EPISODE 22 [INTRODUCTION] [00:00:00] ANNOUNCER: Do you enjoy listening to On The Ear, but wish you could earn ASHA CEUs for it? Start today. SpeechTherapyPD.com has over 175 hours of audio courses on-demand, with an average of 19 new audio courses released each month. Here's the best part, each episode earns you ASHA Continuing Ed Credits. Oh, no wait. This is the best part. As a listener of On The Ear, you can receive $20 off in annual subscription when you use code Ear21. Just head to speechtherapypd.com to sign up and use code Ear21, E-A-R-2-1 for $20 off your annual subscription. [00:00:48] DS: You're listening to On The Ear, an audiology podcast sponsored by speechtherapypd.com. I'm your host, Dr. Dakota Sharp, Au.D, CCC-A, audiologist, clinical professor and lifelong learner. While I primarily work with pediatric cochlear implants and hearing aids. I am absolutely intrigued by the many areas of audiology and communication in general. This podcast aims to explore the science of hearing, balance and communication with a variety of experts in hopes of equipping you to better serve your patients, colleagues and students. Let's go. We are live and On the Ear, brought to you by SpeechTherapyPD.com. [INTERVIEW] [00:01:36] DS: Congenital Cytomegalovirus or CCMV is a common virus that infects individuals of all ages that typically results in symptoms no worse than a cold. Over half of adults age 40 have been infected by CMV. It's really common, but when CMV is transmitted to a baby during gestation, the impacts can be severe. In fact, CMV is the leading cause of non-genetic congenital hearing loss worldwide. Surprisingly, CMV is still not commonly discussed, and many birth hospitals do not even screen for it. Today's guest is going to better explain CMV from a professional and a personal perspective and hopefully give us something we can do to fix that. Amanda Devereaux, RN, BSN has worked in public health for 12 years, most recently focusing on maternal child health. Currently, she works as the program director for the National CMV Foundation, and Amanda became passionate about eliminating congenital CMV when her daughter was born affected by the virus in 2015. Amanda serves on the board of the Congenital and Inherited Disorders Advisory Committee, and the Hands and Voices Board in her home state of Iowa. Considering June is National CMV Awareness Month, it seemed fitting, we could have Amanda join us to help spread awareness of this critically underrepresented condition. So, hi, Amanda. I'm so excited to talk with you. [00:02:44] AD: Hi, thank you for having me. So excited to be here. We're always willing to talk to anybody and spread the word about CMV. So, thank you. [00:02:52] DS: Yes, I'm excited to hear more about what you all are doing for the month of June considering that's your month to really help people better understand this. I wanted to point out when I was in my doctoral program, my class, it was kind of like medical audiology, we talked about some things we might see in a medical setting, or just more complex kind of diagnoses and I had to do a whole presentation and handout on CMV. And I had never heard of it up until that point and then when I started to read about the impacts and how common it was, related to hearing loss, how common it was just in general, it was mind blowing. It's really crazy when you meet someone for the first time who's never heard of CMV. And you're like, “Oh, well, you probably have it” or you know what I mean? It's just so common. [00:03:33] AD: Yeah. And that's my experience, too. Usually, when I start talking to people, they've never heard of it. Even people in the medical field, they might have heard the name, but they don't really understand the impact. Once they hear the truth about CMV, they are kind of flabbergasted and they're like, “Why aren't we doing more to work on this?” So, that's definitely my experience as well. [00:03:53] DS: Oh, my goodness. Okay, so I'm excited to talk about what we are going to do to help change that. But before we do, could you just kind of break down, like when you do give that explanation to people who've never heard this, because I'm going to go ahead and assume some of our listeners have never heard of CMV before. Could you go ahead and just kind of break down what it is, feel free to go as nerdy as you want. We've got a really nerdy crowd who listens to this podcast. So, if you want to get into the molecular level, if you want, like, however far you want to go, let's go. [00:04:21] AD: Okay, sounds good. So, and I won't go too far just because I might get out of my scope. [00:04:28] DS: I was going to say, yeah, probably you're going to lose me halfway in. [00:04:30] AD: So, CMV is a really, really common virus like you mentioned earlier. Most of the time, if someone has symptoms, they could be brushed off as a cold or they might not even have symptoms at all. So, very common virus, 50% to 80% of adults have had it by age 40. So, we're talking the majority of the population had it and didn't even know that they had it. And so, it's a virus in the herpes family. It's not herpes, it doesn't need to be lumped in with herpes or anything but it is in that same family. So, something that's very common is being spread around frequently, especially by children in daycare settings. Up to 80% of kids in daycare are shedding this virus. And when I say shedding, I mean, their urine and saliva and body fluids have the virus in it, and they can pass it on to somebody else. So, children are actually a really frequent source of the infection and when a pregnant woman or when anybody gets a toddler, or young child's urine or saliva, on their hands in their mouth, things like that, they are at risk of contracting that virus. And usually, again, it's not going to cause any harm. But if somebody is pregnant, it doesn't matter if they've had CMV before or not, they are at risk for getting it for the first time or for catching a different strain of it, and potentially having it impact their pregnancy. So, that's kind of the main route of transmission is urine and saliva of young children. It doesn't mean that you can't get it any other way. It's in a lot of body fluids. But that is thought to be the primary way that pregnant women are catching this virus. [00:06:08] DS: Gotcha. So, we've already got to worry about all the different infections that kiddos are sneezing and wiping boogers, and all that good stuff. Also, now they're also shedding CMV, for the most part, too. Could you break down, so that helps me better understand the transmission person to person, adult, not congenitally. But when we think of it as a transmission from mother to baby, I guess I'm curious kind of how it goes from child or adult, whatever, in the outside world to a mother, then to baby. What does that transmission usually look like? [00:06:40] AD: Sure. So again, I'm not a doctor, but my understanding is the virus can cross the placenta and get into the baby and into the amniotic fluid. So, that's one reason why we can diagnose congenital CMV during pregnancy with an amniocentesis because they take a sample of that amniotic fluid in it, it could be in there. So, it's a virus that's crossing the placenta and getting to the baby. It doesn't happen all the time. If a woman contracts CMV, for the first time, she has about a one in three chance of passing the virus to the baby. So, it doesn't always happen, only sometimes. So, there's a clear cut, 100% statistic with CMV, it feels like. There's always a chance of everything. There's a chance that the woman will pass it to the baby, and then there's a chance that that baby will have an impact. Yeah, it's always a statistics game here. [00:07:35] DS: Gotcha. So, when we think of mothers who might be more at risk, teachers, daycare center people, were you in a setting like that around children? [00:07:46] AD: So, I was to a certain extent. I'm a nurse and I was doing home visits for pregnant moms and their babies. So, that's definitely a potential source of the infection. However, we tried really