Rachel Byrne:
Hi everybody, my name is Rachel Byrne and I am the executive director of the Cerebral Palsy Foundation and I am thrilled to be welcoming you back to our New Horizons education town hall series. Today we're going to be talking about cerebral palsy and spasticity management, and we're going to look at providing a general overview of cerebral palsy and as it relates to spasticity. So how spasticity develops and options for management, including botulinum toxin injections, thinking about protocols after interventions, and then thinking about the related considerations and possible ways that families of children with cerebral palsy can better understand and help manage the impact of spasticity, as well as maintaining an improving function and mobility through the lifespan.
Today we have two amazing speakers, the first being Dr. Ann Tilton. Ann is the Professor of Neurology and Pediatrics and Section Chair of Child Neurology at Louisiana State Health Science Center in New Orleans. She is the director of the Rehabilitation Center at the Children's Hospital of New Orleans and the Director of Comprehensive Spasticity Program and has special interests including neuro rehabilitation, neuromuscular disorders and clinical applications, as well as research in novel uses of things like botulinum toxin and intrathecal baclofren in the care of children and young adults with abnormal tone and cerebral palsy.
Our other speaker today is Dr. Joyce Oleszek and Dr. Oleszek is a professor of physical medicine and rehabilitation at the University of Colorado School of Medicine and the co-director of the Complex Surgical Tone and Rhizotomy program at the Children's Hospital of Colorado. She has practiced at Children's Hospital Colorado for over 20 years, and her clinical interest includes cerebral palsy, tone management and neuro disorders. She is involved in the hospital center of Gait and Movement Analysis and she assists in the intraoperative monitoring for rhizotomy surgeries. Today's town hall has been made possible by the generous support of Ibsen Biopharmaceuticals, and so I'd like to have you all join me in welcoming Ann and Joyce.
Thank you both for joining us today.
Ann Tilton:
Thank you.
Joyce Oleszek:
My pleasure.
Rachel Byrne:
So when we think about why actually both of you got into the field of cerebral palsy, why did you decide to specialize in that? And Dr. Tilton, we'll go with you first.
Ann Tilton:
Oh, well thank you. I look back, it's interesting in one's career how different directions sort of happen at times and you find your ways. My first job after I finished my fellowship when I was working at Southwestern in Dallas was in an orthopedic hospital and being new, I did everything and that's where neuromuscular and clearly the spasticity because it was an emphasis of this orthopedic group. And so you got to learn it in a big hurry and I became very interested in it, but also just love the families and love the kids and at that point we didn't have as many things in our sort of toolbox. So it's been wonderful not only to get to know them and the things we did at the time, but to watch it evolve over time. And so I'm thrilled to be part of that whole community.
Rachel Byrne:
And we are very, very lucky to have you and Dr. Oleszek, what brought you here? So you've been practicing for 20 plus years. Why cerebral palsy?
Joyce Oleszek:
Well, I would echo what [inaudible 00:03:31]. When I first started my career, I did everything as well and neuromuscular disorders and cerebral palsy and spinal cord injury and torticollis and everything. And I think over time the really exciting thing to me about children with cerebral palsy is that no two of them are unique, are the same. Every child with CP is unique. And I think it just lends so many opportunities to expand what we do and try something different. And we learn so much from each family. Each family tells their own story to us and helps us become better doctors. And in turn, we then work with each other around the country. I work with Dr. Tilton on projects and I think it helps us all grow as I think a whole team.
Rachel Byrne:
No, I think you bring up a really important point. The ideas of collaboration around cerebral palsy for the individuals and doctors and physicians and therapists that work in CP around the country, it is actually a small group of us really. Most people know each other and particularly in advancing forward interventions and how we can actually create better clinical practice, it really has been a collaborative and group effort and I think it's really important that it is seen that way because as you said, if we're going to move forward with different ideas, collaboration really is the best way. So just asking now, Joyce, so when we're thinking about spasticity and we're thinking about cerebral palsy, why do we focus on spasticity? Why is that something that needs to be managed in CP?
