Rachel Byrne:
Hi, and welcome everybody. My name is Rachel Byrne. I am the Executive Director of the Cerebral Palsy Foundation. I am so excited to welcome you to today's Town Hall, which is focusing on cerebral palsy, adult healthcare throughout the lifespan. I'm really thrilled today to have four incredible speakers joining us. And, I'd like to introduce them to you now. So, Dr. Hank Chambers is the Pediatric Orthopedic Surgeon at Rady Children's Hospital in San Diego, and he also serves as the Professor of Clinical Orthopedic Surgery at the University of California in San Diego. He treats pediatric gait disorders, sports injuries in cerebral palsy, and his research areas include cerebral palsy, motion analysis, and controlling spasticity with botulinum toxin. His wife, Jill, is active in many local and national patient advocacy groups and is a healing touch provider at Rady Children's Hospital. His son, Sean, who has cerebral palsy, is currently an assisted living to situation in San Diego and his other son Reed is a Pediatric Orthopedic Surgeon at Nationwide Children's Hospital.
Rachel Byrne:
I'd also like to welcome Dr. Heukyung Kim who serves as the Vice Chair of Rehabilitation Medicine and Professor of Pediatrics and Orthopedic Surgery at Columbia university, the Irving Medical Center. She's a board certified physiatrist in the USA and South Korea and holds subspecialty boards in pediatric rehabilitation medicine and brain injury medicine. She specializes in single event, multi-level chemo neurolysis with botulinum toxin and fetal alcohol injections. Botulinum toxin injections to salivary glands, musculoskeletal ultrasonography and pain management in children's and adults with cerebral palsy. Her research interest focused on spasticity management and robotic therapy and exercise for weak spastic muscles in children with cerebral palsy and journaling management with botulinum toxin as well as quality of life for adults with cerebral palsy.
Rachel Byrne:
Our next speaker is Dr. Mary Gannotti who is a physical therapist who teaches at the University of Hartford in Connecticut and performs clinical research at the Shriner's Children's Hospital in Springfield, Massachusetts. Dr. Gannotti has more than 25 years of pediatric clinical experience and has been involved in clinical research for more than 15 years. Her clinical practice focuses primarily on the care children and adults with cerebral palsy. Her research involves a study of measurement tools, cultural influences on care and factors influencing treatment effectiveness and the long-term physical and social outcomes of adults with CP.
Rachel Byrne:
Our final panelist today is Dr. Mark Peterson, who is the Charles Little Research Professor at the University of Michigan. Dr. Peterson's work focuses on understanding the factors that influence health and life expectancy among individuals with disabilities across the life span. This includes efforts directed at identifying precision strategies to prevent cardio metabolic dysregulation and secondary physical and psychological morbidity among children and adults with cerebral palsy, as well as a variety of frailty syndromes, and to better understand health disparities among individuals with disabilities, from the context of access to preventative care and community wellness.
Rachel Byrne:
So, without any further ado, I'm going to welcome our panelists now onto the screen. And, I'd also like to say a big thank you to Ipsen Biopharmaceuticals who have sponsored today's town hall, but also who are supporters of all our New Horizons Town Hall series. So, welcome everybody. So now, wonderful, our panel is now live. And, as you can see, we've got an incredible group of speakers here joining us today. And, thank you all. I'm really excited for this conversation. And we have, as I said, a lot of people who have already put in some wonderful questions. But, to get us started because we've obviously already had a little bit of delay, I want to start with a question to you, Dr. Chambers, since cerebral palsy is the most common lifelong motor disability, what are some of the key issues adults with CP should be aware of across the lifespan?
Dr. Hank Chambers:
Well, that's a loaded question. I think that's what we're going to be talking about the rest of the time. But, just because you have cerebral palsy doesn't mean you're not a human being and you're not aging as everyone else is. So, the problems that people that every typically developing people have with high blood pressure, heart disease, those kind of things, that's still a problem for people with cerebral palsy. But, there are other challenges such as loss of mobility, pain, some decreased function, some people lose their ability to speak as well. There are a lot of different problems and I know we're going to go into a lot more detail on all of those. But, I think we need to remember that there are just some additional things on top of being a typically developing adult, that we have to not ignore those things either.
Rachel Byrne:
Yeah, definitely. And, I think we are going to try and cover as much of those different topics as we can today. Obviously, as you said, when we're thinking about adult healthcare, there really is a multitude of things that we could talk about, but we are going to try and, as I said, cover as many as possible. So the next question, and this is for Dr. Kim, when we're thinking about the adult audience and particularly some of our listeners today may not have received some information when they were younger, can we just do a quick explanation on the different types of cerebral palsy and some of the different basic information around functional levels?
Dr. Heukyung Kim:
So, the different types of CP we divide by their movement problems or what area they have difficulties. And then, the other one is based upon their functions. So, if their movement is more based on spasticity which is a stiffness when you stretch, so spastic type dystonia, which is involuntary movement, it comes and goes kind of a stiffness. And then, rigidity we found. So, they're really rigid no matter what we do. So that happens, the rigid type or the mixed type. They all together with some patient is very low term. By function, if you are able to walk without any assisted devices in any terrain, and no difficulties, then one. Two is, they can walk without any assisted devices, but they may have some difficulty with some rough... obviously, outdoor is very tough, or sometimes they have to hold the side rail. Three is, they are very independent on regular wheelchair or a walker. Four is, they're very independent on power wheelchair. Five is, more totally, I would say, dependent. That's the way we divide.
Rachel Byrne:
And, I think understanding sort of the different types of cerebral palsy and the different functional levels, can you sort of explain why that is important? Because, does it change what interventions may be happening and does it change potential trajectories when we think about adults and aging?
Dr. Heukyung Kim:
Should I answer?
Rachel Byrne:
Sure. Yeah. Yes, please.
Dr. Heukyung Kim:
I would say, I think it's extremely important for us to have some idea of a trajectory of their condition. So, we don't want to just treat the patient, but we want to know how they're changing, so therefore we can make the perfect treatment plan or perfect support for our patient population. So having motor function classification is very important for us to make the treatment plan. And then, we also know what we can expect in the future. Then, if we know how they change, it also helps the research team to go over what they can take a look and what kind of outcome we can project.