hard to have pretty good hand hygiene and be pretty careful around our patients. But I had a toddler at home that was in daycare. I worked so my toddler was in daycare with other children, and they’re swapping all sorts of germs. So, like I mentioned earlier, childcare is a common place for kids to pick this up. So, the way it's been described to me is that most likely the source of my affection was my own toddler. Women who are at higher risk, definitely women who are working with young children, but also women who have young children at home and are caring for their own children, especially if those children are around other children who can give them the virus. [00:08:39] DS: Gotcha. Okay. That makes a lot of sense. They're the ones who are out there, maybe more so than you. Do you think that moms who have children in the home or who are in positions where they might be around others, just at any more risk than anyone else? Should they be taking extra precautions other than good hand hygiene? Is that really kind of the best way to combat that? [00:09:01] AD: Yes, we would say that hand hygiene is extremely important. That is one of our prevention tips. But certainly, there's other things. A lot of times, moms are really busy, if you've got a little kid and they're eating their breakfast and they don't finish at all and you just sort of – “Well, I'm not going to throw this food away, I'll just eat it myself and then I don't have to fix myself breakfast.” You're sharing cups. you're sharing spoons, utensils, kissing little kids on the mouth. Not everybody does that. But I have to admit if my toddler came in for a sloppy kiss when I was pregnant, I wasn't like, “No, don't do that”, because I had no idea. [00:09:38] DS: Sure. [00:09:38] AD: We talked about washing hands frequently, especially after diaper changes or handling maybe toys that children have been drooling on. Not sharing those cups, straws, utensils, not kissing little children on the mouth, not putting a pacifier in your mouth. That's another one. If it drops on the ground with mom like cleans it up with mouth and puts it back to the kid, that's definitely not something that we should be doing either. So, these are things that might be – sometimes people say, “Well, that's common sense, you shouldn’t be doing that anyway.” But when you're a busy mom and you're running around, and you're trying to do all these things, it's easy to forget those things, especially if you don't realize that there's a really significant consequence. I just had no idea that I could catch something from my toddler that could impact my pregnancy, my baby. Women deserve to know that, so we just really focus on educating people, and then they can take that information and do with it what they choose. [00:10:40] DS: Yeah, I was going to say all of those things seem very mundane. I definitely – my toddler’s leftovers, my littlest one, there was like a dog hair on it, and it was really stuck on there. So, I eventually just took like a wet finger, and just like brushed that off. These are like, in the moment, quick things that you don't think about at all, because they're your kid, you're like, “Of course, like, I'm going to have to deal with their spit and stuff.” You know what I mean? But when you think about the impacts, I mean, these are such easy ways for us to let things slip by. It makes a lot of sense, I would have never considered that to be an easy route of transmission for something. I mean, it's not even fair to say that because you don't even know the condition exists in the first place. You're not even thinking of anything as a transmission route. It's not even a thought in your head. [00:11:20] AD: Right. And we're spending a lot of time educating pregnant women about other things to prevent, that are way less common. [00:11:28] DS: That's a good point. [00:11:30] AD: Not that we shouldn't be educating pregnant women about all the ways that they can prevent disability in their children. But it would be nice if we proportionally looked at how common they are, and decided what to prioritize, and this would be number one. [00:11:45] DS: That’s a great point. Do we have any idea what's happening in utero when transmission occurs? I know that the symptoms are like – and I guess we can sort of break down symptoms here in a minute too. They can be pretty like wide ranging and the degree can be extremely variable. I know there's kind of like a spectrum. There's a great graphic, maybe we'll include that in the show notes that I saw, I think from the CDC, that's – okay. Well, actually, before we get in there, I guess, let's just talk about asymptomatic versus symptomatic birth. Because I think that kind of dictates a lot of what we're going to see symptom wise, right? [00:12:19] AD: Yeah. So, it's hard to say why exactly some kids are more severely impacted than others. I've read some things indicating that it really can depend on when that pregnant woman catches the virus. So, if you catch that virus, and it's gotten to that baby that's developing, when really important, essential things are developing like the brain, that is probably when we're seeing the most severe consequences. So, it's really impacting the way that these babies develop in utero. So, Miss Pippa, for example, my daughter. I know that I had a primary infection during the first trimester. So, that's when her brain was forming. That's why she has a brain malformation, because when that was happening, the virus was in there causing issues. Now, I can't get into exactly how and why that exactly happened that way, but there is thought that maybe some of the timing of when you get the infection, can determine the consequences. So, we do kind of group infant’s diagnosis CMV at birth as either symptomatic or asymptomatic. There's a little variation sometimes, and maybe just a little friendly discussion about what is asymptomatic and what is symptomatic at birth. It's really difficult because 90% of kids look perfect at birth, and even if they had some subtle symptoms that could be missed, because some symptoms you can't see from the outside just by doing a newborn exam. Some infants about 10% have more obvious symptoms and so they might have a small head size, they might have a – we call it a blueberry muffin rash, where they've got kind of these purple splotches on their body, lumps on their body looks like a blueberry muffin. They might have a large liver and so maybe their abdomen is big. They might have low platelets, or some other things going on with their blood work that isn't quite right. But again, some of these things you can't see. They also might have issues with their brain. So, if a baby had an ultrasound of their brain, of their head at birth, we might see calcifications or some other things. So, those are symptomatic babies. But again, we don't identify at this point, even symptomatic babies were missing the majority of them, because their symptoms are really subtle, and even if they have symptoms that we can see, sometimes, even if you've got these symptoms, people don't think CMV, even people in the medical field. So, even babies with symptoms are being missed. [00:14:56] DS: Wow. I actually have a child on my caseload right now who we think was a missed symptomatic birth with CMV. We haven't locked that in yet. And I think they're working on going back. Maybe they have her bloodspot on file. They're sort of in the process of moving too, so I'm not too involved in what the changes are going on there. But yeah, it's kind of strange to me that there are some visual indicators that birthing hospitals could be looking for. The blueberry, is that called petechiae? Or am I mixing that for something else? [00:15:23] AD: Yeah. [00:15:24] DS: Okay. I couldn't remember if that was something else. I mean, that's probably not that common of a thing to see. You might think, “Okay, we're seeing this”, we definitely want to screen this baby. Although you're right, I think you said, 90% are going to be asymptomatic in the first place. That's really going to make it tricky