Adult Cerebral Palsy Care

Podcast Transcript

Introduction: Neuro Pathways, a Cleveland Clinic podcast exploring the latest research discoveries and clinical advances in the fields of neurology, neurosurgery, neuro rehab, and psychiatry.

Glen Stevens, DO, PhD:

Medical and societal advancements have significantly increased the life expectancy of those with cerebral palsy. However, there are a large number of adults with cerebral palsy who do not have a access to the comprehensive medical care they need. In today's episode, we're discussing how to best serve the often underserved adult cerebral palsy community. I'm your host, Glen Stevens, neurologist/neuro-oncologist in Cleveland Clinic's Neurological Institute. And joining me for today's conversation is Dr. Francois Bethoux. Dr. Bethoux is a physiatrist and Chairman of the Department of Physical Medicine and Rehabilitation in Cleveland Clinic's Neurological Institute. Francois, welcome to Neuro Pathways.

Francois Bethoux, MD:

Thank you, Glen. I'm very thankful for your invitation to discuss this topic. I always feel like we need to talk more about it and about this group of people, which is very significant in our community, yet certainly underserved and also not well known actually by all of the citizens of this country.

Glen Stevens, DO, PhD:

So to get things started, why don't you tell us briefly what cerebral palsy is? So we're all on the same footing. And what types of problems we see with patients with cerebral palsy?

Francois Bethoux, MD:

So you're already asking me a trick question because there is actually not a straightforward definition of cerebral palsy. Everybody acknowledges is it's a group of disorders, but they have something in common, is that they're caused by either an abnormality of the brain development. So the brain doesn't develop as it should, or some damage occurring to the brain, either during in utero or around the time of birth. It may actually not be diagnosed right away. It often is diagnosed a little later when children fail to meet some important milestones, such as sitting up, walking, talking. And so, the hallmark of cerebral palsy or CP is the difficulty moving. And palsy actually means weakness or difficulty using the muscles, so it makes sense. And so, we may have images of children, adolescents and adults having difficulty walking, difficulty keeping their balance, difficulty using their arms and their hands. There is also a potential component of intellectual disability as well in cerebral palsy.

Glen Stevens, DO, PhD:

So Francois, we mentioned in the opening that we're probably doing a better job in the pediatric population with the children with cerebral palsy, because that's where we're identifying it. But we have a lot of work to do at the same level of care in the adult population with cerebral palsy. In your opinion, what has worked well in the pediatric population?

Francois Bethoux, MD:

So what I think worked well for the pediatric population is the acknowledgement that a significant number of babies were born with damage to their brain or abnormal brain development, leading to cerebral palsy. Therefore, there's been a very early stepping up in multidisciplinary care to take these babies, toddlers and children, giving them the best chance of avoiding complications, of having the best

function and quality of life possible. It's estimated there's about 10,000 children born with cerebral palsy every year in the US. And so that leads to an estimated 700,000 people living with cerebral palsy, who may be children or adults. Significant number, and really there's basically two aspects to the pediatric care. One is, taking care of the problems that are presenting themselves. But also the second one is giving these children the best chance to live a long life, to live a productive life, but also to have the greatest quality of life possible. And there seems to be a disconnect when they reach the adult age and where they transition to the adult healthcare.

Glen Stevens, DO, PhD:

So I'm going to throw you just a little curve box. I'm just kind of curious about the answer to this. Is the incidence of cerebral palsy decreasing, staying the same or improving? I would like to believe that we should see less of it as medical care gets better, but maybe it's not. Do you know?

Francois Bethoux, MD:

I don't have the numbers precisely in my brain, but what I remember from previous readings is that while there has been an overall decrease due to progress, in particularly the quality of delivery and care around delivery. There's also, what we now call, social determinants of health that have not been resolved in many developed countries including ours. And that lead to an increased incidence of cerebral palsy. So it's a mixed message there. We certainly have made progress, but some of the factors are not necessarily directly medical and society as a whole has to understand that and step up to improve these numbers even more.

Glen Stevens, DO, PhD:

So as a neuro-oncologist, I look after patients of neurofibromatosis and again, a disorder that's very commonly diagnosed when individuals are children and then get transitioned to adults. And one of the things that we see at least in the NF population, is that the patients don't want to leave their pediatrician. The patients are 40 years old and they're still being followed by the pediatrician because they followed them for years and they're comfortable with it. But it's really getting outside the realm of what's going on with the pediatricians. What about on the adult side of things? Do you see that as well, that the pediatricians hold onto their patients or the patients don't want to leave? Or is it difficult to transition the patients?