Rachel Byrne:
Yeah, definitely. And, sort of the comment that comes up to me there as well is sort of thinking about that moment, particularly if there's parents watching with adolescents and even young children as part of this conversation, how important is for individuals themselves to become part of their healthcare process and understand what's happening with their bodies and all those different pieces. So, I've got a question for you now, Mark, and we've actually had this come through a lot today from our listeners. This is sort of thinking about the adult experience and their CP. And, a lot of the time we're getting asked, is their CP getting worse? How do they know if it's getting worse and what does that even look like?
Dr. Mark Peterson:
Yeah, so by definition, CP is a non progressive neurologic condition. And, as Dr. Chambers alluded to, there's progression in sort of the symptomology of CP because of age and we all experience age related changes in our health and function and capacity to participate with age. It just happens to be at a little bit accelerated rate for folks with cerebral palsy. And so, while the definition of CP holds true that the neurologic condition does not change, it's non-progressive. And so, no, CP does not change and CP does not get worse. The other organ systems, including muscles and bones and heart and brain, does age.
Dr. Mark Peterson:
And so, there is a progression and change of functional capacity across those systems, even early in life. So earlier than what we would expect to see in older adults. Folks with CP might expect to see some of this happen in the third and fourth decades so 30s and 40s, which we may not typically see until 60s and 70s in a typically developed population. So it's a really good question. It's one that I think a lot of people could argue about, but CP does not progress, but body does age.
Rachel Byrne:
And so, I think that's sort of a great to point out. So, while the brain injury itself, or the brain malformation or whatever causes cerebral palsy, while that's not worsening, there's obviously a lot of different elements that are happening. But, the question that we are also getting, well, does the brain change though, as we age? So while cerebral palsy, maybe that side of things isn't progressing, but what's happening in our brain? How is that changing as we age? And, can we do anything about it?
Dr. Mark Peterson:
Do you want me to answer that?
Rachel Byrne:
Yes, please.
Dr. Mark Peterson:
Yeah, so I'm not a neurologist, but I can tell you that every person in this room, including those on the panel, our brain's age, that does not necessarily mean improved performance. So, there's cognitive declines that come with age, just like there is functional declines that come with muscle aging. And so, brains very much like a muscle if you don't use it, you lose it in certain ways. And so, it's really important to continue to be active physically and cognitively across the lifespan to preserve functional capacity of the brain. We know a little bit about cognitive health in adults with CP here at Michigan and those in the audience and those in the panel are very interested in cognitive health and psychological health. And, there may be some evidence now arising that suggests that adults with CP may be a little bit higher risk for early onset dementia and things like psychiatric morbidities, including depression and anxiety earlier in life than typically developed adults without CP.
Dr. Mark Peterson:
So these are things that are important to know, they're important to screen for, important for physicians to know. And, the reason why we're in this room right now is because there is not as many providers for adults with CP as there are for children with CP. And so, that conversation around this age-related changes is exceptionally important for those in the audience to understand so that they can go back and talk to their primary care doctor about some of the stuff that we're going to bring up today.
Dr. Heukyung Kim:
So, can I add?
Rachel Byrne:
Oh, absolutely.
Dr. Heukyung Kim:
Yeah. So, I totally agree with Mark. I think we are getting old, but I think I like to emphasize that... I think he mentioned about mobility... we have to move around not to get deteriorated, but mostly typical like us, we are slowly changing, but people with cerebral palsy has difficulty with moving around that really exacerbate everything, losing their cognitive function or movement. So, what I want to make the point here is that if we know what we are going to get, we can prevent actually many, many things. Even though we just say that, oh, earlier your brain may deteriorate a little bit earlier, or your body may deteriorate a little bit faster than other people. But, if we move around our own body, our brain, you can prevent many, many things. That's what I want to emphasize. That's why we have to educate physicians ourself, provide ourself that we can prevent a lot of different things, but we don't know how to prevent. That's the problem. So patients themselves can bring this one to the providers that they should study. They should protect our patient.
Rachel Byrne:
Yeah, definitely. Oh, sorry. Mary, would you like to add something?
Dr. Mary Gannotti:
Well, I totally agree. Exercise is medicine and it's the answer to anything. And, if you oxygenate the brain, it's going to do better. And, if you exercise, the muscles going to get bigger. But, we do have to be aware that adults with CP, of course, there's neuroplasticity, just like there's typical adults that don't have CP who have neuroplasticity. We can still learn. But, unfortunately, adults with CP are at risk for neurological sequela because of their condition, whether it be a blood vessel because part of their brain isn't completely formed, or whether it is some sort of orthopedic deformity that causes impingement on the spine.
Dr. Mary Gannotti:
So although CP is static, our nervous systems are not. Just like our muscles and our bones and our hearts are plastic so is our nervous system. We have to be aware with aging that you might have increased risk because of your posture or because of the way your central nervous system is formed.
Rachel Byrne:
So I think you brought up some really sort of wonderful things. Hank, I definitely want to go to you next as well. Because, we've spoken about, I suppose, well, what's happening in the brain and, yes, we can change it. And there's all these other things that potentially are happening as well with aging and hopefully through exercise and mobility that we can lead to improvements. Hank, I think, just thinking about how many different topics we want to cover tonight, Mary did sort of mention bone and sort of thinking about what's happening orthopedically and what's happening in adults there as well. Could you expand a little bit more on that? Like, thinking about, all right, what is happening at the joint level, what's happening at the knees and hips and ankles and the spine? And, is there things that we can do from a prevention perspective and are there things, once things potentially have deteriorated, that is best practice?
Dr. Hank Chambers:
Yeah. I'll answer those questions, but I do want to get... Ask me again later about mental health issues because I have a lot of input on that. The bone stuff, I think is really important, and what Dr. Gannotti was talking about, exercising. So, the more you exercise, the more you move your limbs, the better your bones become. It's a law, they become stronger. But, many of our patients, particularly as Dr. Kim was talking about, that are in a wheelchair because that's the level of their cerebral palsy, they aren't able to bear as much weight and their bones are more prone to fractures. And, their muscles are getting weaker as they get older because they're not exercising. So, that's all very important.