to nail down if 90% of these babies were not even seeing in the first place. I think I remember learning some of these symptoms, or I guess, maybe not the symptoms, but definitely the hearing loss can kind of fluctuate back and forth to and can be progressive so they can start with normal hearing, and then that can progress to be worse, too. I'm just interested if there was anything, when your Pippa was born, if there's anything that jumped out that was a red flag to the people involved with her birth, or was it something that came up later? [00:16:08] AD: Sure. So, she's kind of a rare case, a more rare case, because we had findings on ultrasound. So, we knew to expect her to be born with CMV. We had an amniocentesis halfway through the pregnancy. So, we knew that she had CMV, and most people don't have that knowledge. So, right when she was born, they were starting treatment and intervention and checking everything, that could have been impacted, because you can't see everything on ultrasound. So, they were checking everything just to make sure that she was getting the best care possible. What they saw on ultrasound was really subtle. If that ultrasound tech had missed it and she had been born four months later, and she did not have any visual outward signs. So, she passed her hearing screening. I didn't mention hearing loss before, but I should have because that is a symptom. But she passed her our hearing screening. She didn't have a large liver, she didn't have a blueberry muffin rash, she didn't have a small head size. So, she really had not a lot of outward symptoms. She did, on a head ultrasound, they could see calcifications and when they drew her blood and ran a bunch of bloodwork on her, they could see some things there, but not things that were super obvious. So, she is a baby that could have been missed. I really think so even though she was symptomatic at birth. [00:17:32] DS: Wow. What a great reminder for everyone out there. I mean, hey, and props to that ultrasound tech too. I can't imagine that on that teeny little baby that those little calcifications were easy to catch. So, how crazy. That's great. I'm curious what the process was. So, at that time, did they think okay, this is a sign of CMV? Or did they – oh, I guess you said they did the amniocentesis. At that point, do you do any – are you on any antivirals? Is there anything that they do? Or do they just say, “Okay, we'll have to wait and see what happens when she's born.” [00:18:02] AD: So, it really varies depending upon your doctor. So, when this happened to us, just sharing our own personal experience, our doctor said, you can terminate the pregnancy or you can do nothing and just wait and see what happens. We were not really interested in those options, in either of those options. So, we said, we're going to find a second opinion and we went to the next state over. We went up to Minnesota and we connected with a world renowned CMV expert there, Dr. Mark Schleiss and he connected us with the Maternal Fetal Medicine group up there. And we actually did do experimental treatment during the pregnancy. We did infusions called [inaudible 00:18:41]. So, this is like immunoglobulin and very expensive stuff. $20,000 per infusion, we did four of them and insurance paid for it. And the way that was described to us is this might help the baby, but it shouldn't hurt the baby. What we did is we just thought, “Well, it's worth a try. We'll see if we can help her.” So, we had the advantage of doing those infusions. Some people are treated now with oral antivirals during pregnancy. But this is really just something that isn’t completely proven yet. It's definitely not standard practice. But hopefully, we're making some progress on some treatments during pregnancy. I would always encourage people to get a second opinion and make sure that that you're talking to someone who knows what all the options are. Because not all doctors are up to date on the latest regarding treatment during pregnancy. And it's not for everybody. I'm not saying that everybody should do that, but they should definitely – [00:19:40] DS: Know their options. [00:19:41] AD: For sure. [00:19:42] DS: I mean, I completely agree. We had a baby in April, and she was born at an amazing hospital that births a lot of babies every year and they do great stuff. I was just curious because I know about CMV and I teach my students about it, and every year when I do the slide, we're talking through like a lot of different things that can lead to hearing loss. I talk about CMV. Very briefly. Every time I see their jaws drop, and they're like, “How come I've never heard of this before?” And I'm like, “I think the same thing.” So, I was just curious in the process in the hospital, I was wondering, “Okay, well, how could I have them”, because my original goal, because we had a toddler in the home while my wife was pregnant, I knew she was going to be at an increased risk. Fortunately, it was during COVID. So, we weren't really ever seeing other children, which is really most of the risk there. But still, he's a kid, he's doing all kinds of crazy things. Who knows how he could contract CMV? Anyway, so I asked my wife, I was like, “Could you talk to your doctor about screening for it, before we get pregnant, or as soon as we found out, we were pregnant.” She was also a first-grade teacher. So, I assumed, my assumption is, and maybe you can clarify that for me here. So, mothers who are maybe planning to become pregnant or who are in the early stages of pregnancy, if they test positive for CMV, it doesn't necessarily mean they contracted it recently, right? I guess it could show from any point in their lives, but you could still contract it again, while pregnant? [00:21:00] AD: CMV testing during pregnancy, in terms of the woman's status is a little confusing and you really have to find and talk with the doctor who knows what tests to order, and how to read the test. So, we do have information on our website about that. Because what they draw, is they draw two different things. They draw IGG and IGN and one is going to tell you, if you've ever had CMV. One is going to tell you if you've recently had it and it gets a little more complicated than that. But essentially what you want to do is find someone who can tell you, have I had it like in the past, or have I had a recent infection. And that's going to tell you your status. It really doesn't change anything from our perspective, in terms of the precautions you should take during the pregnancy. We're going to say, “If you've had CMV before or not, we want you to take these same precautions.” But it can just be a starting point for women to understand where they're at, before they conceive or after too. [00:21:57] DS: Sure. Yeah, because we kind of ran into some roadblocks. They're like, “What do you want to do? You want to screen for CMV? What are you talking about?” That's not something that we do and her blood draw. I was like, “Please.” So, I got a lot of pushback there. And then even after our daughter was born, and then we were in the nurse – we’re in our hospital room. I'm like, “Okay, can you guys make sure when you do” – I guess they do the heel prick, maybe you can speak to that, too. I know, they're screening for some metabolic disorders that I think are pretty rare. If I'm remembering correctly, is that the PKU? Am I remembering that correctly? [00:22:29] AD: There’s several – well, some states are testing up to 50 different things on that newborn bloodspot card. Each state is a little bit different. But yes, CMV. I am pretty sure it's more common than anything that we currently test for on the newborn screening. For sure, it's more common than all of the metabolic disorders that we test for. So yes. [00:22:49] DS: Sure. And I asked, I was like, “Well, can we include a CMV screening when you send that bloodspot off?” The nursing staff who see babies every single day, they're like, “CMV? That kind of rings a bell. I don't think anybody's ever asked me that before.” And I was like, “What? This is crazy.” So, I