a day for Children, up to 12 years and 12 g a day for 12 years. It's, it's, that's a lot. So always if you're going to start this, start at 3 g a day having a lot of water. And I find um so in kids who are already very healthy, like, you know, some families come in and the kids eat amazing and they, you know, fruits and vegetables all the time and I probably wouldn't do this because I think they're probably already getting plenty of fiber. But the kids who maybe are pickier who like constipation or um on Miralax. So I've had a couple of families that have actually changed over to Metamucil from Miralax and it's worked really well for them. So, um just an idea to get some more um soluble fiber and you can lower your cholesterol that way. And then there's the foods as we know that obviously are higher in soluble fiber. All right. So now we're gonna move on to clinical case two. This is that young lady who had had that increasing BM I um in the family history of obesity. So her BM I was at 100 and 22nd percent of the 95th BM I percentile. She had a normal blood pressure. Her exam was significant just for an innocent heart murmur. Um Most recent labs that we had our total cluster was 213 or HDL was very low at 27. So remember over 45 but I at least like to see a four, at least a 40. You know, that's, that's what we like the 27 I always thought, think when I see in the twenties or some genetics to that LDL, mildly elevated at 140 triglycerides mild to moderate at, you know, at 229 or non HDLB was elevated. So these cases, non HDL cholesterol is very useful and I, we'll go over in the end when I show you her different cholesterol levels. So since NON HDL is a very good marker for like kind of cardiovascular disease risk, it's a single value that you can follow over time. So it always gets hard, you know, you see, oh your HDL went up but your LDL went up and your triglycerides dropped a little bit and the next time, you know, and like, how are we assessing risk with, like, you know, they bounce around, of course. And that NON HDL number is a great number to track. So I can tell them I, I always have like a little, you know, chart and I'm like, look, your NON HDL went from 176 to 168 and now it's at 148. That's amazing. You know, because the parents come in. Oh, but the triglycerides went up. I'm like, yeah, but look at all this. So I, I think it's a really great value and I wanted to show this for FH patients and patients high LDL. It's gonna correlate with the LDL. It's not as helpful, but I think for these patients especially, it's really nice. Um, her other tests were all normal. So she probably has combined dyslipidemia of obesity. You guys are for sure, seeing this in your pediatric practice. Um, we have a lot in our lipid clinic as well. Um, a lot of, you know, the high triglyceride, low HDL, often, like normal to mildly increased LDL cholesterol. I have a lot with very normal, very normal LDL cholesterols. Um, it's really a lot of diet and exercise. Exercise is so important for these patients. Um, you know, in some families, we talk about pharmacotherapy as they get older if like, they're not doing anything. And there's a family history of heart disease. We might discuss statin, but I really try not to treat these patients with medicine. Um, for kids with high triglycerides only or even with mixed dyslipidemia. The most important thing is this limiting simple sugars and carbohydrates. So, when you're asking about diet, what do you eat at school? Because parents? Oh, no, we don't have, we don't eat at home. No soda, no juice, you know, they drink water, whatever it is, water and milk. Oh, but what are you having at school? So in, in Pennsylvania we have free breakfast for everyone. I don't know in New Jersey if you guys have that, but we have free breakfast. So a lot of kids now are getting breakfast at school and lunch at school. So most of their meals are actually outside the home, right? Um, and what comes with breakfast is obviously a juice because that counts as the fruit, right? And then lunch as like my kids tell me, well, lunch, they don't even give water. You can buy the water but they'll give right? The flavored milk and everything. So I always ask. So what are we having for? Oh, ok. You're having that and then you're having that. So you're having two sugar drinks a day actually. So like we work with, ok. What can we do? Can you cut out the one at least let's get down to like the one small chocolate, four ounce chocolate milk. That's probably ok. But let's really work on maybe the breakfast, either you can bring your own water or choose the plain. So, it's, um, getting into that also, we kind of talked about getting that history, like, really asking that diet and that, but that one is a really easy one. Easy, but it's an easy one. I feel like to take away. So it's always hard when they're like, no, no, we drink water all the time and you can't, you know, there's nothing to do. But this is always a nice one. If they are doing that, you can um remove that from their diet. Um And then certainly weight loss or weight stabilization helps hydrate glyceride. So really as our kids are growing, you know, if they're kind of stable, I don't ever, I don't really like the weight loss. But like, you know, if you stabilize your weight and you grow a couple inches