Joyce Oleszek:
Well, I think to answer that question, I think we have to understand what is the cerebral palsy because spasticity is really a hallmark of that. And so to start, cerebral palsy is an injury to, most people say the immature brain, which just means a brain that's in the process of development. So typically in utero, there's an injury whether it be from prematurity causing a hemorrhage or an in utero stroke or a brain malformation. But when those injuries occur, typically the hallmark of cerebral palsy is the abnormality of the motor system. It's a primary motor disorder, it causes problems with movement, coordination, and involuntary movements, the most common involuntary movement is spasticity. And so that is what I think we spend so much of our time really focusing on because that spasticity can interfere with so many things with the child, primarily development when we think about its physical manifestations, but it can also impact comfort, positioning, and cause pain if we don't treat it appropriately.
Rachel Byrne:
And I think it's really important making that connection between, okay, the brain injury itself is actually leading to spasticity and it's really sort of like a secondary element of what we're thinking about with CP. So Ann, when we think about CP across the lifespan, does muscle tone, does those different things change if we're thinking about how it develops and what we see potentially in an infant in a child to them what we see in an adult?
Ann Tilton:
Yes. It's interesting because we talk about cerebral palsy as not being progressive, that it was an injury that occurred and it hasn't changed. Well the original disorder has not changed, but over time it is clear that a lot of other things do and muscle that is not used sort of in this standard or typical way has changes that occur to it. So if there is spasticity, the young child will look different than the older child because of the evolution of the tightness, maybe contracture, which is really a tendons tightness. And so it is an evolution over time and spasticity is different. And yet there's the common denominator that Joyce was just talking about and that really is a muscle stiffness that really interferes with movement. And we can talk about the other associated things that often come along with cerebral palsy, but spasticity itself limits fluid motor and motion.
And we often talk about two components of it, if you don't mind me going a little bit. One of them is some of those things that are gained, the tightness, the reflexes that go up, and then we talk about the toes go up too. And then the other half of it is the loss, which is there's underlying weakness. And then there's also the loss of selective motor control. And as you get older, you expect more selective motor control and you expect more of your coordination and posture, which is often influenced. And of course it's a degree, someone may have a very mild case that they overcome or come up with strategies and it isn't impairing them. And then another person may really have some long term difficulties, which she was talking about in an untreated state.
Rachel Byrne:
I would love to expand on a couple of those things a little bit more, one of them being around spasticity and the muscles that it actually affects. We know that that is not the same for everybody, but say for example, if you have bilateral spasticity, so thinking about your lower limbs are impacted, is it every muscle in your legs that would then have spasticity or is it just a certain few for some? What's the difference for some people?
Joyce Oleszek:
It really varies and that kind of brings back to that unique feature that I was talking about in the beginning. Every child is different and if a child has, when we classify cerebral palsy, we typically classify by the limbs involved. And so if we do have a child that has primarily the lower extremities involved, we'll say they have spasticity, a spastic diplegic type of cerebral palsy, that can look different in every child.
You see some children walk independently when they have this type of cerebral palsy. They may only have spasticity in their calf muscles causing them to be on their toes. Other children have more significant involvement in their legs and have spasticity in their adductors, which causes them to cross their legs, which you know if you have significant spasticity in your quadricep muscles, it's going to cause a stiffening and stiff knee gait. The more significant pattern of involvement in the lower limbs, the harder it is going to be able to walk independently and a child may choose to walk with an assistive device. Really depends on where the injury and how extensive the injury in the brain was to begin with.
Rachel Byrne:
And I think they're really important components to understand because they really impact the management and the interventions that might be appropriate for your child or for you. Really it's not one size fits all. Sometimes we wish it was because it would make our lives so much easier because it could be like, okay, here's the exact protocol and everyone follows it and it's exactly the same. But we know particularly with spasticity management, it is absolutely going to be different for every single child and how that impacts them throughout their life. The other question that I wanted to expand on too, and you spoke about weakness. Can you just describe the difference between what is spasticity and potentially a tight muscle and then what is weakness and maybe still a tight muscle?
Ann Tilton:
That's always sort of a dilemma and it's always a little confusing because you can have weakness in the face of a start with spasticity because the underlying strength of the muscle is lower, to the point of where sometimes we have to really be careful as we address some of the spasticity that we may relax that high tone, which someone may be using to substitute for the strength that they don't have at the time and they actually lose function. So our focus is always on what will functionally make someone better. I tend to talk to people when I've got them in clinic, say I can usually make you looser. I really need to figure out if I can make you better, to be sure with that.