get a lot of pushback there. They're like, “Maybe you should check with your pediatrician. They can order the screening.” I'm like, “Oh, my gosh, I cannot believe it's this hard to get something that is so common.” And if I'm not remembering incorrectly, time is limited, right? It's like, after a few weeks, they could just catch it from brother or sister when they get home. [00:23:23] AD: Yeah, absolutely. So, time is limited, especially if we want to test the newborn’s urine or saliva, which is the most accurate. We need to do that by three weeks of age. So, that's why the states that are doing screening, at this point, are using saliva mainly and urine to confirm it. They're trying to collect that right away. Because after – you’re right, after three weeks, we don't know if this was really a virus that they caught before birth, or if they caught it after birth, in which case, it should not be harmful. We can use the blood card and it's not as accurate as the urine or saliva. But the problem with blood card is it's only kept for a certain amount of time. Some states keep it a really long time and that's great, then they can go back and they can check it for CMV. It's not completely conclusive, is this a case of congenital CMV? You can't tell for sure. If it's positive, it's positive. But if it's negative, it could just be that the test wasn't sensitive enough to pick it up from the blood. So, you can do that not completely accurate. But the problem is some states only keep those cards for a couple months and some of these babies born with CMV will develop problems after that blood spot card is destroyed. So, at that point, when they're six months old or a year old, there's no way to confirm it anymore. So, you're never going to get an accurate diagnosis necessarily where a for sure diagnosis you can get a suspected diagnosis. But also, by that point, it's too late for early intervention. They've missed their window for potential antiviral treatment, if that would have been appropriate for them. We're just missing a window of opportunity to monitor these children intervene early, potentially do antivirals, all these things that are really important that these babies are missing out on. [00:25:13] DS: Wow. Oh, my goodness, that's so frustrating. Which states, do we know? Is there a lot of states that are starting to implement some of these screenings? Or is it pretty rare still? [00:25:23] AD: I mean, I guess I would say pretty rare still. But the states, it all started in Utah, they implemented the first, we call it targeted screening programs. So, what they do is, any baby that fails the newborn hearing screening in the state, is required by law to have a CMV test by 21 days of age. So, when we do that, when we take that approach, we catch about half of the kids born with CMV. So, it's not great, but it's way better than identifying less than 5%, which is what we do. [00:25:56] DS: Oh, my gosh, less than 5% we're catching? [00:25:58] AD: Yes, less than 5%. So, it's kind of a step and other states have followed suit, so other states are doing it too. Connecticut, New York, Iowa, where I live, we passed a law a few years ago. So, more and more states are adding it mainly as a targeted program. There are individual hospital systems that do it universally. Someone talked to me from Tennessee a couple days ago, or a couple weeks ago, and they're all hospitals doing it. So, some health systems are taking their own approach and adding it, and then there's two states right now, Minnesota and Massachusetts that still have live or active bills to mandate universal testing of newborns. We could have the first state with a universal testing requirement soon. I hope so. But right now, it's mainly these targeted states, and then individual health systems, adding the test either as a targeted approach or as a universal approach. [00:26:55] DS: Okay, that makes sense. I think that's a great first step, if you're using hearing loss, as your precursor You're still going to miss a few. But still, yeah, it's definitely a step in the right direction. I do feel like, yeah, I think the only thing we can really look forward to is a universal screening for this, because it's just so common and time is so limited, that we can't go back and check that, yeah, I really feel like – I'm really hoping that's kind of the step that we take is to get there. If a baby is born symptomatic, are doctors usually doing anything? I mean, I think I had written down, there's maybe some antivirals they might take if born symptomatic. I mean, I guess, like the baby in my example, she was born symptomatic, and it still wasn't caught. So, I guess in those situations, they're not going to do anything. But can they take antivirals? Is that going to help with symptoms? You know what I mean? Can that lessen the severity? [00:27:47] AD: Sure. So, the way antivirals are described to me is that they should be used for children with symptoms, and not just hearing loss. Infants with just hearing loss, in general, they're not recommending that those infants take antivirals. It's really for infants with hearing loss, and or all these other things that can come with CMV. So, there's the study that really spurred. This was in the New England Journal of Medicine and showed that was six months of antiviral treatment, the children had better hearing and neurodevelopmental outcomes. So, that's the big thing now is, let's get these kiddos on these antivirals, if it's appropriate. Hopefully, it will help with their hearing and neural development. However, it's not without any risks. So, they need to be monitored closely for side effects. So, that's one thing and sometimes pushback that we get from people as well. We don't want all kids born with CMV to be on these antivirals. Of course, we wouldn't recommend that either. We really want a physician who's knowledgeable about these antivirals and about CMV to decide or work with the family to decide, is this appropriate or not. But all children born with the virus, we want to have routine developmental monitoring, hearing assessments. They need to have hearing assessments every three months. Because I mean, a lot of people listening to this will know that when we identify hearing loss, we want to intervene as early as possible. So that can show up anywhere, vision exams also, because vision can be impacted by congenital CMV as well. So, we really want antiviral treatment, but we also want early intervention monitoring for these kiddos, whether they're on antivirals or not. [00:29:34] DS: Yeah, that's a great reminder. That's a great segue too, because I did want to sort of talk a little bit more about the specific symptoms and as much as you want to incorporate kind of Pippa’s experience into this, feel free. But I know when I look at the CDC list, I guess it's actually coming from the National CMV foundation, this range between born symptomatic to born asymptomatic, and the degree of impacts we might see from severe to moderate to mild. On one end of the spectrum, there's no visible signs of anything at birth, and there's no visible delays or impairments throughout life. Okay. That's basically no change at all, versus the other end of the spectrum. I mean, children can be born and immediately pass away, or just be medically fragile for the rest of their lives with CP or seizures or have failure to thrive. What a range, right? That's kind of crazy. I'm curious, either in your experience with the CMV foundation or with Pippa, what kind of range of symptoms you've seen or stories, you've heard from people you've interacted with, in terms of their experience in this really wide spectrum of symptoms? [00:30:44] AD: Sure. So yeah, you're right, there is a really wide spectrum and some kids are being identified that have no symptoms, and never will have symptoms. Mostly, if they're part of like some kind of universal pilot study or something like that. Because in general, if they have no symptoms, and nothing ever pops up, they're not going to be identified. But yeah, we've got all the way along that