often, you'll see kind of resolution of some of this dyslipidemia and then omega three fatty acids. So I always encourage um wild salmon um or uh tuna, wild caught tuna. Um ground flax seed is great, like ultra ground flax seed, you can put it, mix it into pancakes and like when you bake some people like it in their yogurt. Um I had one young lady who liked to eat it by the teaspoon, which was I, I could not believe she was doing that, but she did it, um, it was, uh, interesting. So, treatment of high triglycerides is really high dose, um, fish oil, which this was, was about the diet. But, like, it's hard to get through diet alone and usually you have to do prescription, but unfortunately, you know, prescription, you need to have triglycerides over 500. So, if you're gonna treat, which we'll talk about in a minute, about how to treat hydro glycerides with, uh, medicine. Um, if you don't get it prescribed, it's a lot of pills for the kids and probably they're gonna be non compliant. All right. So let's talk about, um, briefly on the doings. I think this is like, actually one of the most important things, um, for our Children. Um, so all Children should participate in one hour of enjoyable, moderate, vigorous intensity activities daily and enjoyable is I think that the key and it, you know, it kills me because what do our kids do as they get older? Right. We get to middle school and high school. What do they do? They take away the recess, they take away their gym class and it's, it's really rushing, I think, especially for the kids, um, who don't do sports. You know, it's like we've taken away and then we wonder why everyone's getting obese. But that's a whole other thing. But, you know, I think we have to encourage as parents, we do the best we can is getting them out and I don't care, going for a walk, playing with your friends. I don't, you know, it doesn't have to be some formal, um, exercise program. Um, but I will say so my daughter, I'm gonna show you a picture, uh a picture of her. So she, we tried everything with her growing up. She didn't want all any sport. And finally she found cycling. So I showed a picture of her. She raced, um, this last summer, she raced with the women. She comes in, she came in second. It was like our local in Ardmore. I know if you guys know Pennsylvania, but it was so great. It's really wonderful seeing, you know, when your child actually finds something that they enjoy. And that's really what I think is the important thing. Like you can't push them into doing it, but like, let's find something that you like to do. Um, and then drugs, I mean, so then, you know, kind of what we have to do sometimes. So when do we consider medications, um, for high LDL cholesterol. So generally after, you know, diet and lifestyle and then if you're, if they're 10 years of age and older or eight, so the reason I put the eight is, um, definitely there's a very strong family history of heart disease. So if you tell me that, you know, I've got many families where the mom or the dad, um, has had heart disease in their twenties, twenties, early thirties, those kids are getting on statins at seven, I don't care. 78, we're go, we're going on early. Those families totally agree. Like, there's a very aggressive, probably g mutation that they have. Um, otherwise if there's no family history of heart disease or, you know, 52 year old grandfather had a heart attack, I'm fine, you know, fine with waiting until 10. Um, so generally it is around 10 and statin is the first line therapy. Um This came out in 2019 as a scientific statement, cardiovascular risk reduction in high risk pediatric patients and they stratified um by risk. And I really like these categories. So we have this high risk, moderate risk and at risk, high risk. Certainly the homozygous FH are diabetics and then a lot of the, you know, um subspecialties at Kawasaki with persistent aneurysms, but I thought was very important for the first time. They actually split out severe obesity from obesity and this was done for a reason. So severe obesity is defined as greater than equal to the 120th percent of the 95th BM I percentile or an absolute BM I of 35 kg per meter squared or greater, whichever one is lower. Um based on age and this is in alignment with class two obesity in adults, which is considered a high risk category of obesity associated with early mortality. And as I'm sure we all would agree, the £380 kid you know, the BM I of Lord, you know, 8070 is very different from like the kid who's a little pudgy and like, you know, is gonna go through his growth spurt soon. So I think, and I'm very happy that they separated these out. I agree a very different kind of way of managing these kids. Um in the moderate risk category is the heterozygote, you know, a patient being treated for hypertension like a protein little A goes in the moderate risk. Um And then at risk, um I like that they put white coat hypertension, which actually I think is interesting. Um and then some of the other other um cardiac diseases and cancer. So um initiating statins um by risk level. So once again, you've got high, put over here, high, moderate and at risk. So that high risk category once again up here. So, you know, for what you guys might see like dia diabetics really, um I mean, we should be treating the diabetics, I