Dr. Hank Chambers:
One of the problems that we see in adults with cerebral palsy, because they don't have a full range of motion of their knee, for example, or their hip when they're walking, they end up walking on a very small area on their bone and they wear that area down and they have premature arthritis. So, those of us who might have full range of motion, some of us don't have full range of motion, but you start wearing those areas down and you might need a total joint replacement when you're 70. But, many of our patients, our adults are having total hip replacements in their forties, and total knee replacements in their 40s because they just worn down that because they have an abnormal movement, they have weak muscles and unfortunately they have weak bones. And so, the bones break right around the joints and then you get a deformity.
Dr. Hank Chambers:
Then, of course, if your bones aren't very strong because you don't have good nutrition, you're not exercising or you've just fallen or something and have a fracture. You have more chances of having a bad outcome, even after the adult surgeries that we do.
Rachel Byrne:
So, that's a question that I have for you then. So, when we're thinking about the sort of progression of these, whether it be bone deformities, or what's happening at the joint, how can we be proactive? So we sort of mentioned exercise. Is there a point that sort of there's early warning signs thinking about, okay, I should be going back and seeing my orthopedic surgeon or I need to go in and get a referral to know what's happening at my joint?
Dr. Hank Chambers:
Yeah. That's true. One of the problems is, there's a sweet spot in treating many deformities in kids. You have to treat them as kids. When you try to do surgery on adults with cerebral palsy, the outcomes just aren't as good. The muscles are really stiff. The joints have already been injured because they've been in a bad position their whole life. So I know a lot of people on this are adults or their parents are adults and they go, we've kind of missed that boat. But, I want to stress that, that's the important time. We can do some things in adults surgically to help, but it's not as good as when they're young. But, to increase your joint health, to be in bone health, just to take vitamin D and calcium, get out in the sun. Even if you not doing it yourself, having your joints move through a range of motion so they don't get stiff. I think those are all important aspects for adults.
Rachel Byrne:
And so, Mary, I'm sort of going to ask you this question now. When we're thinking about mobility, it's sort of been brought up as something that really can help and do all those different things. But, when we talk about mobility, obviously there's a couple of things that can sort of impair someone's ability to be mobile and to be physically active. That it can be, A, painful and just really hard to do and it takes up all their energy, but, also, we spoke about spasticity, but, also, their muscles could be really tight as well. And, it's just hard. So what sort of recommendations do you have out there, or what's the research telling us when it comes to physical activity and exercise for adults so that we don't cause overuse injuries or we don't cause something else that's going to do more harm?
Dr. Mary Gannotti:
Well, I agree with Dr. Chambers about the fact that when you work with adults with CP, you do have to be very mindful of the muscles and the bones. And, aquatic therapy is really fantastic. And, my colleague, Dr. Debbie Thorpe at Chapel Hill has evidence to show that even people that ambulate, you can increase your bone mental density if you work really hard in the pool with CP. So, I think there are alternatives, the recumbent bike. I talk and treat a lot of adults with CP, like they have rheumatoid arthritis. And, talk about a lot of energy conservation and joint protection and then targeted physical activity for aerobic or strengthening. But, really, thinking about getting through your day on a scooter and minimizing weight bearing and stress, but then going home and having a good exercise program. So-
Rachel Byrne:
Yeah. I love when you said sort of this energy conservation, because we've got a lot of people asking questions about this in the chat right now. That it's like, okay, great, tell me to exercise, but I can't exercise because it hurts. Or, I have to [crosstalk 00:21:28] I'm too tired.
Dr. Mary Gannotti:
So, one of my colleagues, she's a disability rights lawyer and I have a couple of them, but this one in particular, she has a really high level of fatigue. And so, I said to her, "Well, Melissa, use the scooter." And, she went from a non-working lawyer to full-time employed for the state and now for the federal government as a disability rights lawyer, because she transitioned to the scooter and then got on an exercise bike. Then, was able to work and do her work really nicely and not be in pain. So, I think that working with mindful therapists or other support, whoever your support people are, how to adapt your environment and still meet your target heart rate, or doing yoga and breathing, there's a lot of accessible, beautiful exercise that is not painful. And, yoga is one of them and aquatics is another. Yoga is much more accessible than aquatics.
Rachel Byrne:
And, I love that recommendation, like thinking about, okay, when you're doing the exercise, do the right exercise and the good exercise, it's going to give you all these health benefits, but then, also, you give yourself permission to take the rest when you need to. Dr. Kim, this next question's for you because it's actually around spasticity. So, it's come in as a question in the chat as well. So when we're thinking about spasticity in adult muscles and thinking about treatment for this spasticity, if that's sort of their main complaint and issue, is it similar to children? Do the same sort of techniques work? So, for example, botulinum toxin and those more pharmaceutical things as well.
Dr. Heukyung Kim:
Yes or no, because now I have a really number of patients I can count. So I did the retrospective study to take a look how they're responding, how I'm doing injections. So actually, adults with cerebral palsy, they don't require frequently repeated injection at all. And then, they require much lower dose of botulinum toxin if they have spasticity. So, I have now another patient, I start to see more, not spasticity because, with mobility, their spasticity is not there that much anymore. Contractile muscle fiber is not there that much, which means they're just rigid. So, we see more rigidity, we see more dystonia then, when they were children. So I actually change my treatment paradigm now to really isolate each different movement to tailor the treatment based upon their different movement. So, I don't do just spasticity management. I don't say that anymore. I really carefully take a look.
Dr. Heukyung Kim:
I actually treat a lot with rigidity at this time. That's why I was emphasizing immobility because immobility really causes real logic phenomenon because it becomes rigid. You are not moving so your body is accumulating hydronic acid, which makes the patient rigid. So, that's why I was emphasizing a lot of immobilization, range of motion, dancing, whatever you can do it, move your body. You can put some hand on the ceiling and just move. Whatever you can do, just move your joint and your muscle, that's going to be the best therapy. And, the rest of them, if they have leftover spasticity, leftover rigidity, we can medically manage or we can do injections. And then, we can do some work with the orthopedic surgeon to relieve the contracted part. But, I'm totally changing my treatment plan for the adult with the tone problems now. So, I think there is more research that has to be done, but absolutely different management, I'm aiming to help them out at this time.
Rachel Byrne:
[crosstalk 00:25:42]. Go, Mary.