So you can have high tone or low tone and still be strong or you can be spastic and do that. So it's almost as if they're again collaborating, but they're two different systems. I mentioned about the diagnosis of someone who often has cerebral palsy and it affects not only the high tone but it affects the underlying weakness. So those people often have low tone or excuse me, low strength underlying someone and that may be part of why they don't walk as well if they have diplegia, which you mentioned or they may not because they have underlying weakness and it's not so much that the tone itself is a problem but the weakness. So strength is the actual strength of the muscle, tone is that overlying, I guess I would say increase in stiffness of the muscle?
Rachel Byrne:
No, and I think it's just a really good thing to remember because sometimes interventions won't be recommended because of the underlying muscle weakness and knowing that okay, if that spasticity is taken away, the muscle itself is so weak that then things like potential standing or independent walking wouldn't actually be able to happen.
Ann Tilton:
Correct.
Rachel Byrne:
And I think it sort then comes down to these ideas about when should spasticity be evaluated? We've now spoken about it that it happens across a lifespan, it happens in everybody differently. Would if actually if your child had spasticity and what sort of evaluations should be getting done? And Joyce I'll let you answer that one.
Joyce Oleszek:
Yeah, I think whenever I see a child in clinic with cerebral palsy, my first thing is to really evaluate their development and what needs to be improved. What would we like this child to be able to do better and what is limiting this child? And in many cases it is the movement disorder, most commonly spasticity. And we really can look at how a child is developing and for instance, if I see a child who is really trying to pull to stand but yet is so high up on the toes that they just can't get the balance or they are trying to crawl and they have what's called a bunny hop cause maneuver to their crawl, they can't reciprocate their legs, I know that likely there is probably their muscles are contracting more than they are relaxing.
So that spasticity is really interfering with their function. And so that is one reason I want to try to treat spasticity, whether it be with a medication or botulinum toxin injection, certainly therapy, bracing, and really try to figure out how we... Because the goal really in all of this, and I think it's always important to figure out what that goal is, but primarily the goal is enhancing function. And so looking at what do we feel like we can help to make this child function better?
Rachel Byrne:
And are there specific measures that we would do in relation to spasticity? So we had actually a great question coming in from one of our listeners right now and it was like, "well how do you tell the difference between strength and spasticity?" And I know in the clinic for example, you would do particular assessments looking at muscle tone.
Ann Tilton:
So in terms of assessment, there are a lot of different ones. And one of the things that I've seen evolve, because I've been doing this a bit as well, is that we used to just look at technical measures. We used to just look at did that muscle get looser? That sort of thing. And now we are developing, we call them instruments, ways in order to measure whether or not their outcome was better on a functional basis. And one of the things that Joyce was alluding to is that another major part of the function is to identify the goals of the child and the family. And so there're things called a goal attainment scale so that you can say what are the main things that you want to have accomplished by this intervention? And then the therapist for instance can say that, oh boy, I don't know if anyone could get over that bar, it's too high or whatever.
And then you can measure how you did. And that's a functional individual measure because everybody's a unique person. And so if I just say walking, well that may not be one of the goals, maybe not falling is a goal or something else. Anyway, so that's one example. Then there are examples of those that look at upper extremity function, lower extremity function, speed, timing, and then actual measures of tone and strength to a certain degree, although mostly we're trying to maintain strength in the face of reducing spasticity. And maybe Joyce, you have some other thoughts on that?
Joyce Oleszek:
I think you said it all. I think the goal is really important. I think you said it well when you said the walking, that might not be the functional goal. Maybe it's just being able to be positioned appropriately in your wheelchair, not extending out of your wheelchair. We've all seen kids who just don't have the ability to sit comfortably and because of their tone and if that's the case, they can't see the world if they're scrunched down or if they're extending out, they can't use their arms as well. And so I think if their hand is fisted, it may impact function, but it's also impacting hygiene. And so I think there's so many things we have to think about and have the families help guide us and on what is really problematic in the home.
