Chapters Transcript Diagnosis and Management of Cerebral Palsy Dr. Aravamuthan shares an overview of the diagnosis and management of Cerebral Palsy. So our first speaker is Doctor Boma, Sierra Marty Aravan Muthan. Aravan Muthen. Perfect. OK. She received her medical degree at Washington University School of Medicine and did her pediatric residency at St. Louis Children's Hospital in St. Louis. She then to Boston Children's Hospital in Massachusetts to complete her residency in child neurology and movement disorders. Doctor Aravo Mathin is currently assistant professor of neurology with the division of Child Neurology and Movement Disorders with the WSU or physicians. She specializes in dystonia, cerebral palsy, tics, child neurology, pediatric movement disorders, and sees patients at the downtown location. And then today, Doctor Av Malin is going to be talking, diagnosis and management of cerebral palsy. So, thank you so much. I really appreciate you, um, presenting on this, on this great topic. Thanks and thanks for having me. Excited to um talk to this group about this. Um, I've done my best to highlight the full lifespan approach, um, and, uh, things to think about as, as people with CPH. So, first, um, these are my disclosures, um, none relevant to today. Um, I wanted to start out by talking about our general approach to the CP, um, to CP and mobility Care, the CP and Mobility Center. This is probably my biggest disclosure is that I direct the CP and Mobility Center. At Saint Louis Children's and, um, WashU. So, um, we have, um, at, at every visit, um, a person with CP who's seen in our center is, um, seeing, um, practitioners across all of these ranges of practices. Um, every visit is staffed by A pediatric movement disorders trained neurologist. On certain designated visits, we'll, um, uh, collaborate with our physiatry colleagues and with our orthopedic surgeon colleagues, um, for election for certain procedures or surgical candidacy. At every visit, um, a family can see physical therapy. Um, at every visit, a family can see someone to help optimize, um, their wheelchair positioning and they talk about seating and talk about bracing. Um, we take a lifespan approach to our care, which includes facilitating, um, care transition preparedness, including thinking about guardianship and financial planning. We do that, um, through our social worker who's present for every single clinic visit and can be pulled into a room. Um, at family's discretion or clinic, discretion is needed. Um, on Mondays and Wednesdays in our clinic, our clinic is, is 3 days a week. On Mondays and Wednesdays, we have an occupational therapist in our clinic with us as well. And we also run a series of community-focused programs, um, and we share this with families in our clinic as well. As well. Um, there's the Try My Best Adaptive Triathlon, which just happened in September. Camp Independence, which runs twice a year, which is, um, a physical therapy intensive day camp that runs during the winter and the summer for 2 weeks over the winter, I'm sorry, for 1 week over the winter and 3 weeks over the summer. And then, um, in the summer, we also have something called CP Family Day that helps, um, connects families with community resources and helps them prepare for care transition and take a lifespan approach to cerebral palsy care. So, um, uh, you'll see at the bottom of these slides is my email and the phone number for child neurology. You can use either or both of those to reach out to me if you have any questions about anything I talked about today, including, um, any questions about the center. So, um, the main things I'm going to be covering today are, um, are these, and I'm gonna go through them in order. Um, so first, um, for people that are seeing, um, babies, uh, I'll talk about how to recognize CP signs early and why that's important. Um, first, what is CP? So CP is very common, um, globally, it affects 4 in 1000 people that, um, at this point outpaces the prevalence of Parkinson's disease and is several-fold more frequent than multiple sclerosis, um, across the lifespan. This is a lifelong condition. So, um, you very likely have people with CP in your practices across all ages. And, um, it's also very costly condition in terms of, um, medical costs and medical associated, medical associated costs. So, Um, about $12 billion in costs are incurred, um, over the lifespan of, um, all people born with CP in a given calendar year. So optimizing those costs and thinking through, um, what medical services these people need is, is a big imperative for us as, as physicians. Um, there's a huge push now, as it turns out, to standardize the diagnosis of CP. Um, the group of people listed here, I'm very closely collaborating with to do that. These are a few, um, papers that we've recently published talking about how we would standardize diagnosis. And sort of a summary of that work is Um, something that you can, uh, take with you when thinking through how to diagnose or confirm a CP diagnosis. So CP is a lifelong neurodevelopmental condition, so it lasts your whole life. And in fact, as we'll talk about later, most people alive with CP today are in fact adults over the age of 18. Um, CP has to affect functioning in some way. And it's due to atypical brain development that causes some sort of motor sign before you turn 2, and that motor sign can be a range of different things, but someone has to see something in that child before they're 2 that relates to how they move, and that child cannot lose motor skills by the time they're 5. So, if you were to see someone who's 40, And they come with you in the chart with the diagnosis of CP and they say that they were doing fine until they were 10, that person does not have CP cause they needed to have motor signs, something present before they were 2 that didn't cause motor regression