Dr. Mary Gannotti:
I'm sorry to interrupt. I just wanted to confirm with Dr. Kim, that in one of our surveys, when we ask adults with CP, we only have 300 people, so if you want to participate, I would like to get at least 500. The top three primary reasons are pain, changes in spasticity, and dystonia.
Dr. Heukyung Kim:
Right.
Dr. Mary Gannotti:
So although CP is a static condition, the neurological changes that Dr. Kim is talking about, patients are reporting increased spasticity and changes in dystonia as adults, whether that's related to the contractile properties of the muscle or actual neurological changes is yet to be discovered.
Rachel Byrne:
Dr. Chambers. Hank, what's sort of some of the pieces that you are sort of actively looking at the moment as well. We're looking.
Dr. Hank Chambers:
Yeah. So, I'm looking right now, on some of the treatment methods for pain that just aren't... so they aren't taking opioids. I'm using botulinum toxin for pain relief rather than just spasticity relief. And, we've got to study with about 50 patients that we've done for hip pain. It seems to be that I'm getting ready to publish or send in... Hopefully, we get published. And then, also looking at gait deterioration in adults, in the gait lab and seeing where they were when they were. There are a lot of studies that are looking at that as well. But, a lot are coming out of the University of Colorado, seeing adults and seeing what's happening to them and seeing that the gait actually does deteriorate, even though maybe they don't feel like it is. So I think that's something that, as we follow our patients throughout their lifespan, I think we're going to learn more about that.
Rachel Byrne:
Yeah, the big message is, it's always going to be individualized obviously to anybody sort of watching, but thinking that there are options. Because, I do think it's important to go, okay, have these conversations with your medical providers that, all right, the options may be mobility and exercise. It may be, if there is increased spasticity that injections are required or whether it's rigidity, then it's more another type of pharmacological intervention or as Hanks sort of spoke about, that there is orthopedic options if necessary.
Rachel Byrne:
There is a question that's come through that I think is important and it's for those of us who had mild cerebral palsy as children and who were not offered interventions like surgery then, who are now finding that their cerebral palsy is getting worse. Are there specific interventions that they want to talk to their doctors about, that they want to be sort of proactive about. And Mark, I'll sort of throw this over to you to begin with because, obviously, it depends on what they mean by their cerebral palsy getting worse, obviously. But, there are a lot of things we could be testing for and could be looking at that we do have interventions for as well.
Dr. Mark Peterson:
So, I'm as assuming if somebody is saying their CP is getting worse, what their meaning is, their function has changed with age. And, if that's the case, it probably goes back to what Dr. Kim and what Dr. Chambers, Dr. Gannotti said is that, their mobility has changed because they're rigid and stiff. And, that is not necessarily because of spasticity, but that it could be a function of muscle weakness and essentially fascia stiffening. So, the connective tissue that surrounds a muscle and that connects to the joint ultimately gets stiffer. And, that's much different than spastic muscle. And, if you can address those two things through targeted exercise.
Dr. Mark Peterson:
That might include mobility range of motion, both active and passive range of motion, and then strengthening exercise. And, it doesn't have to be physical therapy. It doesn't need to be couched as physical therapy. It shouldn't be actually, it should be direct, targeted resistance training to the muscle. There are both functional and metabolic outcomes that can come from resistance exercise that can really benefit a person, even if they don't necessarily see functional changes, those muscles are going to be healthier, the bone will be healthier, and, ultimately, the metabolic health of the muscle will be better.
Dr. Mark Peterson:
So I assume that when a person says that, what they're referring to is their functional capacity has changed with time. And to that, I would say, be kind to yourself and to go back to what the original comment was that Mary had said, Dr. Gannotti had said, allow yourself to use your mobility device, to get to and from work and school, and then target exercise when you can in a dedicated way, because the notion that we have to ambulate and that's normalized is, in my opinion, doing a huge disservice to people. And, also, packaging exercise the way Americans have packaged exercise is also doing a huge disservice to people. And so, getting in the water, doing some kind of a recumbent step or a bike, and then doing some specific mobility of the back and the hip and the upper body and the trunk, and then strengthening exercises, can really go a long way to, and not just maintain function, but also health across the lifespan.
Rachel Byrne:
Yeah. Thanks, Mark. And, sort of your comment around metabolic health of the muscle and sort of understanding that, can you just expand a little bit by what you mean by that? What is actually happening to the muscle and what changes are we hoping to get from it?
Dr. Mark Peterson:
When somebody refers to the contractual tissue of the muscle, that's not just a tissue hanging there. There's a metabolic component to that, that is using fuel all the time, whether we're active or whether we're at rest. And so, the more active a muscle is, essentially, the more metabolically healthy and active that muscle can be. The more inactive a muscle is, the less active, the less healthy and the less muscle mass there actually is. And so, maintaining muscle mass is a really important part of metabolic health. We always think of the heart and the kidneys and the liver as metabolic organs, but actually your muscle is the largest metabolic organ in your body. And so, using the muscle actually is a direct way to improve metabolic health, even if it's not in an aerobic fashion.
Dr. Mark Peterson:
So everybody has, again, used aerobic exercise as the end all, be all. And frankly, for a lot of folks, not just with cerebral palsy, but general population, aerobic exercise is not palatable. It's not fun. It's not enjoyable. It can be done and it's directly effective for the heart. But, I don't know, I'll speak for myself, I don't like repetitive aerobic exercise. So, it can be done and it's really important for improving heart health. But, you can have a huge effect on metabolic health and that risk for diabetes, for example, by engaging in resistance exercise. And, there's a thousand studies on the impact of resistance exercise to improve the metabolic health of the muscle and overall system.
Rachel Byrne:
So Hank, we've got a sort of specific question that's come through for you and it's in relation to those that have had previous orthopedic surgery, and now find themselves in a position where they potentially need a hip replacement or a knee replacement. You mentioned a little bit about the sort of success rate of those as adults, but do we know sort of what is the best treatment, for example, if you've had an osteotomy in the past, and now you need a different sort of hip replacement or something like that?