spectrum all the way to about 400 infant deaths per year, and also a big contributor to miscarriage and stillbirth. There was an interesting article that I think 15% of stillbirth, a study. There was CMV in the placenta. So, this is a big contributor to those things, too. So, a very wide range of symptoms. I would say at the National CMV Foundation, when people contact me who have children with CMV. Most of the time, we're getting contacted by people with more severe symptoms, because those are the majority of the infants that we're probably catching right now. If we're catching a baby, it's probably because they had severe enough symptoms for someone to think to test them. [00:31:49] DS: Sure. [00:31:51] AD: So, I would say a lot of our families have been severely impacted and that drives a lot of our families too, to work on this and try and spread awareness because how significantly it's impacted their family. In terms of my own daughter, Pippa, it's so hard sometimes to decide where to put kiddos on kind of the spectrum. It’s kind of hard to decide, but she's so – [00:32:12] DS: It changes over time, too, right? It can be progressive, so you can start one at place. [00:32:17] AD: Absolutely. So yeah, Pippa is somewhere between moderate and severe. So, she's, like I said, she has a brain malformation. It's called polymicrogyria, which people abbreviate that PMG. So, the brain is smooth, instead of having nice deep folds like it should. So, that really impacts her quite significantly. She has an intellectual disability. She's nonverbal. She has issues with feeding. She still takes a bottle. She's almost six years old. But actually, we're really grateful that she can take some food by mouth. But she's got hearing loss and as you and I were discussing a little bit earlier, that didn't show up until she was three and a half. Now, she had medical treatment in utero and after birth for six months, so I don't know, maybe that helped. So, she's got mild hearing loss. Now on both sides, she wears hearing aids. She's got just developmental delay in general, and seizures. That's another big one. We see that a lot in our families. Like I said, most of our families are more significantly impacted. So, a lot of them are dealing with that, too. There's a whole range of symptoms and then we've also got families who maybe they’re only dealing with one thing, and not to minimize that at all. Because anything that happens is very hard for families to deal with. We do have children who hearing loss seems to be the only issue that they have. We've got this whole range of things. But I think, and I don't have a scientific study to say this, but I think in general, probably right now, most of the kids being identified are more severely impacted. [00:33:48] DS: Gotcha. Yeah, it makes a lot of sense. One of the things we're going to look for, if they're presenting with these things, let's think about what could possibly be going on here. But you can't help but wonder. I mean, I can just think about a lot of children on my caseload who show up with hearing loss later. And it's like, “Were you missed in the hearing screening process? Or is something more progressive here?” Maybe they'll do genetic testing and nothing pops up, and I'm like, “What else could it be? Because CMV is pretty common and it makes sense that this might be what's going on here.” So, yeah, that's one of the interesting things with that one family I was mentioning how it was – she was much later identified with her profound hearing loss. I saw her on her way to cochlear implants and in that kind of probing process, things started to bubble up, like, “You know what, she did have this interesting rash when she was born, but they said it was no problem.” And you know what she did? I mean, it was like things just started to flood in. Now, it helps so much to know, what to be expecting, because it can definitely impact what their expectations are with their cochlear implants. If she might have calcifications in her brain that can definitely impact her speech and language development, unrelated to her hearing loss. It's helpful to know the big picture here and I worry that a lot of families aren't getting that, because that's not being caught early enough. [00:35:09] AD: Yeah, absolutely. I think about that a lot, too. My sister works at a school. She's a school psychologist, and she said, she deals with a lot of children with learning disabilities and things like that and they never have found out a cause. She always thinks now she thinks, I wonder if that was CMV? Because it's one of the most common causes of disabilities in children. When I saw infants, when I was doing home visitation nursing, I was told the nurses I worked with and myself too, after I had Pippa, if we have an infant that doesn't pass the newborn hearing screening, you should be thinking about CMV, because you've got almost 20% to 25% chance that that's what it is. So, we should be thinking about that, that 20% to 25% of childhood hearing loss is due to this. So, when you have a symptom, it should trigger you to think about it, but it doesn't always work that way. [00:36:05] DS: Yeah. I'm wondering too, just in your process with Pippa, I mean, beyond, I'm interested in more like, I'm honestly really curious what color are her hearing aids? I feel like they're probably – [00:36:15] AD: She’s got purple ones. [00:36:17] DS: Yeah, they had to be fun. Pippa’s purple. That makes a lot of sense. But I'm curious if there's anything, just like more on a personal level. I mean, you're clearly very knowledgeable on CMV now, but if there were things that in the process you wish you'd known or that you'd share with people who have no idea or maybe who do know, like might have tested positive for CMV early on in their pregnancy, but they don't know what that impact is going to be on their baby or anything like that, just from like a more personal level, like advice you would share? [00:36:48] AD: Well, two things I try and tell people is, and I already said this once, but I always say get a second opinion. Always, always get a second opinion, especially if it seems like the person that you're talking to, doesn't know much about CMV. Because there are people out there who know everything about it, as much as possible right now. So, I always say that, whether you're still pregnant, and you're dealing with CMV, or your baby's just been diagnosed or anything like that, always get a second opinion, and really check it out and make sure that you're getting the best possible options. For people with a new diagnosis, my advice is always just to deal with the short term, don't think too far ahead. I have been doing that myself and it has been hard. But I've kind of finally learned to just look like three months ahead and not look farther than that. Because if I look farther ahead, that's when I really started to get upset and worried and really stressed out about Pippa, and what things are going to be like, and what's going to happen. So, I'm always just trying to look in the short term, and what am I doing for her now, and is there anything else that needs to be doing for her? Is she happy? That's the other big thing is we're just mainly concerned with making sure that she's happy, which we're very lucky because she is happy and relatively healthy, and comfortable and all those things. So, I guess that's my advice is to think short term. [00:38:15] DS: Yeah, that's amazing advice. I really appreciate you sharing that. I think that's really helpful. I think a lot of families out there, regardless of if it's CMV, or something else that's impacting their child's development, I think that perspective is extremely healthy and helpful. So, I really appreciate you sharing that. [00:38:31] AD: Sure. [00:38:33] DS: So, one other thing, too. I actually just remembered this. I had a coworker, when I was in a pediatric hospital setting and she must have been really knowledgeable on CMV, and I think she tested negative and negative before she and her husband decided to start trying to have a