by the time they were 5. If the first notice they had of any symptom was when when they were 10, that person does not have CP and deserves a completely different diagnostic workup. So, Um, and that happens a lot, right? People have diagnoses that are carried through their charts throughout their life, um, but CP has to be manifesting at less than age 2 without a loss of skills. In the same way, if you see, let's say a 12-year-old in clinic that, um, at 5 was walking, but at 12 is no longer walking and is now in a wheelchair to get around, that could also very well not be CP. So think through, um, if you see that sort of pattern of, um, symptoms in, in people in your clinic. Um, think through whether or not CP is the right diagnosis and, and consider referrals to, um, neurology or other subspecialists to help figure that out. Um, there's been a lot of, um, reticence to diagnose CP early. The field, the CP field is largely pushed for early diagnosis, meaning in infancy at less than a year old. Some, um, physicians cited barriers to early diagnosis or that it may cause unnecessary stress on the family, and what if you diagnose CP too early, and, um, you know, what if you're wrong? Um, when families are surveyed, um, so these are families that, um, have a child who may, may have risk factors for CP like they were born premature or they spent time in the neonatal ICU, um, and whether or not they had a CP diagnosis eventually, they all, and, and this is shocking to me, 100% of these families surveyed through, um, the Cerebral palsy Foundation. said they would prefer to have had the discussion about a CP diagnosis within the first year of life, that discussing that label and even conferring that label would have been valuable for their understanding of how their child's motor symptoms would have evolved. So, um, families are not scared by this discussion of CP, at least that's what the data shows. Um, and the Moreover, the value in conferring the CP diagnosis is that there are really accurate ways to do that now using standardized exams, and conferring that CP diagnosis early really triggers the kind of early intervention that people need. Um, to prevent later life motor impairments. So, at this point, the main disease modifying treatment we have for cerebral palsy is early physical therapy, and that's early or early therapies within the first couple of years of life. So if we're waiting until age 2 to make a CP diagnosis or even until age 1 to make a CP diagnosis, that is a long time of, um, that's wasted time. And particularly in states like Missouri where eligibility for early interventions is at a developmental cutoff of, you have to be 50% delayed to qualify for a lot of the early intervention therapies, um, it's or first steps therapies, um, that diagnosis can really unlock access to a lot of the therapies our, our children need to succeed, not just now, but across the lifespan. Um, I have this QR code up here in the corner. This is a cheat sheet that you guys can scan. I hope it's not getting blocked on your screen, but, um, it, it will lead you to a PDF cheat sheet of um referral criteria to prompt consideration of a CP diagnosis. It includes these six consensus, international consensus-based referral criteria to prompt um consideration of a CP diagnosis, and that can include referral to our center, for example, to confirm that. Um, and you can see them listed here. I, I, I'm not gonna go through them one by one. A lot of these are, are very key, um, developmental milestones that you're already likely tracking if you're seeing these infants. But if you see one of these, if, if you notice one of these in a child, um, the international consensus guidelines or to refer that child for consideration of a CP diagnosis, and in this setting, we can get, um, babies in for this evaluation. Within one week, if not less, honestly, within the CP center. So, um, if, if you have a child meeting these criteria, please, please get them in to see us so we can make that diagnosis and, and get them the therapies they need. Another way to think through, um, referral criteria is using a standardized exam. This is, um, the brief Hammersmith Infant neurological examination or the brief HI. The full HI, um, is, is a few pages long of an exam. The brief HI is, is now a validated one-pager, um, and this is just a snapshot from it. It's not the full one-pager. The full one-pager, though, if you scan this QR code is in that PDF. Um, so you, you circle the scores, um, for this child, um, across these different, um, criteria, and then when you add up the scores at the bottom, it gives you a score-based cutoff for, um, uh, likelihood of high probability of CP. So, if, if a child is meeting any of these score-based cutoffs on the, uh, brief hind, that's also a criteria for referral. So, um, it's, I know that, that visits are, um, There's only so much you can do in, in a well-baby check. Um, I think incorporating one or the other of these sorts of referral criteria can get children to us early for either um confirmation of a CP diagnosis or figuring out why these um abnormal motor signs are there. So, um, This, again, scan that QR code. It's a, it's, this QR code is a one-page PDF front and back. Have it in your pocket. It can be something that um can help you get a child to us quickly to get the therapies they need. OK. Um, once the, uh, diagnosis is established, something that we focus on in the CP Center is establishing etiology. And, um, just like everything now, we're learning more and more that genetic etiologies are increasingly common, a common contributor to cerebral palsy. Um, the majority of people with CP want to know what caused their CP, um, and this is from a study that we did a few years ago, um, and then we also in that same study found out that it was the minority of CP that actually knew would cause their