mean, the the or the endocrinologist. But really that LDLC point for these is 100 and 30 which is not that high. I mean, that's, you know, most of the time I look at that and I'm like, uh you know, big deal. Your LDL is 100 and 30. But for these patients, you know, uh diabetes is a coronary arteries equivalent. And so it's very important. So their treatment goal is an LDL less than 100 in the moderate and at risk, you know, we're gonna take some more time. You can take three months at least, I mean, usually more time to be honest and then the at risk you can take some more time and that um, goal is the threshold is 160 or greater with a goal of 130 or less. Um, and like I said, statins are really the first line therapy, Um not gonna go into the other stuff for homozygos. Um Statins approved for Children. So we have a Tova lovastatin, pravastatin, pua and simvastatin. Um I almost always start with the Tova 10 occasionally sim for 10, especially the family members on it. Um Rosuva five. So res Suvas statin is our most potent drug. So Rosuva five is equivalent to about 30 ish of, of atorvastatin. So you get a huge bang for your buck, but I like to kind of keep that in my back pocket. Um because people might have a very good response even with very high cholesterol to atorvastatin. And then you have this Crestor Suvas in your back pocket to use if they don't respond as well as you might like, I've never used lovastatin and pravastatin. Yes, it's approved down to age eight. So is ver Suvas Statin and honestly, I use atorvastatin at age eight. It doesn't matter. Um Pravastatin is just, it's like one of the least potent ones. So I just, we don't really use it much. Um, always start with the lowest dose that's going to be 10 for most, most except for SUVA is five. And then we always check AC K level liver test. I just, I just usually the comprehensive metabolic panel that will cover the A LTAST glucose looks at the creatinine as well. And then if they're obese, I always check a hemoglobin A one C we do this because of the very rare side of, at least in, in our age group. Um, you know, you can certainly have the muscle symptoms. That's why we get the baseline CK. You do not need to keep checking CK if they do have any kind of strange symptoms and let you know, I always just say stop the statin, we'll check AC K level. I've never yet seen a high CK level, um, from it. But, you know, it's, it's, it's there, it does happen. Um, so the whole thing with liver, you guys would probably get, ah, but the liver. So, in, in the newer guidelines, they actually, in adults, they just obtain baseline liver tests after initiation and then any dose change. Otherwise they don't keep checking there. In the billions patient years, there's been no finate liver failure from statins. And actually in people with, with NAL with non alcoholic fatty liver disease, statins actually help lower the, the A LT and A ST. So I, I don't have a problem with the liver, but you know, I'll check it every year, you know, I, I'll definitely just do yearly is because, because they're kids, you know, um, you can have um, increased fasting glucose and hemoglobin A one c, especially in the patients who are gonna be more prone to it. Um, so I definitely, you know, I check an annual A one C and the kids who are obese that you, that are on a statin and then they, they can be teratogenic. You know, we, I I don't make my kids go on birth control, but we always have a discussion. Um This may be changing about the toto nature but as of now, we it is still in the, the guidance. So um does it work? You know, that's always like, oh, but you, my kid is gonna be on medicine their whole life. Well, yes, they are. You know, if you've got FH you are going to be on medicine, probably not a statin. I don't know, there are so many other drugs out there now. Um But yes, for now we are because statins work. So only in the Netherlands can you have these beautiful, you know, 20 year follow up studies. So there was this original cohort back, you know, a couple decades ago looking at um pravastatin um use in FH patients, they matched them with their siblings and they also had parent data. They followed these kids through, they started them at eight and followed them through puberty was one of the, the good, nice studies that showed that we can like start these kids young before puberty. They were able to get 8 86% of the patients um from the original cohort and they had most of the siblings, they had literally like almost all of the data on cardiovascular events in 20 years. I mean, it's amazing. Um the mean LDL cholesterol in the patients that have been treated dropped from 237 to 100 and 60 which is a, a good decrease. But I I'm gonna point it's not super low. I I was very surprised actually, you know, 100 and 60 is still to me on the higher side, but I think it's um you know, it's good to show that we had a very, very nice response. So these are Kaplan Meier curves of those treated with statins up here in the red, the Children with FH um versus their parents who did not start statins until later in life. And you can just see, I mean, these are cardiovascular events starting, you know, in their twenties, these are the parents, you know, just they had cardiovascular events and death. You know, we're talking 2530 the sign, I mean, no, no