baby. She got a test to determine if she'd ever had CMV, which you've actually helped me understand that a little bit better. So, even if you did, even if you have had it, maybe in your childhood or whatever, if you were always around kids, there's still a chance you could contract it again, while pregnant. It's not like, “Oh, I'm essentially vaccinated from this disease and I don't have to worry about it.” It's still definitely a risk. [00:39:14] AD: Unfortunately, not. Unfortunately, it's not completely protective. It does seem like there is a little protection and babies born to moms with a primary infection or the first time that they've ever had CMV, they seem to be more severely affected, but not always. There are instances people I know who I've talked to who had a secondary infection with a new strain or a reactivation. Unfortunately, it can reactivate too, the virus stays in your body forever, and it can just kind of reactivate. That happens during the pregnancy too. I know people who have severely impacted children from that. So, it's not completely protective if you've had it before. And so that's why we're always – well, first of all, we want a vaccine so that people don't have to you even think about this. It could be like rubella where people don't even think about it anymore, because it's so uncommon, because we're preventing it with a vaccine. But that's also why we tell people, we want people to follow the exact same precautions, whether they've had the virus before or not. [00:40:18] DS: Gotcha. That's really good to know, because I didn't realize that. I thought that there was maybe a chance if you'd have done it before, you're probably just fine. But no, that's really good. I know there are multiple strains and even if it's not, I guess it still could reactivate in some way. So, that's good to know. But she had been tested and found out that she'd never had it before. So, she treated it like, “Okay, I don't want to risk any kind of primary infection and I work with children all day, every day, up close, looking in their ears, in the booth up close. I'm always around them and there's always a chance I'm picking up toys that have been dirty and things like that.” So, she was always – this is well before COVID, when other people just started wearing gloves a lot more all the time. But she was in gloves between every patient for every little thing that she could possibly touch. And I was like, “That's so interesting. I've never heard anybody”, like when I learned about CMV, but I've never really seen anyone have a plan or like, think about it in any way, seriously. So, that's always stuck with me, too, is that example of, I wonder if it's – that’s why my wife, the first time we were planning on getting pregnant, I was like, “I think you should go get tested for this. You’re a teacher, you probably had it. But still it would be good to know. Because if you didn't, it would be so bad.” Yeah, I think that's a good reminder. A, maybe do that if you are hoping to have a baby go get tested to see if you've had it before, because that primary infection is an increased risk. But even still, it's good to just be aware of CMV practice and good hand hygiene anyway. Right? [00:41:38] AD: Yeah, absolutely. That made me think about two things. First of all, probably somewhere around half of pregnant women have not ever had it. [00:41:47] DS: That's a pretty big number. [00:41:48] AD: To me, that's a big number. Maybe it's a little more, maybe it's closer to 60. But either way, that's still a significant amount of people who've never had it. And sometimes I do hear that from doctors and for women. I'm sure I've had it. Well, I was in daycare, my whole childhood. I was in school. I had a little sibling, that was younger than me, and I never got it. So, I always say, “Well, you can't just assume that you've had it.” But second of all, that reminded me of something else that's really important to say is sometimes we get healthcare workers talking to us about avoiding kids who for sure have congenital CMV or have a CCMV diagnosis in their past. We really try and make sure that we spread the message that, again, 95% or more of kids born with CMV are never identified. So, just because a child has a CMV diagnosis, there are many other children born with CMV that you don't know, and you're four times more likely to come in contact with CMV from a child that didn't have a CMV diagnosis. So, sometimes we get that from health care workers that they're going to avoid kids with CMV and then that will protect them. But the message we want to share is, all kids should be treated with the same precautions, because you do not know which kid is shedding CMV and which kid is not. [00:43:06] DS: Yeah, that's kind of bizarre to have that kind of mentality for any – that’s bizarre to me. But yeah, I totally agree. It's very irrational to have any fear of something, that is. I think you had a great statistic four times more likely to come from someone you'd never expect. [00:43:26] AD: And we get that sometimes from our families. In a way, they're kind of almost stigmatized, and they've had therapists that don't want to work with their kids and daycares that say, “Your kid can't come.” Or things like that and that's really not recommended and that goes against, I think it's AAP and CDC both. I can state that you shouldn't exclude children born with CMV, because the next kid that walks in the door is just as likely to be shedding it. [00:43:53] DS: Oh, my goodness. Yeah. [00:43:55] AD: So, there is an official stance on that. [00:43:58] DS: Wow, I'm glad that they put that out there. Because that sounds a little – that perspective is a little silly. So, I'm glad we have some really direct instruction there. Okay, so I appreciate you shared about Utah's kind of targeted screening through their hearings, because I mean, I guess if you have one screening program already in place, it's a little bit easier to probably build off that. But what does the future look like? You also mentioned the vaccine. That was my first thought earlier when we talked about this I'm like, “What if we just treated it like a viral infection unrelated to the congenital pathway, not that transmitted by mother but just like from a person to person thing that does later lead to a lot of health care needs overall for these babies later born?” Is there any movement in terms of a vaccine? [00:44:38] AD: Yeah, there's I think there's good movement. I'm actually feeling optimistic about this avenue of stopping CMV is through the vaccine route. So, there are several companies working on it. I mean, some of them that people are very familiar with now because of COVID. But Moderna being one of them. We’re great friends with Moderna and we do a lot of work with them and they have been working on a CMV vaccine for a while. So, they're working on – I think their phase three trial sometime soon. So, we're hoping that this is going to be something that young women before they get pregnant can be vaccinated for. And maybe eventually, once vaccines are available, maybe it will become part just of childhood immunizations the way that rubella is. [00:45:26] DS: Sure. [00:45:26] AD: So, I hope that's the future. That's my dream. That's my dream is that we just prevent this to begin with and then we don't have to worry so much about screening every baby for it, or how do we treat these babies after this damage, I mean, irreversible changes have happened. [00:45:43] DS: Wow, that's way more promising than I was expecting. I did not realize phase three of a clinical trial. That's amazing. I mean, that sounds very realistic in the near future. How amazing. You know what, I hadn't even thought it. I really was just thinking of this as sort of like, in the first couple years of life for a baby, not that this would be sort of in the rounds of vaccinations for mothers who are planning or kind of in that young phase where they might be considering it. That's a great idea. That makes a lot of sense. [00:46:12] AD: Yeah, so we've got to – the thing about that