Rachel Byrne:
And I think it helps obviously when we're thinking about those different assessment tools, like the goal attainment scale and we'll link to these different pieces for families to go read more on cpresource.org. But with that, it sort of asks the next question. So if we're thinking about spasticity management and are there certain protocols? You've mentioned some different interventions that could work, whether it be injections like botulinum toxin, therapy, sometimes even surgical different interventions may indicated. But when these happen, are there different protocols that families can follow or is it really individualized? And Ann, oh no, Joyce. All right.
Ann Tilton:
Okay. Both will be happy.
Rachel Byrne:
I know.
Joyce Oleszek:
I think there's not a specific protocol, but I think most of us who treat children with cerebral palsy and spasticity have this, I like to say a pyramid approach where you're utilizing your least invasive things at the base of the pyramid. So things like stretching and therapy and splinting, AFOs, hand splints, and then going up slowly up the pyramid as the pyramid narrows, you're beginning to get the more invasive things such as medications, botulinum toxin injections, and then surgical things for pain management such as selective do autotomies and intrathecal baclofen pumps. But I think it's always important when we think about whatever it is we're doing, if we are doing a medication or injection, we're always continuing those lower tiers, we're always continuing the therapy, we're always continuing some sort of bracing, proper positioning, and so it's really again, tailored to the child.
Rachel Byrne:
Well I think it's important because there's not going to be a quick fix, right? So when we are thinking about any of those different things with spasticity, actually there's got to be other things that need to get done. And I love that you said, okay, therapy needs to continue and there could be actually very specific therapy that you would want to do say after a botulinum toxin injections or after surgery and you would want to make sure that you are doing that obviously with the advice of your physician and with the therapy team around you.
Ann Tilton:
That's true.
Rachel Byrne:
And so, just to clarify... Oh, Ann do you-
Ann Tilton:
I was just going to add one thing, another thing that I've seen sort of evolve over time is it used to be a ladder. So you always had the fundamentals of two parts. You always had the fundamentals of the therapy that Joyce mentioned. And I remember having someone go, oh thank goodness I've gotten botulinum toxin, now we don't have to exercise anymore. And it was like, no. You don't seem to understand this is a window of opportunity. You need to really, really sort take care of things.
The other is that it used to be that you did the therapy and if it didn't work, then you looked at medications and if it didn't work, then you looked at the next thing and if it didn't work then you went to the... And it's really not like that anymore. What we recognize is there're foundational things such as the therapy and then we can divide it into someone has just, we call it a focal, one specific area that's a major problem or they have multiple areas that need intervention.
And it doesn't mean that if you have one area, you don't use the other system that's going to take care of... Multi segmental we call it. So it's really not only tailored for the individual, but now we have enough in that toolbox I was talking about that we might choose a procedure or choose some of the things that we do, but someone may not be able to do their joystick. So we add on a botulinum toxin to relax that arm even more. So it's not like fail, fail, fail, fail, whatever, it's actually much more of a center wheel and spokes of different things that you use. And timing in one's life may make a difference on, for instance, when they have orthopedic surgery or when they're old enough for this or old enough for that. So it really is more variable.
Rachel Byrne:
I suppose that leads into my next question because is there a beginning and end time frame for spasticity management across the lifespan or is it something that really is ongoing? Joyce?
Joyce Oleszek:
You hit that one on the head because it is. Something might work at this age, you might not be ready for that at this age, you might be ready, it's later. And so I think we're always looking at what we can do in the beginning, but as kids grow as kids... Like Ann said in the beginning, this is non progressive so things don't necessarily get worse, but we have changing manifestations due to growth. So with growth we can get tighter and those area, those muscles that are prone to be tight can get tighter with growth. So as many of those, the lower things on the pyramid we were working on, maybe they're not working anymore and we have to go to higher up on the pyramid to maybe thinking about a surgery because...
And it's not, maybe not every child again is different and every 13 year old who's gone through puberty isn't going to necessarily need surgery. But those are when we start thinking more about some of the more invasive things is as kids get older. I wouldn't say there's ever an end. I think that our approach differs when growth isn't as prominent, that's for sure.