CP. Um, CP is a clinical diagnosis that's not dependent on etiology. So, the diagnosis itself can be made by exam and by, um, looking to see if a child's meeting certain history or physical exam criteria as I outlined before in, in the definition. Importantly, a genetic etiology does not exclude a CP diagnosis, very much like a genetic etiology does not exclude a diagnosis of autism. And it turns out that the more, the more information we get on this, um, estimates range from 12 to 30% of people with CP have contributing genetic etiologies, and that includes people with other risk factors for CP. So even if, if you have a baby who's born preterm, who has CP, If they were to get genetic testing, there is a genetic contributor in about 8% of those babies. So, um, why does this matter? Um, recently, the American College of Medical Genetics recommended that whole exome sequencing or whole genome sequencing is now first-line diagnostic evaluation, um, for developmental disability including CP. And the reason they did that was when they aggregated the data across numerous studies, they saw that there was a change in acute or long-term management up to 10% of the time. And this isn't just um family planning cause I think a lot of people think about, well, you know, I'm not gonna have any more kids, so it doesn't really matter if we test my child for um a genetic diagnosis or my child is not going to have children for sure. I, I don't, it doesn't matter if they pass this on. Um, but the, the management that actually changes with the genetic diagnosis is screening for coexisting conditions. So there are, there are actually quite a few genetic etiologies of CP that are associated with, um, cardiac conditions that can be prevented if you screen for them, or renal conditions that can be prevented if you screen for them. Um, or, uh, a certain type of, um, bone condition that is worth, uh, talking to families about for surgical prognosis. So, um, it's these sorts of, uh, screening for coexisting condition outcomes that genetic, uh, identifying a specific genetic etiology can help with. And, um, recent data, um, that we've done in collaboration with other colleagues, um, like Siruvastava have shown that, um, a CMA is, is no longer enough, that the diagnostic yield of a whole exome is far superior to the diagnostic yield of a chromosomal microarray, and the overwhelming recommendation now in national guidelines is that the whole exome should be a first-line diagnostic evaluation for people with CP. Now, it that recommendation comes with a lot of um difficulty in execution. Um, I will say that, um, we, in our CP center, um, every single person with a CP diagnosis is referred for whole exome, uh, sequencing, which, and we manage the insurance authorization and genetic counselor workflow for that. If you have, um, a child with CP or a person with CP that you're following who has not yet had genetic evaluation, um, considering referring to us to help facilitate that etiologic evaluation is something that we can also do. Um, I understand that, you know, we're very, it's a luxury to have a genetic counselor built in to our neurology division that manages all of these referrals, and we definitely take advantage of her, so I would encourage you to, um, take advantage of that workflow through us as well. OK. So first point, um, you've recognized the CP signs early, you've made the diagnosis, made the referral. Now, how do you think through management? So, um, When we think through management, um, classically, um, often, uh, you have a health condition, and then how it affects their body functions and structure. So you have, in this case, um, someone's had a stroke, they have certain, um, they, they may have facial weakness, they have spasticity in their hand, and they have certain patterns of weakness. You refer them for, you know, physical therapy, occupational therapy, and then consideration of botulinum toxin. To, um, their hand to help, um, those intrinsic hand muscles relax. Um, what we really strive to do when thinking about someone with, um, CP or anybody with a disability is use, um, goal-driven care, um, that's guided by the WHO International Classification of Functioning Disability and Health, or the ICF framework. And that includes identifying things like what are the activities that are limiting you that you might need help with, um, how does this affect your participation? So if you have trouble gripping things in this, in this hypothetical case, um, what is it that, that's preventing you from doing? And it turns out it's preventing this person from date night cause they can't use utensils at date night. What are the environmental factors that play into your ability to be independent in your environment? Is your house adaptable, for example? And then what are your personal factors that are important to you? In this case, for example, this person has a very overprotective spouse that he's, he's struggling to navigate in terms of how to, how to care for, um, his, uh, his needs and his, and his goals. So each of these items trigger a different set of um medical and medical associated referrals. So, targeted OT referrals, targeted social work referrals, or OT referrals for driving assessments, which is something we do often. Um. And, um, uh, other specific guidance for thinking through, um, if your house is not adaptable, how are ways that we can, um, help you strengthen certain skills that you have to make sure that you're safe at home, um, how can we facilitate your partner, if that's important to you, being a part of these visits. And then lastly, how do we document beyond the activity that you're interested in how this condition affects other aspects of your life regarding feeding, communication, mobility overall. Etc. So, um, I think we all at every single visit try to do all of these things. Uh, this