Dr. Hank Chambers:
Yeah, well, it depends on what level the hip osteotomy was done, for example, or the osteo around the knee. But usually, that's not a problem. We can work around that most of the time, we talked earlier, if the quality of the bone is not very good. Or, as we've been talking a lot about how muscle becomes stiff as you get older, that also affects what we do in orthopedic surgery. So if your hips are stiff because you can't pull them apart, but you have arthritis when we lengthen the muscle, it just doesn't lengthen as well because the property of the muscle's actually changed.
Dr. Hank Chambers:
So, we've done some research with Dr. Lieber. I work in his lab and not him in my lab. But, it's where we noticed that there's a lot of extra collagen between muscles and, as you get older, that collagen, which is the line of muscle, if you eat a steak, it's the tough stuff on the outside, that gets thicker and thicker and then the muscle doesn't move. So, when I do surgery on a young person, I lengthen the tendon and I can straighten the leg out like that. On an adult, I might do the exact same lengthening and it might go to there. That's a big deal because then the total joint doesn't work as well. And then, the point of doing that is to improve function and decrease pain. And, we lose that as people get older. So I totally agree with the movement problems, the doing the exercise.
Dr. Hank Chambers:
I want to go back to something Dr. Kim said, and it's the problem with our health system. Because, Dr. Kim and I follow patients throughout their lifespan and we see what they're like when they're 10 or 11, 12. Then, most of the people that are watching this right now have to go to an adult provider who's never seen them before. They don't know that their dystonia is now worse. That's a first time they've seen then, they go, this person has dystonia... Or, they don't even know what dystonia is... [crosstalk 00:35:26] how to treat that. So, I think a lot of people are maltreated during this time. Not on purpose, it's just that no one knows that they've actually changed.
Dr. Hank Chambers:
I know the definition of cerebral palsy is a non-progressive neurological disorder, but I see progressive neurological changes all the time. And, what Dr. Kim is saying about dystonia getting worse, I have about 20 kids, young adults in their 30s who have Parkinson's disease.
Dr. Heukyung Kim:
Oh, yes.
Dr. Hank Chambers:
Not a typical Parkinson's disease, but that causes the rigidity and the whole thing where they're, what's called, pill rolling. And, if you image their brain, it looks the same as it did when they were 10, but there's something going on in their brain. So, that's a progression of their neurological disorder.
Dr. Mary Gannotti:
What about MS? I have a lot of adults with CP that have MS.
Dr. Hank Chambers:
Yeah. I have MS or MS-like symptoms. It may not be MS.
Dr. Mary Gannotti:
Yeah. Well, MS is kind of a garbage [crosstalk 00:36:28]-
Dr. Hank Chambers:
Right.
Rachel Byrne:
So, for all of you adults watching, because I think this is the thing, right, there's a lot of adults watching, obviously, on all different functional levels. And so, I think one of the things that we've spoken about and is going, all right, so, as an adult, who should be part of your clinical care team? Mark, if you are going to put together your best team or your A team, what sort of providers should people be looking for? A, for preventative things and also for screening of things that we know potentially they are at higher risk for, or that you are sort of talking about here?
Dr. Mark Peterson:
Yeah. So, I'm not an MD in the room here. I would probably defer to Drs. Kim or Chambers. But, if I was to say, what I have seen in our clinic is that, the folks with CP adults with CP need to have a primary care doctor that knows something about CP. And, the problem with that is that as Dr. Chambers just said, primary care doctors don't know anything about CP. So what does that mean? They probably should have a quarterback physician who is a CP physiatrist and/or an orthopedic surgeon who has dealt with CP from a lifespan perspective. And then, after that, there should be an internist on the team that has an idea of looking at, essentially, the health of all organ systems.
Dr. Mark Peterson:
And if you were to say, what's the dream team? That would be a behavioralist, some kind of a mental health provider and then therapists that can speak to the needs for things like occupation therapy, sleep therapy, and obviously physical or physiologic therapy. And so, I think, basically we go from head to toe there, but there needs to be a coordinating doctor that knows something about CP. And, it shouldn't be a pediatrician. I'm sorry, it really should not be a pediatrician.
Dr. Heukyung Kim:
So, can I add?
Rachel Byrne:
Yeah, absolutely.
Dr. Heukyung Kim:
I agree with Mark, but I'm always dreaming about this kind of setting, what you just described, but it's not possible at this point. But, I also think about the physiatrist who are interested in this patient population should be the primary care doctor for them. As you said, yeah, internist, we can work with them. Or, we can have a family nurse practitioner along with that. But, someone who has to know them from the beginning. I found so many problems when they went to adult physical therapist or adult orthopedic surgeon, they never have a time to listen to them anyway, they don't listen. That's another problem. And then, they have no chance to address these things and they have to come back to us.
Dr. Heukyung Kim:
But, they don't need to explain to us when we see them, we know what is going on. We don't have a solution. That's the problem. Because, we don't have a physiatrist who are interested in this patient population. But, I kind of disagree with Mark, we need to have a partnership, but we have to have adult and pediatric care provider in one clinic together. But, I would say it has to be together. You cannot rely on adult physiatrist or adult orthopedic surgeon or internist.
Dr. Mark Peterson:
Yeah.
Dr. Mary Gannotti:
[crosstalk 00:39:54]. This is Mary. Thinking about being a mom of a child with special healthcare needs and care coordination and all of that, there needs to be... I think Dr. Chambers probably has it and Dr. Kim also, sort of the care coordinator, the nurse practitioner, that brings everything together. And, that's a service, unfortunately, that people either have to pay for or provide themselves.
Rachel Byrne:
And so, one of the comments that we've got is, and I think this comes down to actually lack of care coordination is the problem that adults face needing to have to explain their to providers over and over again. And, they get a new provider that comes in not understanding the history of cerebral palsy, who then says sort of something different. Maybe this is something that we can help provide at the foundation as well. Is there a template that any of you have, or how can we actually start encouraging more of that care coordination and making that happen? Mary, you mentioned that it's something that obviously doesn't usually get funded and it's sort of that extra cost for people that they probably can't afford [crosstalk 00:41:08]-
Dr. Mary Gannotti:
Well, we have the Title V program in each state, which does above and beyond care coordination for children with special healthcare needs. And, it doesn't extend into the adult population. In the state of Connecticut and in Michigan and Oklahoma, other states have certain waivers that allow for care coordination through the lifespan. But, it is something that I know that at can child, Dr. Gorter, had with his transition app. People are trying to work on creating notebooks or so forth. And, I know that with my son, every time we go to a new provider, I just give him a notebook and it's their responsibility to read it and integrate it, which they usually don't.