Rachel Byrne:
So we've just actually had a question come in from the audience and it they're asking are there ways to prevent plasticity? But I suppose before we answer that question, do muscles get spastic over time? So say for example a muscle that potentially didn't have spasticity in your infant or early years, can that get spasticity over time? What happens when we are thinking about spasticity? Does it progress not just in the individual muscle but in other muscles that are surrounding that muscle? And Ann, I'm going to put this one to you.
Ann Tilton:
Yes. Well speaking of spokes of the wheel, you started about a five mid arm conversation here. Yes, several points. A newborn infant, even if they have high risk for cerebral palsy because of the way the central nervous system works originally for about the first four or five months, they are just doing reflexes. I mean we're used to the moral reflex and all these little reflexes. And so you may not even see the beginning of spasticity until about four or five months of age because it takes a little while for the voluntary nervous system to come in because it's when you move and you want to move that a lot of this is more obvious. If it's very dramatic very early or someone keeps all those reflexive milestones longer than they should, then you also as a physician have information that there's probably going to be some movement disorders and some other things that really accompany that.
So spasticity does change to a certain degree over time. We often think of it as muscle groups and people who have an injury to their nervous system or their spinal cord tend to be tighter in their arms flexed. Interestingly, in cerebral palsy we see their legs often flexed with the tight hamstrings and gastrocs, those sort of things. Particularly if there isn't intervention, I think that someone really can develop the consequences of prolonged spasticity, which means contractures and orthopedic issues and discomfort and all the consequences of basically being much too tight, much too long. So not only does it change early on, it changes depending a bit on what interventions you do in between. And then we know about increasing discomfort as adults and onward they go. That may be a little detailed and yet not detailed in part of what you were interested in, but that's-
Rachel Byrne:
Well I'll ask this question-
Ann Tilton:
It's evolving.
Rachel Byrne:
Yeah, I'll ask this question. So say for example, you may have spasticity in your legs, is it then would it transfer to your arms or something like that as you age or is it pretty much contained to different muscle groups, and if it's in that muscle group, it's most likely going to stay in those muscle groups.
Ann Tilton:
If you want me to continue on that, what's interesting, and I think Joyce will be a very good discussant about part of this is that you can have what's called overflow. And so someone can be very tight in their legs and their arms will tighten up too, even their speech. And yet if you turn down the dial in their legs, their arms might relax and even their speech might improve. So it's complicated. But in addition, there is overflow and if you tested those muscles, it's often not just isolating one area. I mean you have other things that are underlying from the original injury or the original disorder because it isn't always an injury, it's just the way you were made sometimes. But the overflow of increased tone in one area may spill in and accentuate, I always think of it as a dimmer switch. And people with spasticity, their dimmer switch is either off or on and usually on and often other areas in the body don't regulate real well and so they often go on too where they probably don't have as much of an underlying reason for spasticity.
Rachel Byrne:
Joyce, do you have anything else to add to that piece?
Joyce Oleszek:
I like the dimmer switch.
Ann Tilton:
I use those a lot as examples.
Joyce Oleszek:
I think that's so true. Yeah, I think really just to reiterate, the area in the brain that is injured or affected during development does dictate what muscles are involved. Whether it be the arms, the legs, the legs more than the arms. However, yes, you can get posturing in your upper body when you're doing something with your lower body, even if you have pretty good function of your arms. So it really is, it's complex.
Rachel Byrne:
No, and I think this is, it's important to think about because sometimes I think we can try to oversimplify things and say, okay, a simple treatment or intervention, but the flow on effects of those interventions as you said, and if you actually look at doing interventions in the legs, that can actually then impact the arms and how you're speaking and facial pieces as well. And I think that's why the complex nature of CP, why you need to have a really good team around you.
First and foremost, what we're learning most today is making sure who is your physician team, your therapy team, and are they working together when you're thinking about all these different interventions and what's going to be the best goals for you or your child? But just to go on from this a little bit more, if we're thinking about, we've touched on it a little bit, but what about if spasticity isn't adequately managed? What are some of the red flags that I think we should all be looking out for and what can happen for some of these children and particularly into adulthood? Joyce.