is, I think, it, it's, it guides all of our care, and it's something that we really specialize in when caring for people with motor disabilities. So, um, this is something that we can help optimize or help think people, help people think through. As, um, we move past, um, a body function structure face or, um, facing, uh, intervention to more global, uh, life optimization and goal optimization-based interventions. Um, with that said, there are, there's lots that we can do to help, uh, achieve that. Um, I'm just going to run through some examples here of things that we Um, do in the clinic, um, to help manage people's, um, motor-specific outcomes, so increase the range of motion, optimize their tone, optimize their gait and positioning. Um, as, as I mentioned, we have an orthotist in clinic with us. We evaluate people for different bracing, ankle-foot orthosis, and that's AFO and supramalleolar orthosis or SMOs to help optimize gait. Um, people with CPE often, um, have, uh, indwelling thumbs where their thumb moves into their palm, which can be, um, difficult for using their hand, and even for people who are not using their hand can be difficult for hygiene. So their hands. that we often measure people for. For people that have a lot of hamstring spasticity that need to improve that range of motion, we'll think through knee immobilizers at night. A lot of our younger children, in particular, have a lot of, um, trunk low tone. So trunk support vests, Benneck vests, um, can be really helpful for sitting, um, which can also be really helpful for, um, having someone engage with the rest of the world. If you can't sit, you can't look at the person who's trying to talk to you. So, um, trunk support vests can be really useful for that. Stretching splints are useful at the ankle for people that have a lot of um uh gastrocnemius or um soleo spasticity. It can really help improve range of motion there. And, um, for people with, with, uh, mild weakness or hypotonia at the ankles, um, even very simple shoe inserts that we can assess people for can be helpful. Um, of course, we use a wide range of pharmacologic interventions for tone. Um, I have the slide up here, um, and it's, it's directly taken from a paper we published last year talking about, um, the indications for different, um, medications for tone management, contraindications starting in max dose. Um, I, I have it here mainly as a reference slide, but also to let you know that there's a wide range of pharmacologic intervention options that we use for tone management. Um, in addition to that, we have injectables that we use. So, um, in a goal-targeted way for people with focal tone needs, we use botulinum toxin that we do every 3 to 6 months for 2 to 3 years at a time. We really do not like using this as long-term management. Um, We really try to do this in a time-limited way. The emerging data suggests that this kind of makes sense if we just think about it in a gut check level. Um, chronically weakening someone's muscles using Botox for, you know, 1020 years, which is, you know, what happens to a lot of our kids with CP if we start Botox very young, um, is not great for the eventual health of that muscle. So, we really try to use Botox in a goal-targeted way. Um, so, for example, if someone is, um, really trying to optimize reaching and, and grabbing for, um, an object, so reaching and grabbing for a toy they like, but they have a lot of bicep spasticity, we'll pair, um, bracing with Botox, with occupational therapy for, um, a, a predefined set, set of time. Um, to make sure that, that we can get that child to get that motor pattern and trained. And after that, we'll wean off the Botox. So, um, this is a newer sort of development in how we use Botox, and it's something we very carefully follow with CP management, um, with children that have a lot of, um, leg, um, abduction tightness, so kids that have a lot of trouble with diaper. because their legs are crossed, um, or people that have a lot of trouble walking because their legs keep crossing. There are other, um, options for injections, um, called phenol or ethanol injections that can be painful. So that is done under anesthesia. We do our Botox, um, very often, not under anesthesia as an outpatient procedure. Um, so these are other interventions that we offer. Um, everybody with CP, um, should undergo hip and scoliosis screening, um, with, um, X-rays. There are very clear guidelines for hip screening, and, uh, the link is there, um, and then that's based on your ambulatory status, which I'll, um, show you in a little bit, um, how you determine that ambulatory status. And scoliosis screening again is based on ambulatory status and should be yearly done after age 8. The reason for this is, um, catching, um, evidence of hip dysplasia or rapidly progressive scoliosis early can prevent future pain, future arthritis if, if we address it early. So, um, that's why we start, we do the screening young and, and keep a close eye on it. Um, surgical options that we offer, um, and we do a lot of work trying to determine collaborative, collaboratively who's the best candidate for each of these surgeries. So, um, we have a really robust, um, collaboration with our orthopedic surgeons who, um, will do, um, either, or both, soft tissue and bony surgery, um. They, um, in the legs and in the arms to help address, um, people that have issues with contractures or issues with needing greater range of motion. Some of those, um, example surgeries are listed here. Um, we will put intrathecal baclofen pumps in people that, for example, have really good response to oral baclofen, but, um, The bioavailability of oral baclofen drops pretty considerably at higher doses, so intrathecal baclofen can really help with tone management if we need to increase the dose of baclofen. Um, you all may know that, um, WSU and, and Saint