is, I think half of all pregnancies in the United States are not planned. So, we've got to make sure that we get people vaccinated, before that happens. I mean, it's still years away, but I'm hopeful I feel hopeful about that, all the work being done on that. [00:46:32] DS: Yeah, that there's something in progress is just fantastic. Okay. So, beyond the vaccine, how are – I know you said some hospitals are having the saliva and then maybe the urine screening in Utah. They have it based on a failed hearing screening. What other movements are happening throughout the US, or I guess even the world that you're aware of? I'm sure the national CMV foundation is involved in some of these pushes towards implementing a screening program. Anything else you're aware of that's kind of going on out there that people should be aware of? [00:47:03] AD: Yeah, sure. So, the first universal screening program, to my knowledge is in Ontario, Canada currently happening. So, they are currently screening every baby with the bloodspot, the newborn bloodspot. So, that's great. They were the first. So, we’re very, very happy to work with the CMV Canada Foundation. And hopefully, they'll have important data to be releasing about all that. So, that’s the first place in the world, to the best of my knowledge, that is doing a universal required screening for CMV. We, at the National CMV Foundation, are submitting – well, we have submitted an application to what's called the RUSP, which is the Recommended Uniform Screening Panel. And that is a committee, through the Department of Health and Human Services, I think, comprised of a few different disciplines, doctors and others who have an opinion and and that professional experience with newborn screening. What happens is a group of people decide, we should add this disorder or whatever to the RUSP. So, there's this application you have to fill out, it requires a lot of medical knowledge, and you've got to cite all these sources and get all this together. I mean, it usually ends up being hundreds of pages of information that you submit, and then the panel looks at it and they decide, “Yes, we will recommend that everybody do this”, or “No, here's the research you need to make your case better or whatever.” [00:48:41] DS: Sure. [00:48:42] AD: So, we submitted an application for that couple of years ago. We got some very initial feedback, and we're hoping to resubmit. What happens is, if something gets added to the RUSP, that just means that the United States Department of Health and Human Services is recommending that all the states add it. But each state gets to decide individually, what they add and what they don't add. So, some states follow the RUSP completely and they have it in their law that whenever something gets added to the RUSP, we add it to our newborn screening panel. And then most states don't. So, it's still kind of a state by state basis. The other thing is, the modality or how we're going to be doing this screening is really a little difficult for people right now. People would really love us to be able to put it on the newborn bloodspot card. But like we've mentioned before, that's not as accurate as urine or saliva. So, right now, we still think that saliva is the best way to screen babies. So, hopefully, we'll be re-submitting the application in the fall, and then we'll get feedback on what we still need to research to really make the case that every baby should be screened. [00:49:50] DS: Gotcha. Is there anything that – so, I was thinking when you're describing like the process and how lengthy it could be just to get to a point where hospitals start to enact that, so for people who are listening or who are aware of CMV, or they've been affected by it, but let's say they're like me, and they ran into some, I'm not even really sure I know what that is from the health care workers involved. What are you recommending people can do sort of like on a person to person base to be proactive? I guess send materials to your pediatrician about CMV? Or is there any kind of screening where I could request a test tube from somebody and send my baby spit? You know what I mean? Are there any options like that out there? [00:50:30] AD: Sure. Well, so there's a couple things there, I think. [00:50:35] DS: That's a pretty rough way to describe it, but you get what I'm asking there. [00:50:38] AD: Yeah. So, sometimes I run into physicians who have heard of CMV, but maybe have an idea in their mind, about how common it is, what we can do about it, how serious it is, things like that. So, they might have an idea in their head that isn't completely accurate and maybe they're relying on something they learned in medical school, 15, 20 plus years ago. We actually have a doctor on her board and she said, she has a daughter with congenital CMV and she reviewed what they learned in medical school, and it wasn't as thorough as it maybe should have been for how common this is. So, I think providing physicians with up to date information does not hurt. There's this too. I'm a nurse. And I didn't know enough about this at all and I went back and looked in my nursing book, and there was a teeny tiny paragraph that in no way did justice to how common or how serious this can be. So, people don't always know the latest about CMV. So, I don't think that there's anything wrong with giving people updated information and say, “No, look, this is more common than you think it is”, or “This is more serious than you might realize.” So, that's one thing. The other thing is, if you want to have your baby tested, there is going to be a way to get it done. Not all hospitals and labs have the ability to do it. Because there's certain equipment and laboratory things needed to do the test and not everybody has it. So, there could be a way to collect it and send it off to a lab that does do it or a hospital that does do it. It's going to take a little bit of groundwork, a little bit of footwork to try and figure that out. We do have families come to us with a newborn bloodspot card. Well, they don't come to me, but they contact me and say, “I've got the bloodspot card. My pediatrician can't test it. What do I do?” We can connect you with doctors throughout the country that are doing testing on the bloodspot card. And again, like I said, it's not completely accurate, but they'll test it for you. So, we do have people reach out to us with that. So, it can be a little bit tricky. I think you're just going to have to really do your research and try and figure out where can I get this tested near me. [00:52:53] DS: Gotcha. But it's good to know that the CMV Foundation can have some resources there. I do, in just a second, I want to talk a little bit about the CMV Foundation and kind of June and CMV Awareness Month and that kind of thing. But before I do, either with your experience with Pippa or just what you've heard from other families, we've got audiologists, and speech language pathologists and students out there listening, is there anything you wish they would know about CMV, or working with children diagnosed with CCMV in their clinical duties, just something you wish as a parent, or as someone who is really knowledgeable about CMV, that they would know. [00:53:28] AD: My biggest thing is I want them to know how common this is. So, there’s actually a good research article, and I should send it to you just in case you haven't seen it. [00:53:36] DS: Please do. [00:53:38] AD: They assessed CMV awareness among audiologists and SLPs and the audiologist did pretty well. It was like 86% or something knew about CMV. But the SLPs didn't do as well and I love SLPs. Do not get me wrong. No way, need to insult any SLPs because they have been a lifesaver for our family. But the awareness level was low, it was like 26%. So, I think just being aware of it, and knowing about it and realizing that this is a common cause of disabilities in children, and so you probably have people on your caseload that have this diagnosis, and they may or may not even know it. I think that that's really important. I also just