Joyce Oleszek:
When spasticity isn't managed as well as it can be, children are more at risk to develop tightness in the muscle. I think that's what happens over time is spasticity causes the muscle to contract, which if untreated can cause a muscle to shorten and that looks different in different muscles, different body parts. And in the calf for example, if the calf muscle shortens, the achilles tendon shortens, you get a contracture of your heel cord, which causes your foot to be planterflexed and not be able to be dorsiflexed or be flat on the ground. Similarly, other muscles, if you have spasticity in your elbow flexor, you can slowly get tightening of this muscle over time to develop a contracture where you cannot then extend your arm fully. And all of those, we know that we need a certain arc of range of motion to move and do things with our body. So if we can prevent that muscle tightness and contracture, then hopefully we can enhance function and comfort.
Rachel Byrne:
I think it's really important. And just to clarify, when we were talking about plantarflexion, dorsiflexion, so thinking about plantarflexion is when your foot can be on the ground, dorsiflexion is when you bring your toes up. But I think those different pieces are important because if you get stuck in positions it makes it, as you said, a lot harder than to either be able to move throughout your environment, but it can also become painful. It can also become a really uncomfortable place to be able to sit, to be able to sleep and all those different things as well. And just to follow on from that a little bit, we spoke about the different, I suppose, levels of a cerebral palsy. Can you just explain a little bit, well what are the different levels of cerebral palsy? We talk about say mild cerebral palsy or severe cerebral palsy. What does that look like with spasticity?
Ann Tilton:
Again, it's sort of a functional measure I think. And there is a gross motor function GMFCS classification and it's not scale... I'm trying to think about system or something. Anyway, I always want to say the other and it describes the individual. And the nice thing about something like that, is spasticity impacts this and it sort of measures it again by function. So if someone is able to go upstairs freely or something, then they're level one. Even though they have, if I tapped on their knees, they'd have increased reflexes, they would have maybe if I rapidly move the leg, which is one of the measures of spasticity, it's directional and it's also velocity dependent. In other words, if I rapidly move the leg, it may catch and then it relaxes. So they truly have spasticity but functionally they can go on. And that goes from there from one to five.
So a two may have more problems if you will. A three may use the appliances, the little posture walkers and things that Joyce alluded to. Four maybe a wheelchair and they propel it themselves. And five is someone who is more dependent for the care. So indirectly we can talk among each other saying, well that individual has a GMFCS level of three. Oh, I know that they will probably come with an appliance maybe not in [inaudible 00:35:54], and maybe a four or two and whatever and we can sort of communicate in that way. So that's an indirect way of, and using an indirect measure to say what that spasticity is, I think people kind of measure it in their mind, oh this is really hard or not. You can also use something called a MAS score, which is a modified Ashworth.
It's not exactly spasticity, It really measures tone and as I was alluded to, moving someone through their range and do they have a catch? That's the tone component. The true spasticity measure was developed by a man in France and now been modified again. And it uses that fast and slow range called the [inaudible 00:36:45] to really see what the maximum at really nice slow movements is and then go fast and say oh there's a difference of so many degrees and maybe my intervention will make a difference in that. I'm looking in my mind's eye at someone with their knee bent and I lift their heel up and that angle and those sort of things. It also tells me is if no matter how, I'll answer the following question, but if I move very slowly and there's limitation, the leg won't go all the way up or the wrist won't come all the way out. Then my next question with the family is when they're sound asleep, are they completely relaxed?
And sometimes someone that I see this, they'll go, oh, they're a noodle and you're like really? Or, and that leads us into another aspect that's sort of like spasticity only different, or they go, no matter what I do, they can be sound asleep, I could put them under anesthesia, it doesn't matter. My elbow's not going any farther, that's the end of my limit. That's a contraction. And you can also find those when you're measuring these things. So as a tangent, I'll come back around, their functional measures, their measures of tone, and then a little bit more complicated measure of spasticity and they measure that one, twos, and threes.
Joyce Oleszek:
One.
Ann Tilton:
Yeah, one, two threes.
Rachel Byrne:
I think it's helpful though the way you just described that because then it sort of gives us a little insight into thinking, well how does spasticity then impact you when you're moving? So say for example if you're trying to bend your leg with spasticity and it's an active movement, it's most likely going to stop before it can go through that full range. Even though if you are moving it, if someone else is moving it for you, you can go through that full range. So it makes movement patterns be different and it can make the ability to go through full movements very, very difficult. So question for you now, Joyce. So we've spoken about different interventions and one of them we've sort of talked fair bit about today is botulinum toxin injections, but we haven't actually defined what botulinum toxin injections are. Can you just explain a little bit more about what this is?