Dr. Mary Gannotti:
So, I think that in this technological age, we're able to even have a flash drive with our information on it and we have to be individual advocates for ourselves.
Rachel Byrne:
Yeah.
Rachel Byrne:
So, we have got so many questions coming through, it's amazing. This audience really wants to hear from all of you. I'm going to sort of shift gears a little bit, because this question has been asked a lot and it's around sleep. So any information on sleep quality for adults with CP? Sleep apnea and things like that. Does anyone have something that they can add to that part of the conversation?
Dr. Mary Gannotti:
Sleep is a big issue that is constantly brought up. And, I usually hear about it in regards to pain and the fact that physical therapists are involved sometimes with the OT in terms of purchasing appropriate mattresses and beds and things like that. But, sleep hygiene is really important. And, it is a big problem. I don't have the physiological input like Dr. Kim or Dr. Chambers, or Dr. Peterson would have on sleep apnea.
Dr. Heukyung Kim:
I don't know.
Dr. Hank Chambers:
Go ahead.
Dr. Heukyung Kim:
No, I was going to say, I don't know that is really sleep apnea. You know what I mean? Because it can be sleep apnea, if the study has been done, right, number one. Number two, it could be very much related to pain. Number three, if they have a type of scoliosis, it can cause the restrictive lung disorder. So they have difficulty with falling asleep or maintaining sleep. So I was encouraging people to go for the test first, which test always results is inconclusive, no matter what. But, anyway, still, I think we have to really investigate why they have this problem. I think we have to really carefully address this part.
Dr. Hank Chambers:
Yeah, I think that's important. There's a lot of different ways, reasons, for sleep problems that, and you have to work them up. Some of them are respiratory. One of the things that we found because we have so many people with Baclofen pumps, which I think are fantastic, but when you lay down, sometimes the Baclofen pump makes you only breathe like five times a minute instead of how often you're supposed to breathe. And, we've seen that when we've done sleep studies. So, it's almost related to the medication. So, also, something like that, as simple as popping up the bed makes a difference for those kids. So, you can't just say there's one treatment. Take a melatonin and you'll be fine. There's a lot of things that go into sleep problems.
Dr. Mary Gannotti:
What about constipation? I think that, that might have some contribution. It could be anything.
Dr. Heukyung Kim:
I think constipation can cause pain, right, or respiratory problem if they have two severe constipation which causes even gas distention. Then, the lung component is going to be squeezed so they can have a breathing problem. All the medical problems can cause the sleep problem, but we have to identify what is causing the sleep problems.
Rachel Byrne:
Yeah. I think that's a really big one, right, to be able to do an effective treatment for sleep, you've really got to know the reason why. And so, there's not sort of a universal piece looking at sleep hygiene and how to make it better. It really is like an individualized approach. Now, we sort of mentioned pain there and we've got to a lot of questions that are coming through in relation to pain. And Mark, I'm sort of going to throw this question back to you a little bit, thinking about the causes of pain and what they could be, can you just give an overview of, what does cause pain as aging and those different things in cerebral palsy?
Dr. Mark Peterson:
Yeah, I think that's a good segue because there is some pain that is directly caused by poor sleep and other behaviors. And, obviously, in CP, pain has been widely studied. It's one of the hallmark symptoms of cerebral palsy, especially across the lifespan. Pain also comes with aging too in non-CP populations. But certainly, it has been primarily looked at as a musculoskeletal source of pain. Not, until recently, has other sources of pain, including neurologic pain and even centralized pain, which was historically called a fibromyalgia, is now starting to be recognized as a potential major contributing for factor for pain experienced by individuals, especially adults with CP. Importantly, fibromyalgia or centralized pain is largely caused by poor sleep. So, if you can get people to sleep better. Sleep is not necessarily just an outcome, but it can be a downstream cause for issues like poor mental health, pain, and those obviously are interrelated bidirectionally.
Dr. Mark Peterson:
So, somebody who has poor sleep may have more pain. Somebody who has more pain will then have less sleep and then they may develop symptoms related to anxiety and depression. So, I always think like Mary does, that exercise is medicine, but I also think sleep is medicine, too. And indeed, there's an association between sleep and exercise. And so, they all very much are interrelated. And if-
Dr. Mary Gannotti:
And, pooping.
Dr. Mark Peterson:
Yeah. Right, and bowel and bladder function. So if we can get people exercising and sleeping and have healthy nutrition, we can solve a lot of the problems in our population, in our patients. And so, again, not as an MD on the panel, but somebody who is really very heavily interested in the research about the pathophysiology of some of the things happening in CP. A lot of this does boil down to behaviors and sleep and exercise and nutrition. And, this is not just specific to CP, we can say this across populations, that those three behaviors can take care of almost everything, especially when it comes to organ health and mental health. So, I definitely think that, that's a nice segue, Rachel, thank you for bringing that up. So there are other sources of pain that are not necessarily caused by CP and this just happens to be one of them.
Dr. Hank Chambers:
Some of the things that I see that cause pain that are undiagnosed are people that develop gallbladder disease. People don't recognize that. One of the most common one that's missed are kidney stones because many people with cerebral palsy don't hydrate well. And, especially when their parents aren't giving them water all the time, because they're now adults and they're dehydrated and they get kidney stones. I'd probably say I have a hundred kids who have had kidney stones. People just say, oh, you have back pain. A lot of people have back pain, but this is really serious back pain.
Dr. Mary Gannotti:
Yeah.
Dr. Hank Chambers:
And, the other thing that I find that causes lack of sleep and it's a hallmark and it's a something that you need for your providers, and for the people who on this, having this, is manic depression. So bipolar disorder. In my clinic, I have over 5,000 patients now. And, in my adult clinic, over 20% of our patients have bipolar disorder. And, one of the ways that it shows up is, they go, my hasn't slept for four days. That's because he's on a manic spree. And, it may not have anything to do with pain or anything else like that. But, I agree with Mark that once you have lack of sleep, your pain is amplified. Then, you start getting on this weird cycle of taking different kind of medications and doing CBD and all kinds of stuff to try to get some sleep and it doesn't work.