one died in the patients who have been treated pravastatin. So I think this makes a strong case. I mean, for not just the lower the better. Once again, this wasn't crazy. Low, the LDL they dropped by 30% but it wasn't down even below our guidelines of 130. But the younger, the better these kids started at a young age and I think that this really shows that we can make a huge difference in cardiovascular morbidity and mortality. Little shift briefly, further management for high triglycerides because we see a lot of this too. I feel like it's like FH, or these, like high triglycerides and then, you know, you get the little bit of like a mild stuff but, you know, it's like either of these two, so moderate hyper triglyceride. So triglycerides of 130 up to 400. So I know people get freaked out. Oh, they're 300 something with a normal non HDL cholesterol. Their cardiovascular disease risk is exceedingly low. And these guidelines kind of say that, you know, it's like reassess in a few months and then you can check them periodically. Kind of like, uh, you know, we don't care so much. We think these are probably very low risk patients alternately the severe hypertriglyceridemia when you get 1/1000. Always recheck that right away. I've seen some that have been like 2000. We check them and they're 250 I still believe it, but it makes the, like the need to maybe start meds right away a little bit less. But if you recheck it, it's still that very high, we certainly would want to start therapy right away. These patients are actually at highest risk for pancreatitis. That's the more immediate risk. Their long term risk is often actually from cardiovascular disease is actually very low. Our type one hyperlipoproteinemia patients with very high triglycerides in the thousands, they have a lot of pancreatitis, but they often they don't really get cardiovascular disease. So that's really our reason for treating these patients actually on a very pretty urgent basis. Then I want to point out this group, this is the group, I feel like very tough to treat like that. The my patient went to go over the clinical case, two patient. These are patients with like significant high triglycerides. Like, you know, now, you know, it's 708 100 or you know, triglycerides of 2 5300. But also this high non HDL and these patients, you're gonna go over and use this, these risk categories again. And basic basically depend on your risk category. It's gonna be kind of how urgent we start treatment, right? Kind of the lower the risk category. I think more time spent on on TLC. And um before starting pharmacotherapy, the um treatment goal is triglyceride, less than 150 no HDL less than 145. And I will be honest with you in the real world um that I don't um I don't probably get the trig lister that low and I think that's, I think that's totally fine. Um So going back to clinical case one. Thank you guys have been great. I know this has been a little bit long, so I really appreciate you guys um listening. So this is um our severe heterozygous FH His invita was positive for an LDLRG mutation. He had a normal lipoprotein little A I started him on a tour of a 10. His LDL dropped from 366 202. It's a very nice decrease but not enough. Went up to 20 LDL of 165 which I might have stopped in a patient without a family history of heart disease. But given that dad had, had heart disease like very early and grand grandmother. Um I wanted to go up more. So went to 40 kept LDL of 122. Um clinical case two. That young lady with um pretty significant mixed dys epidemic to high non HDL. They really worked on physical activity, took out the soda, she actually stabilized her weight and came back with this HDL. A little better. Everything was a little bit better. Non HDL was still a bit high. We put her on like 2 g of Omega three fatty acid, didn't go nuts a little bit. And really, they really worked on their physical activity, I think was really the key and you can see she has a low HDL. I think it's just genetic LDL is borderline or triglycerides had totally normalized her. Importantly, her, non HDL. Cholesterol was now normal less than 145. I think that's where we could say. Ok, I'm, I'm good. So, in summary screening once between ages 9 to 11 and once from 17 to 21 at risk at 2 to 8 and 12 to 16. Selective LP A screening for age two and older, we're gonna consider statin therapy if, despite lifestyle changes and you're eight or 10 older and your, the deal is consistently 160 or greater for most and 130 or greater for certain high risk um patients. And the me it's considered fish oil or additional pharmacotherapy for very high triglycerides or kind of the, the high triglycerides or um uh kind of a more mixed bag with a high, um non HDL cholesterol. Um I have put this in here. You guys probably don't live around here. You should of course, refer to Morristown. But if any of your patients or if any of you guys are from more like the Philly area or Delaware or whatever, we are happy to see your patients. Um We do go to many satellites and we go to South Jersey if any of you are from South Jersey. Um Certainly anyone around here go to Morristown and we also do do um telemedicine um as well and that's my email if you ever have any questions, I'm always happy to answer anything. So, thank you so much. Published Created by