Joyce Oleszek:
Sure. So botulinum toxin is a neurotoxin and it affects the way the skeletal muscle contracts. The history of botulinum toxin is actually really interesting because it was [inaudible 00:39:17] botulinum toxin was for eye muscles, for strabismus, 1977, but it wasn't FDA approved by the US. FDA approval came for that indication, for strabismus, in 1989. Since then all sorts of indications have arisen and including pediatric and adult spasticity.
We have to understand the way the muscle contracts to begin with. So you have a motor nerve terminal that releases a substance called acetylcholine and that binds to receptors in a muscle fiber and the muscle contracts. When you inject botulinum toxin, it actually blocks the release of that acetylcholine from the nerve terminal and thereby affects the muscle contraction. But what's good and bad is that over the three to four months, that neuromuscular junction reestablishes its original function and so the results are reversible. So the spasticity you did achieve wears off. And a lot of times that's when we say, oh we really love those results. But I think in some cases it's good to be able to wear, have that wear off and be able to really focus more on strengthening and therapy.
Rachel Byrne:
And I suppose this is a question that's just come in. So when we talk about botulinum toxin, is that the same drug as when we think about any wrinkle injectables, is it the same or is it different? When we're thinking about for children and adults on spasticity>
Joyce Oleszek:
It actually is. Botulinum toxins have different indications and they have a few different serotypes and formulations. But the botulinum toxin itself is the same. The vial will say botulinum toxin cosmetic, but it is the same type of botulinum toxin.
Rachel Byrne:
Okay. No, it's just out of interest, right? Because I think it's that evolution of where it's now getting used and what most people probably know it's commonly used for. So when we're thinking about obviously interventions and you mentioned a little bit about strength training choice, do we do these things before and after, say something like botulinum toxin or is it something that you would really want to do after once it's effects have taken place?
Joyce Oleszek:
Well, I think strength training is important regardless, or some type of therapy is important regardless. I think our goals may change and our type of strength training may change after a botulinum toxin injection, especially depending on where you inject. If botulinum toxin is injected say in the gastroc muscle, I'm going to really want to focus on strengthening that opposing muscle, the anterior tibialis muscle. But it's always important to strengthen the hip muscles no matter what. And so I think our focus changes, but I think always trying to work on strength as much as possible and motor control maybe is just as important if not more important.
Rachel Byrne:
We'll get to motor control in a second. And I've got another question that's come in. Is it possible to strengthen a muscle that has had botulinum toxin?
Ann Tilton:
To me? Yes. I use strengthening just as she said on both sides of this. As we relax muscle, as I was talking about before, there's probably underlying weakness. And so strengthening up and to that point is also very important. But afterwards, not only is strengthening been shown to really help, but it may stretch the muscle some, and we've been able to see that with botulinum toxin the muscle lengthens and your strength correlates with the length as well.
So I think that it's very important. The only time that I don't do botulinum toxin or I give them a separate washout right now is in research protocols, so that they cannot have something else that interferes so that they can actually measure that it makes a difference and it's been pretty consistent now. Totally blind, the whole bit, but those people who have been into that feel stronger and do better because of strengthening. It used to be thought, oh you can't strengthen, you shouldn't exercise a spastic muscle, it'll only make it worse. Not what we're finding. And there's been some really nice research on that. And since it is small community, we know a lot of the people doing this and they're very, very good scientists.
Rachel Byrne:
Yeah, no and we've actually got a couple of town halls that we've done on this and a few different things, particularly around strength training and what that looks like. And we know there's even some studies available right now thinking about power training and actually one of them is out of New Orleans. So-
Ann Tilton:
Yes, the guys like it too because they think free sports, they're all about.
Rachel Byrne:
I think that is just something I want to sort of talk about is you talked about the types of strength training and I just brought up the word power. What are the different types of strength training that we're talking about here?