Louis Children's Hospital in particular are leaders in, um, offering selective dorsal rhizotomy and deep brain stimulation to manage spasticity and dystonia, which are two very common types of tone issues in people with CP. Um, the selection of the right patient for each of these surgeries is absolutely critical, and this is something we collaborate with our neurosurgeons with. Amy Vihover in our clinic is, um, one of the world's experts in deep brain stimulation, candidacy and programming for this particular population. So, um, this is something that, uh, is added to our armamentarium of, of things that children are eligible for, um, to help manage their tone and make sure that they can achieve the goals that they need to achieve. Lastly, um, we do a lot of work making sure that people have the equipment they need. So, um, we go through needs for, um, early mobility, um, like go baby, go. So at, at a very young age, really even starting at, at 9 to 12 months, we will figure out in, in children who Um, may not be able to walk independently, how we can assist them with getting around and exploring their world, um, using other, um, opportunities. GoBaby Go is, um, these just really heartwarming, um, cars, uh, adaptive, um, little cars that we, um, can set, uh, young children, um, with CP up with to make sure that they have the option to explore their world. Um, we go through manual versus power wheelchairs, the value of adaptive strollers, how to help people, um, sit safely in a bath to get a bath. Um, same thing for activity chairs. As people get older, um, their homes will often need lifts for transfers, um, from bed to toilet, for example. So we'll talk through how to do that. For people who don't stand independently, there are standards that, um, we'll set people up with. Um, through our equipment partners and we evaluate people for that, measure for that in our clinic. And then also very critically, very early for people who, um, do not communicate verbally, we will, um, set up, um, or we will evaluate them for eligibility for certain communication devices, and our augmentative communication partners at Children's are, in my opinion, unparalleled in, um, being able to figure out the right communication device for the right. Kid to make sure that they can communicate with the outside world. Um, getting people referred for these as early as possible is great. Um, many of these, um, there are issues with insurance coverage as people get older. So, um, anything you can do to get people referred to get the equipment they need before they have to come off their parents' insurance is, is great. Um, so these are, these are the kinds of things we, we consider. OK. So, um, that was a whirlwind overview of how we approach care and the things that, um, we consider offering, um, to help manage tone, range of motion, and mobility, um, and optimize quality of life in people with CP. Um, next, I'll talk about, um, the, the way that we document baseline gross motor function and, and the way that, that you can too, to make sure that we're tracking, um, mobility appropriately in people with CP. So, gross motor function. NCPs is identified through the Gloss Motor Function Classification System or GMFCS. Um, you, you saw that we use that, um, I already used that acronym earlier when I was talking about HIP screening. Um, it's a 5-level system. So, um, levels 1 and 2, this is a child that can ambulate independently without using an assistive device. The only difference between someone at level 1 and level 2 is whether they need to hold on to something to go up and down stairs. If they do, that's a level 2. If they don't, that's a level 1. For people at level 3, they're, um, they need to use an assistive device to be able to ambulate independently, but they can still, um, ambulate independently. Level 4 is someone who primarily uses a wheelchair to get around, um, and they're able to operate their wheelchair independently. So this is someone who may, um, be eligible for a power wheelchair. And level 5 is someone who uses a wheelchair to get around and requires someone else to operate that wheelchair for them to be able to, um, mobilize around, around their world. So these levels are, um, valuable for us because they really triage all of our screening interventions and, um, Uh, correlate with the rates of coexisting conditions in people with CP. Um, they're also important, oops, OK. They're also important to think through, um, when we're looking at tracking gross motor function overall. So, Um, each of, on the Y axis here is a more detailed measure called the GMFM, the gross motor function measure, which is a, a score for, um, motor impairments. The higher the score, um, the, the fewer the motor impairments. And you'll see that level one stabilizes at a certain score and level 5 stabilizes at a certain score. The important thing that I want to highlight here is the stability. So, if you are at a level 2 at age 6, you still should be at a level 2 at age 15. If you're at a level 3 at age 6, at level, at age 15, you should still be at a level 3. You should not be dropping levels. Um, the reason that's important is if you see one of your kids with CP for any reason drop a level, um, that is a criteria for potentially very urgent investigation as to what's happening with that child, um, and, um, an urgent sort of at least call to neurology to think through, you know, why is this child who was previously walking. No longer walking, or why is this child who was previously not using a walker and now having to use a walker? Um, that could mean a variety of different things and should prompt, um, evaluations. So just, just keep in mind that if you have a kid with CP and they're changing on one of these levels, uh, let us know cause that, that should trigger evaluation. Um, there are a wide range of other validated functional