want them to know for themselves how to prevent this. [inaudible 00:54:23] expanding their family or whatever, that they're aware of it. And then I guess thirdly, just like I said before, I want them to realize that those kiddos with congenital CMV do not pose a higher risk to them than any other kid walking in the door, and that they should be using the precautions with all children. So, I think those are my big three things, I guess. [00:54:42] DS: Yeah, those are perfect. Like I was saying I teach graduate level SLP students and when we're in that lecture, and we talk about CMV, the jaws drop. So, I think that's a good point. I do think the impacts, maybe the kiddos that SLPs are seeing who have CMV, they might not realize it as much, whereas hearing loss is a little bit more obvious. You know what I mean? If a child is born with hearing loss, they're going to be more likely to try to determine the etiology. Maybe audiologists have to be more in the loop on that. You know what I mean? So, definitely, I see that difference there and I hope that that's something that can change for sure. As a segue, the kind of organization who is going to implement that change and educating people, the National CMV foundation. I know that June is National CMV Awareness Month. Is there anything you want to share about the initiatives that you all are doing there? Or just kind of what the work in general looks like? [00:55:33] AD: Sure, yeah. So, the National CMV Foundation, it was started by a few different moms and dads too that kind of combined smaller organizations together. So, I think we incorporated in 2014 or 2015. So, that's when we really became the National CMV Foundation and the mission now was just recently updated. So, our mission is to prevent pregnancy loss, childhood death, and disability to congenital CMV. So, that's our mission. This month is CMV Awareness Month. We do a whole big campaign. We try and get the word out as much as possible, and usually we have a theme. This year, the theme is screen for CMV. So, we've had some, like a screening panel online and we're trying to put out all the information about why screening for CMV is so important. So, that's been going on. We also do a campaign ‘Faces of CMV’. So, we share two faces of children with CMV every day, online, on our social media channels. That's where a lot of our action happens. We have very passionate parents and families who share their stories and share all of our content. But we, at the foundation, we also do research. We fund research in the congenital CMV space. We have an ambassador program called the CMV Community Alliance, where we have volunteers, they are parents, or just people who are really passionate about congenital CMV and they volunteer to work on spreading awareness in their areas. So, we're working on some more local and targeted approaches. We have educational materials on our website that people can download. None of them are copyrighted, so you can download them and give them out. We also just provide connections for people too, so if people ever email us, or call us, or whatever the case may be, we're always trying to answer their questions and connect them with somebody who can help. Those are kind of our main activities. We only have a two-person team, in terms of staff. So, we try really hard to get as much accomplished as possible. And we have dozens of wonderful volunteers who help with our mission as well and we have a nine-member board, volunteer board who sets our direction for the next three years. We have a three-year strategic plan and that's all available on our website, too. [00:57:54] DS: That's great. I'm curious for those listening, it’s CMV Awareness Month, hopefully they've listened all the way through this, and they're much more aware of CMV. That would be my goal. But I'm curious, is there anything else we can be doing as people who maybe haven't personally been affected by CMV, but definitely want to see change in terms of initiatives, calling hospitals or legislative representatives? What kind of like on the ground things? I mean, I guess just amplifying voices on social media is a good place to start too. Any recommendations there? [00:58:25] AD: Yeah, well, if there's legislative efforts happening in your state, there's certainly a need for people to reach out to legislators, but just in general, reaching out to legislators and letting them know about CMV and letting them know that that this is an important issue, and that the agencies that work on CMV need funding so that they can continue to work on CMV projects and maybe they could even designate money for CMV projects. That would be great. We have a petition, an online petition for our nomination to add CMV to the uniform screening panel that's available on our website too. People can sign that. But I think the biggest thing is just tell everybody, tell all the women that are thinking about becoming pregnant, or the men who can hopefully give this information to their partners or their wives or whatever, because that's what really – I mean, in my case, Pippa got the best treatment possible, and she was diagnosed before birth. So, early screening wouldn't have helped us and all the treatment in the world isn't going to erase what happened to her. The only thing that really would have changed the outcome for us is if we had known about this to begin with, and that's what families say, when they get this diagnosis. They say, “Why didn't anyone tell me about this?” And I don't have a great answer for that. Because I don't think that there is a good enough answer for that, why no one's telling people. So, that's my biggest thing is I know pregnant women are dealing with a lot but again, we should look at what's the most common threats to pregnancies and this would be one of the most common ones. [01:00:05] DS: Yeah. Wow. That's a really great perspective. Thank you for sharing that. Thank you for joining me. This has just been so fruitful, so eye opening. I really hope we get a lot of people who listen in here and take your advice and tell a few friends. Because I really think that's the only way, we're going to see just structural change here. If people are interested in connecting with you, or the National CMV Foundation, if they have more questions, where can they find you? [01:00:29] AD: Yeah. So, our website is www.nationalcmv.org. There are ways to submit questions on there. But we are always available to take questions via Facebook and Instagram. It's just National CMV is the handle. People can email me, amanda.devereaux@nationalcmv.org. My last name is hard to spell though. So, that's difficult. [01:00:52] DS: It'll be in the episode title. We’ll have it there. [01:00:56] AD: So yeah, we're always willing to connect and chat with people and answer any questions and make a connection. If we don't know the answer. We don't always know all the answers, but we can find someone who does. [01:01:09] DS: Absolutely. Thank you so much, so much for sharing your story. I'm excited and checking in and hearing how Pippa is doing. It sounds like you guys are just taking absolute amazing care of her and I'm just so excited to see what kind of movements we see in terms of vaccine, in terms of screening. It just sounds like the future is really bright. So, that's great. [01:01:28] AD: Oh, yeah, I hope so. We've got a good outlook. We're still really fired up and we got a lot of work to do. But I think we're making some progress. [01:01:37] DS: Awesome. Well, thank you so much for joining me, Amanda. It was great talking with you. [01:01:41] AD: All right, thank you. [END OF INTERVIEW] [01:01:43] DS: That's all for today. Thank you so much for listening, subscribing, and rating. This podcast is part of an audio course offered for continuing education, through speech therapy PD. Check out the website if you'd like to learn more about the CEU opportunities available for this episode, as well as archived episodes. Just head to speechtherapypd.com/ear. That’s speechtherapypd.com/ear. [END] OTE 22 Transcript © 2021 On The Ear 1