Rachel Byrne:
Just so you know, as I said, we're getting so many questions in, people are saying, thank you so much, we're really feeling seen by these links between pain, sleep, anxiety and depression. Because, obviously, it's something that really people are experiencing on the daily thing. Now, you've got sort of a lot of research happening right now in relation to pain and adults with CP, can you sort of explain some of the latest research findings that you are seeing from your study? And, I know you want to get more and I know you want to get more information, but if you can share any of those preliminary results?
Dr. Mark Peterson:
Are you talking to me, Rachel?
Rachel Byrne:
Oh, Mary, sorry.
Dr. Mark Peterson:
Sorry.
Dr. Hank Chambers:
It seems like we're all [crosstalk 00:50:39] right now.
Rachel Byrne:
Which is amazing. So, I think this might be actually, I'll ask all of you. So, we've talked about research, you've-
Dr. Mary Gannotti:
Yeah, we are trying [crosstalk 00:50:51] patient reported surveys on MyCP from the CPRN research network and we only have about 300 people, but we are asking about functional decline and pain location, pain intensity, pain onset, and use of opioids from a patient perspective, which is a little bit different from what Dr. Peterson has done so magnificently with the electronic records from insurance. And, what we have found is that the onset of pain is really young. For the most part, it's not getting better. The neck and the stomach and the lower extremities are the most painful. And, we don't have a lot of information from people that are wheelchair users, but we do see higher incidents of opioid misuse than we would like for the people that we did report. And, I think that's probably in line with some of Mark's health insurance research.
Dr. Mary Gannotti:
We did see that spasticity, dystonia and pain are the major issues for functional decline. Functional decline and pain are really high in the age ranges of 38 to 50, which makes sense chronologically. So, that's what we found out so far. We would love to have a few more hundred people, mycp.org, so that we could look across people across the different GMF CS levels.
Rachel Byrne:
Yeah, absolutely. And, I think this is the really important piece of participation in research. So, this is sort of the next piece. I want to ask all of you because all of you actually at the moment have research that is happening in this space. Mark, can you give me a quick update on some of the research that you are doing in this particular space right now and what you are looking to try and find out?
Dr. Mark Peterson:
We're actively pursuing pain intervention work and hoping to try to better understand the links that are between pain and mental health disorders. That, to me, seems like one of the most important things, because if we can identify the mechanism of pain and if it's being treated inappropriately using opioids as a go-to treatment. But, certain types of pain are actually very poorly treated with opioids and can cause much worse outcomes, both in the pain experience, but also the potential mental health problems that arise with opioid addiction. If we can come up with alternative strategies, both behavioral and pharmaceutical interventions that are tailored to the specific type of pain, that would include things like sleep therapy, like exercise, like cognitive behavior therapy, those things can be extremely effective. And so, we're actively pursuing research in that space from the context of health related quality of life for folks with CP.
Rachel Byrne:
And, Dr. Kim.
Dr. Heukyung Kim:
Oh, we don't do that much research, but we are doing pain biomarkers for genes 4, 5, who can use. Some people who are are not able to express their pain. So we are doing a biomarker study. And then, the other study is, we are doing pharmacologic study for cognitive function in cerebral palsy. And, the other one is telehealth with the exercise home exercise program for each adult with cerebral palsy. That's why we are doing it at this time for the adult with the CCP.
Rachel Byrne:
Yeah. And, I think that last one's really important because we do have a lot of people who are watching who want to know what can be done, what can they do now? And, obviously, there's some things that probably sounded a little bit scary and there was one person that said, this is making me anxious. But, there are things that can happen, right, and we know that we want to make sure that really reiterate the piece around mobility and physical activity, getting a good care team around you and sort of who understands cerebral palsy. And, while some of these things may happen as we age, if they get identified, there are lots of things that can get done about them. So, just want to reiterate that to everyone that there are things that can get done to help either prevent something or if it's already happened, you actually [inaudible 00:55:27]. Mary.
Dr. Mary Gannotti:
What I usually tell adults that after they're like Mary, I hate you, you just scared the crap out of me. As I say, look, go and get some baseline of valves. You're whatever years old, go to, whatever, OPTP, Gait Lab, whatever. Get a baseline MRI, get a baseline bone scan, get a baseline, whatever, metabolic panel or aerobic test. And then, just keep a tab on it, as part of your physical and know that you just have to be a little bit more vigilant maybe about your bone than somebody else. But, some people with CP, their bones are fine. Part of it is genetics.
Dr. Mary Gannotti:
And, some people that I know in chairs, their hearts are fine, you know? So you just have to sort of be aware, just like, we have to go to the dentist and get your colonoscopy and your mammogram. Maybe there's a few more things to put on your annual evaluation for a checklist. And then, if something isn't to where you want it to be, there's exercise and there's support systems to try and address that issue.
Rachel Byrne:
Dr. Kim, Dr. Chambers, with that baseline list, because I think people are going, oh, okay, what's that list? Is it anywhere? And obviously, I know patients that are coming to your clinics, you would have that list. But, is there somewhere if someone doesn't have a CP doctor as part of their care team, is there a list somewhere that they could get, that lets them know all those things that they should be potentially screening for or what they should be doing?
Dr. Heukyung Kim:
I think, to me, all the physicians are supposed to do a review of the system. We have to ask all the questions, top to bottom; eyes, hearing, teeth, swallowing. If the physician diligently asks the questions, they'll find out. So maybe, the physician just say, oh, because I hear that even a couple of days ago, oh you have cerebral palsy, don't come back, we have nothing to do. That's what physician says. So, I think patients, ourselves, has to stand up. You ask a physician, why you didn't ask me every system. Let's go by system by system. Patients should ask the questions if the doctor doesn't do that. We have to educate people. Educate physicians or care providers.
Dr. Hank Chambers:
But, I think that's a good challenge that we will come up with a list and put it on the CP foundation website. And then, you'll be able to do that, that should be easy to do.
Dr. Mary Gannotti:
Yeah [crosstalk 00:58:21]-
Rachel Byrne:
My physicals coming up, give me my checklist.
Dr. Mary Gannotti:
There's a book chapter that we wrote, David Frumberg and I, for Freeman Miller. We wrote a book chapter on clinical care for adults with CP.