Joyce Oleszek:
Well, at Children's Hospital Colorado, and I know around the country, the therapists are really... I know therapy has evolved too. And I think we have evolved more from doing once a week therapy every week forever and ever to let's do an intensive bout of therapy, maybe three or four weeks with some really meaningful goals, and we call that intensive model. And families have really loved that. And frankly, so if the kids, because I think there's [inaudible 00:45:29] a weekly therapy program that doesn't seem to have an end in sight. So the intensive models, there are definitely power intensives, strength intensives, there are constraint intensives. So we think of more of the power and strength intensives for the lower extremities. And we have kids who are pushing sleds in the gym and talking about how many reps of squats they're doing and doing arm bikes and really doing some... Really feeling like they're working out and really having manageable, meaningful goals that they can achieve.
But then there's also the constraint intensives. So constraint induced movement therapy for the upper extremity for the kids to practice having their, especially in kids who have hemiplegia, have one arm restrained. So it forces the use of their involved arm. And that can be great because it's again, a very... You have a cast on for many weeks up to 12 weeks and really have a forced use of that... A cast on the good side and the arm that's more involved and that can be great, just that intensive therapy to really maximize our improvements.
Rachel Byrne:
Yeah, I think it's so interesting actually how all these different things are evolving and how we are learning more and a lot of that has come through research. So to finish us off, what is some of the best advice... I'm going to ask this question to both of you. So what is the best advice you would give a family about helping to manage their child's spasticity and to maintain, improve function and mobility? Joyce, do you want to go first?
Joyce Oleszek:
Well I have a motto, it takes a village and we all know that in our personal lives, but it's true with these families. And I have a diagram that I have in some of my talks and patient is in the center and then I'll round it... We're back to our wheel. Around are all the people that this child needs. Every therapist, the assistive technology folks, all of the specialists, the rehab, neurology, orthopedics, ophthalmology. I could keep naming them.
And it goes around and I think we can help them develop their village within our institution. And I think social media has been such a big help too, I have to say. Because so many families come to me and say, "Oh, what do you think about this piece of equipment?" And I'll often say, "I've never heard about that, tell me more about that," and I'll pass it on to the next family. So just the simple things as a pair of shoes that fit over an AFO and we all work together and I think that's what I would tell families is to find your village and we're going to be with you in that village.
Rachel Byrne:
I think that's amazing advice. And Ann, what about your last piece?
Ann Tilton:
I always agree with Joyce anyway, but I certainly agree with this comment. I think it is a multimodal and I think we have to not only utilize those people around, because sometimes you don't have the whole team, that we have the parents as advocates and then eventually have the kids as them being their own advocates too and becoming as independent as they can possibly be just for their lifelong independence. So helping the parents feel like they have some control over it and help them become advocates and then they can become peer advocates for others.
And that's building that whole foundation again, we all have these foundations that allows everyone to benefit. But it does, it takes the school to understand, it takes the therapist, it takes the... Be sure the vision's okay, what's the hearing all about? Has there been imaging? Is there another reason for this? Is there... There's a lot to be done and I think it's probably pretty overwhelming as a parent and it would be, it's really nice if they feel like they have some touchstones along the way to do that. And I think we as working in pediatrics kind of see the medical home and we try to help with that.
Rachel Byrne:
I think it's so important, right? Finding your local institution, thinking about who are going to be your champions that you can work with across with your child now, but then thinking across the lifespan as well. And we know there are some amazing CP programs across the country and really making sure if you can, linking into those I think is really wonderful advice for our families. And so I just want to thank both of you obviously for taking the time today. And yeah, we've been thrilled to have you and if anyone does have any other questions, please put them in the chat box and we'll try to make sure we can get those answers for you at a later date. Thanks so much, Joyce. Thanks Ann.
Ann Tilton:
And thanks to people who brought us here. Thank you so much.
Joyce Oleszek:
Thank you so much to all of our families out there.
Rachel Byrne:
In finishing today, I would just like to say another big thank you to our panelists as well as all of you in our community and our listeners who sent in your questions. And please if there are any other questions that you would like answered, put them in the chat box and we will really try to make sure we can give you the resources that you need. I would also like to thank Ipsen Biopharmaceuticals for their continued support of our New Horizon town hall series.