classification systems to consider across other modalities. I have them listed here. If you're interested in how to apply them for kids with CP, that's the QR code linking you to that PDF cheat sheet. Um, I will say that we document all of these functional classification systems in our, in our notes to help us track function over time. I would say that in, if, when you see someone with CP, the GMFCS is, I think, the, the paramount one to document. So tracking that one can really help triage evaluation, um, and then the other classification systems are available to you if you, um, would, would like to use them. OK. So we've talked about how to document baseline gross motor function in people with CP. Now, let's talk about all the coexisting conditions that people with CP have, cause right now, we've just talked about movement and tone and range of motion, but what about all the other stuff? So, we recently published um an overview of all of the coexisting symptoms that were present in our clinic. Um, this was a group of 633 kids that we've seen over the previous 9 months, and This is a, a graph of our catchment area. This is where um these 633 people had come from. I say kids, but that this actually spanned until age 30. And, um, for an overview, um, for this paper, which really does give an overview of how CP presents in the Midwestern US based on the, the folks in our clinic, you can, that's the QR code to scan for that. Um, the range of associated symptoms are, are quite broad, so, and all of them can affect function. So about 20% of people have have sensory impairments. Um, we see that often that sensory impairments can drive, uh, pain, irritability, and mobility. If you can't feel where your arm is, you're, you're more likely to hit it against things and not be able to know how to use it. Same thing for your feet. So, um, with our occupational therapy colleagues and with medications, actually, we work on optimizing sensory issues in people with CP to, um, foster mobility. Um, a, a good proportion of people with CP have, um, epilepsy, uh, and some of those seizures have been present, um, since birth, since that initial. Um, ideologic driving, uh, force for cerebral palsy. There's also a higher rate of new onset epilepsy in people with CP. So, um, it's important to screen for that, or if you see that a child with CP has cognitive regression, so was, um, uh, maybe speaking a little bit better at the beginning of the year, but is now not speaking as well anymore, um, evaluating for potentially, um, seizure-based contributors to that could be valuable, and obviously we can help with that. Um, a good chunk of people with CP have sleep issues. Um, checking for that can really help optimizing their function overall. Um, over a third, and at, at this point for aggregate, um, contributors to this, it's likely far over 1/3 of people with CP have, um, mental health or behavioral health issues that includes anxiety and autism, which are the two most common ones. So, um, we do our best to target therapies and, um, medications specifically for that. The majority of people with CP have pain. So, um, the etiologies for this pain can be multifactorial. It can be pain from being tight, pain from positioning, um, pain from bony issues, um, pain from, um, other sort of visceral issues like constipation or indigestion. Um, it can also be a central pain. So, people with CP, um, the majority of people with CP have sustained some sort of global brain injury. So, that affects the thalamus, which predisposes you to central pain syndromes and can make you more susceptible to pain as you age, is what our data is actually showing. So, um, that, and that pain can be very functionally limiting. So, um, thinking through how to manage that pain and the etiologies of that pain can be, uh, valuable for optimizing function in people with CP. Um, as I already mentioned, communication is a, is a big issue for people with CP. So over a third have some issues with, um, communicating, um, with or without the use of a communication device. So, uh, referring to communication experts, um, to help optimize that can be valuable, and over half have other sort of systemic issues, primarily respiratory and gastrointestinal. Um, this includes things like constipation, delayed or, um, uh, reduced gastric motility. Um, uh, asthma on the milder end of respiratory issues. Um, the minority of our children had, um, uh, required tracheostomies when coming out of the NICU and so have residual things like, um, drooling and poor secretion control. That then make them predisposed to things like aspiration pneumonias as they age. So these are all things that um we watch for and can be more common in people with CP and managing these things proactively can really help optimize um uh mobility and function. So these are, these are things that um we do our best to manage as we um think through, uh, how to care for people with CP in our clinics. OK. So, um, coexisting conditions, there are a lot of them. Important to screen for them and optimize them, um, which we do our best to do. And then lastly, I wanna focus on, um, lifespan care. So, um, I think we often think about CP as a pediatric thing, um, but it's not, as it turns out. So, um, as I, as I mentioned very briefly earlier, The majority of people with CP alive today or over the age of 18. CP is no longer a life-limiting condition, so to have CP, particularly if you are independently ambulatory and have CP, there is no change to your expected lifespan. And currently over 40% of people with CP are over the age of 30, so it's not just that the majority is over the age of 18. A good chunk of people are, are well outside of the range at which a children's hospital might typically see them. And, um, they are, I'm sure, very likely in, in your clinics. So, um, a big issue in thinking through, um, on our