Dr. Heukyung Kim:
Oh.
Dr. Mary Gannotti:
And, Dr. Frumberg put in that chapter a list, basically, that came from the US, what you're supposed to do for everybody. But, just thinking about, like Mark said, with the accelerated aging, instead of getting your DXA at 50, maybe get it at 30. What good is that DXA if you didn't have one at 18 or 20 anyways, and you had CP? Your bone health is something that should be monitored through your life, as should your metabolic panel, as should your hormones, as should your mental health, as should your bowel and bladder in your sleep. It's all interconnected. So, it's just kind of like keeping a notebook.
Rachel Byrne:
We just got a question through, should people with cerebral palsy get things like bone scans and GI evaluations. Well, the answer to that is yes. And, when Mary just mentioned the word DXA, that is a bone scan. It is looking at your bone density and your bone health. Now, there's one sort of final piece of the puzzle. Hank, you mentioned it a little bit when it came to mental health.
Dr. Hank Chambers:
Yeah.
Rachel Byrne:
And, thinking about the different things that you're seeing. But, obviously, mental health is a really important issue and it's something that we're probably seeing increase just within community and society over the last 18 months due to things that have happened with the pandemic. But, what are some of these things that we can do around mental health right now?
Dr. Hank Chambers:
Well, first of all, to understand that it's real and that many people have problems with mental health. And, I think it's accelerated or it's worsened for people that have cerebral palsy because they already have a brain injury. So I think that's part of it. Circumstantial, just the fact that you're in a wheelchair and you've lost a lot of your support group. When you were kids, you had high school and you had friends and all of a sudden your support group's gone and you have lost. And, I've noted that with my son, he was very popular in high school and then all his friends went to college and he didn't have anybody, he had to make all new friends. And, I think that contributes to depression. But, I think getting into mental health professionals, whether it's a psychologist or a therapist or a psychiatrist can help because some of these disorders like anxiety, bipolar disorder and depression can be treated with medication and will really change your quality of life, your sleeping, everything else.
Dr. Hank Chambers:
So if you're depressed or you're anxious, all the stuff that we're saying, exercise, take care of your health, eat well, you're not going to do that. Because, people with those mental health issues don't have it. I think the mental health issue are one of the primary problems in adults with cerebral palsy. Pain is important. But, I think that may be also related to the mental health issue. So, for those people, they should go out and hopefully seek that, that would be helpful.
Dr. Heukyung Kim:
I agree with the Hank very much cause mental health, I truly believe that it should come as a number one problem to be handled. But, have to know the real situation. No psychiatrist wants to see them without cash.
Dr. Hank Chambers:
That's true.
Dr. Heukyung Kim:
I am the psychiatrist, I am the therapist because no wants to take care of them without the cash, everybody requests cash. I don't know how we're going to handle this one. Maybe CPF, we can talk about this on how we can help those people. And another one, I was thinking very deeply that, when they were young, when they become like 8, 9, 10, I already start to address this one with the parents, but they have so many surgical intervention procedures, hospital, admissions. These are all the things that are aggravating their anxiety, too. And then anxiety is related to depression. So these things has to be really studied and addressed and we have to do some politics to help them take care of this patient. Otherwise, we talk about it, but it's never going to be solved
Rachel Byrne:
And, we'll make sure that we put all these different resources and listing together. And absolutely, it is a focus of the foundation and something that we have as a priority area moving forward. And, Mark, do you want to sort of talk about a little bit about what you hope to see happening in the future when it comes to some of this research, but also the outcomes of what that would look like?
Dr. Mark Peterson:
Yeah. We can continue doing research and pointing spotlights on problems, but ultimately being able to deliver on interventions is really the most important building infrastructure and delivering on interventions, is the most important thing. And I think, if anything that good has come out of this entire pandemic, we've been able to connect with people in a much different way, in a much more seamless way than we've ever been able to before. And, mental health provisions through telemedicine is definitely possible. And, I do think that folks with cerebral palsy, especially the physical barriers, have historically limited their access points to mental health providers. So, not only do we have a problem with a dearth of providers, but we have basically barriers to access to those providers that do exist. And so, telemedicine is going to represent a really nice and seamless way to access providers for folks who have access issues to begin with.
Dr. Mark Peterson:
So, I do think I'd like to see the future of mental health provisions for cerebral palsy be wide open with floodgate through telehealth. And, I think that's something that we're just starting to scratch a surface with that and it's going to end up improving access points. Whether that improves mental health care is to be determined, but we have to start with access first and providers first and building that infrastructure and building the means to access people, is the first line, I think, and then education is also simultaneously very important.
Rachel Byrne:
Yeah, absolutely. And, as I said, watch this space. We had so many adults let us know that for research, they are putting their hand up to be part of anything to come up with some of these solutions to the problems that we've discussed today and really be part of the team. Because, I think that's one thing that I'm excited to see as well when we are trying to find solutions to some of these problems. And, we are going to do it together. And, I think having stakeholders and young adults and adults across the lifespan involved in this from the beginning, is going to be so important so that we can come up with solutions that are both scalable, but really actually effective and things that everyone can use.
Rachel Byrne:
I just want to say a big thank you obviously to the whole panel this evening. And, obviously, to everyone who attended, I apologize for the technical difficulties to begin with that. This has been an incredible conversation. And, one that I promise we will continue. I really see this as being part one of a series on adult health and all things cerebral palsy. So I just wanted to thank all the panelists for being here this evening.
Dr. Hank Chambers:
You're welcome. Thank you, Greenie.
Dr. Mark Peterson:
Thank you for inviting me.
Dr. Hank Chambers:
[crosstalk 01:05:56]. Keep going.
Dr. Mary Gannotti:
Yeah. Keep it up, Rachel. You're doing [inaudible 01:06:00].
Rachel Byrne:
And, as I said again, thank you again to our panelists. We've covered so many different topics, but we will absolutely be holding more Town Hall series on adult healthcare. And, I would also like to thank Ipsen Biopharmaceuticals who are sponsoring tonight's Town Hall, but also have sponsored all of our New Horizons Town Hall series. And, we look forward to seeing you at the next one. Thank you very much, everybody.
Rachel Byrne:
(silence)