side, how to transition people from uh pediatric multidisciplinary CP care to adult care is thinking through, um, guardianship. So, um, how they'll make medical decisions, what they're going to do all day after they're done with school, um, financial support. So is, is your child going to be able to have a job? If not, what's the financial plan? And really at the bottom of that list is what, what sort of medical needs do you have, um, which we can, you know, help organize and refer for, um, but it's these other questions that I think, um, often keep families up at night, um, with regards to, you know, what's gonna happen with to my child with CP when I die. So, um, we have a systematic approach to this in our clinic. We start preparing kids, um, at age 14 for a transition to adult care. We follow our, um, families through, um, until the person with CP is, is 24. And then there is an adult CP center at Barnes that we can refer to. You saw our catchment area was really broad. Not everybody can, can come to Barnes to the adult CP Center, and that adult CP center has quite a long wait on its own. So, we work very closely with local providers to make sure that, um, all of the, um, person's needs. are met, um, from a medical standpoint and also thinking through what resources they might need for, for example, legal resources for guardianship, um, or local resources for thinking through day programs or access to a homemade and thinking through financial planning. And our social worker, in-clinic social worker helps with a lot of these things. Um, you'll see that, um, all of this information is, is initially entered by the family, which we pull into our note. Um, so this is a part of all of our notes that, um, I'm, I'm sure many of you already have people that are seen in our center, so you may have seen this documented in our, in our notes, um, for the people that we care for that are seen by you. Um, for adults with CP, uh, the, the impact and, and the ongoing needs for care do shift. Um, and the neurologic screening recommendations in particular, um, we outlined in a, um, multi-site, um, set of recommendations, um, that we published in 2021. And this is recognizing that adults with CP have twice the risk of stroke compared to adults without CP. Um, this next one is critical. Adults with CP have 8 times the risk of myelopathy. So, if you have an adult with CP that was previously not calfing, and now they are, that's a huge red flag. That's not necessarily the typical evolution of CP. In fact, I can promise you that is not the typical evolution of CP. Um, This high risk of myelopathy often goes, is often very difficult to detect in people with CP and can go missed. So, um, there are actually recommendations that, particularly in people with a lot of high neck tone, that they should be getting regular screening for myelopathy, um, or for at least, um, um, uh, cervical stenosis regularly, um, and that's because of this, this high risk. So, if you see any signs of someone in adulthood, Um, all of a sudden having to cath or complaining that, um, their hands are losing sensation or losing mobility function, um, that is, that is not typical and should warrant investigation, um, but because those signs are so difficult to catch, often people, adults with CP will lose mobility and will catch these things too late. So, um, be wary of these risks, um, and, and look for them in adults with CP to catch them early. Um, adults with CP also have 9 times the risk of dementia, or, and that's early onset dementia, Alzheimer's disease-related dementias. So again, in, in an adult, even if they're in, they're in their 30s or 40s, if they're complaining of memory loss or cognitive slowing, um, obviously the workup for that is quite broad, but, but think about referral for cognitive screening and thinking through whether, um, dementia, early onset dementia could be something that this person is affected by. And then as I mentioned before, there's um a 9% rate of adult onset epilepsy. So this means people with CP who, who are not on any anti-seizure meds, may not have had any seizures in childhood, um, 9% of those people will have new seizures in adulthood, and that's about 9 times higher than the general population. The general population risk of epilepsy, adult-onset epilepsy is around 1%, so 9% is, is quite a bit higher. Um, so again, triggers for this, um, thinking through cognitive decline or behavioral changes or, um, mental status changes in an adult, in an adult with CP should, um, prompt evaluation for these things or referral for evaluation for these things. Um, I mentioned that, um. Uh, the goal is to not, is to not lose mobility as an adult with CP is to not succumb to things like, um, um, stroke risk or myelopathy risk. But the truth of the matter is that these things are often caught very late, um, in adults with CP and also adults with CP lose access to things like therapy services or multidisciplinary CP clinics. And so, um, loss of mobility in people, in adults with CP is quite common, um, as, as we've, as we've published. So, Um, if you see that, um, it's, I, I would just kind of reiterate that this isn't, um, a natural evolution necessarily of what CP looks like as people age. It should warrant, um, uh, neurologic evaluation for a potential neurologic or other contributor to the, to the reason for that loss of mobility. Um, there's also, um, the majority of adults with CP experience chronic fatigue and chronic pain. That could be to things like um new onset sleep apnea or central pain syndromes, and the natural evolution of CP is in fact to have increasing pain as you age, um, starting in your early twenties and increasing after that. So thinking through pain management strategies and, um, sleep management strategies, including sleep studies, referrals can be helpful, um, for both of those things. All of this, um, this data and, and guidelines for what to screen for, um, I mentioned that we published this a few years ago. This is the paper, it's, it's free, um, to access, and, um, there's a table in it that, um, we hope is helpful. Um, it's targeted to neurologists caring for adults with CP, but, um, for, uh, clinicians that are, um, caring for adults with CP, these are the kinds of things that we would recommend establishing, um, to ensure that, um, someone with CP to be able to track whether someone with CP is declining or not. Um, many adults with CP do not have a baseline brain MRI. Um, I had someone, um, an, an adult who, um, flew in from California to see us. He's, he's 35, and they brought their MRI films from when they were a baby. It was actually a miracle they had MRI films at all, but they brought their MRI films on, on, um, On a film, on like a radiologic plate that, um, at this point, that plate was degraded to the point where like we couldn't quite appreciate what the issues were, and they had lived their life without a baseline brain MRI um since then. And why is that important? Um, if someone has um an Detected progressive condition, or if someone is accumulating um motor disability through very small strokes, having a baseline brain MRI to compare to can be incredibly valuable to know what's changing from a neurologic or from a, from a neurodegenerative standpoint. So, um, this is something to, to make sure that, um, folks with CP have on file. Um, Genetic testing is on here, um. Most adults with CP sort of miss this genetic testing revolution that we are experiencing now on the pediatric side, and it can really help guide screening for other coexisting and potentially life-threatening conditions like those cardiac conditions I was mentioning. So, um, thinking about whether you can refer for that for adults with CP is valuable. And then also just having their baseline function documented. Here, I say baseline neurologic exam. If you have the GMFCS or the ambulatory status for someone with CP documented, that is in and of itself a huge boon because then if another practitioner or someone on the inpatient service who they might be admitted and is evaluating this person sees that they came in walking and, and now they're not, that should trigger um an urgent evaluation for why that's the case cause CP should not do that. So, um, these are the kinds of things we outline, um, specific screening recommendations for each of the risk factors in adults with CP to look for. Um, feel free to access that or, or email or call with any questions about those things. So, um, summary of that, um, for people that see those babies, those infants, recognize the CP signs early. Um, if you missed the, the link, the link to that pocket cheat sheet, feel free to reach out to me. I'm happy to send it to you. Um, approach care as goal-driven care, thinking about the, the context that that person lives in. Um, I laid out the wide variety of things that can be done to optimize care for people with CP. We're no longer in a place where, you know, you just have CP and you have to deal with it. There's a lot that can be done, so please do feel free to refer to us so we can help, um, think through those management strategies. Um, do your best. To document baseline gross motor function in someone with CP, particularly noting that, especially in childhood, that should not change at all. And then in adulthood, it might change, but that may be because you're missing other things that should be addressed, like, um, silent strokes or myelopathy. Um, recognize the impact of coexisting conditions and on, um, the, the primary care side that can really include sleep and anxiety. Management of both of those can be dramatically helpful for improving mobility. And then lastly, think about lifespan care and the myriad ways in which CP requires, um, screening and referral, um, through adulthood. Um, CP is no longer a life-limiting condition. The majority of people with CP alive today are adults. Um, please consider these management strategies for them too. Um, this is, uh, just some brief information for our clinic on how to refer. This is a link to that cheat sheet again, which also has those referral strategies. Um, I talked a lot about CP today, but we see, um, any child, so anybody less than 18 years old with any movement condition, we see. Um, and a lot of that is CP, but it doesn't have to be. So, Um, as, uh, Nikki mentioned at the beginning, I specialize in tics, Tourette's, um, dystonia, which is another movement, um, condition that is enlisted here. But if, if someone you're seeing has a movement issue, feel free to send them on over. We'll, um, Uh, do our best to diagnose, treat, and optimize their, um, their quality of life. And we do follow people at our CP center up to age 24, after which, um, as I outlined, we coordinate, um, transition of care and referral to other adult practitioners, including if the family chooses the Barnes Adult CP Center, which is run through physiatry. Um, if you want to reach out to us for a referral, the fastest way to do that is to email me, and to be honest, probably even faster is to email my nurse coordinator. That's Lauren Gottschalk listed here. As I'm sure many of you know, the, the nurses are the entry point to everything, and Lauren Gottschalk is, in my very biased opinion, the best of the best. So, um, please email us with any questions or if you, if you feel like a referral needs to be fast-tracked, you can also call the child neurology office or call Children's Direct and ask for the CP Center. Um, and with that, I, uh, this is a picture of all of us, um, at Christmas. I'd like to thank my CP Center and, and you all for, um, listening to me talk for so long. Thank you. Created by Presenters Bhooma Aravamuthan, MD, DPhil Pediatric Neurology, Neurology View full profile