Francois Bethoux, MD:

Yes, it is actually. It is. So what I've seen, again, that speaks to the fact that the pediatric world and particularly the pediatric rehabilitation world is very nurturing for these patients and their families. We should never forget that the parents of these children are often very closely involved in their management, in their day to day life. And so they're so nurtured by a multidisciplinary team that, who would want to leave, right? And so, now that the Cleveland Clinic has engaged in a more active transition process, that starts around age 12, 14, and is supposed to end around age 22.

First, you notice that we are not in a hurry to transition people when they turn 18, we give them time to prepare for that transition and then to have a successful transition to adult care. But we've seen some families, some patients just refuse. Saying, they're not ready and it's scarier there. Or maybe they've tried to contact an adult provider and things didn't work out the way they wanted. Sometimes they couldn't get scheduled with the right providers. Sometimes they came and were not impressed with the understanding that the provider had of their condition and their needs. And they may return or ask to return to the pediatric world.

Glen Stevens, DO, PhD:

Yeah, I'm sure it's difficult in a large academic center. The peds people are in close proximity to the adult people or at least fairly close so the transition isn't so bad, but in private practice, people could be very disparate practices and it may be difficult to transition. Do you have care coordinators here that help with the transition or is it really physician to physician based?

Francois Bethoux, MD:

So it is much better when we don't make it physician to physician based. It's a little bit of a joke because I've actually for years, taken direct messages from some of my colleagues saying, Hey, I have this 25 year old, very nice young man or lady, and could you help with a transition? And I have, but when we want to do it on a large scale, then having care coordinators on board is absolutely essential. So we have a pediatric and an adult care coordinator, they communicate with each other. We keep a running list and we've reached out outside of our immediate circle of our health system to other pediatric CP providers. And they've expressed great interest in also kind of having us as their transition facilitators.

Glen Stevens, DO, PhD:

And I'm sure you have a team that takes care of these patients. Why don't you tell us who's on the team and what the roles of the individuals are?

Francois Bethoux, MD:

Certainly. Yeah. I mean, we're trying to make it as similar as we can through the pediatric experience again. It's very important to understand that people with cerebral palsy have a wide range of limitations and needs. You have people with cerebral palsy who have yes, some difficulty walking, but they drive, work, they're married, they have kids, they live what we would call a normal life and very fulfilling life. We have others who are in a wheelchair and will remain in a wheelchair, may have some, what we call, comorbidities. They may have kidney problems, heart problems, and require specialized care for this. They often have orthopedic problems. You can imagine that if one has kind of a abnormal gait, right, and puts a lot of stress on their joints and their spine very early in life, they may have pain, they may have early arthritis actually.

And so we try to have, of course, a lot of rehab resources, physical therapists, occupational therapists, speech therapists, nursing care, social workers, very important. Imagine the transition, maybe from living with your parents, to living without the parents, but having challenges finding an accessible lodging. Fighting work with adaptations, to be able to work despite some limitations and making sure you have the right healthcare coverage and so on and so forth. And we have psychologists, neuropsychologists, and contacts within subspecialties, such as orthopedics, such as dental care. Some people with CP require full anesthesia to do their dental care. It's not easy to find a dentist who will have the facilities, the team and the willingness to do this as well. So we try to reproduce that.

And I often crack jokes that in the pediatric world, it's very common to have an orthopedic specialist who will take care of your spine, hips, knees and feet. And then you transition to adult care, and it seems like you need to have one orthopedics for your spine and one for your hip and one for your knee and one for your foot. So we try to recreate, at least for routine management, the one stop shopping kind of experience, using more specialized practitioners when there's a need maybe for surgery or a more difficult case.

Glen Stevens, DO, PhD:

So outside of surgery, what kind of medicines are we using for these patients?

Francois Bethoux, MD:

Well, the medicines used, we try to keep them down to a minimum because we don't want poly medication that can lead to other problems. But treatments for spasticity are very common in individuals with cerebral palsy, because they're spasticity may cause them discomfort and interfere with the way they want to function. There may be treatments for pain and particularly for either joint pain or back pain, but also nerve pain that may be caused by the brain damage. And so there are medications specific for what we call neuropathic pain from the brain damage. There may be medications for bladder function, medications for bowel function as well, medications for depression or for anxiety. So it's the same types of medications that we would use for other disorders. I'm not aware of any medication that's specific for cerebral palsy, but we borrow from other medications used for other neurological conditions to address the needs of those with cerebral palsy.

Glen Stevens, DO, PhD:

I remember when I was training in practice, they used to place baclofen pumps in patients that had very bad spasticity. But my recollection, and maybe I'm wrong, at the time, was that these were really patients that were bedbound and they were trying to decrease the spasticity, the contracture so that patients would have less pain. But I think that this has been nuanced now and patients can now have a baclofen pump and still ambulate and function. So it sounds like maybe you guys have come a long ways in how you titrate these types of things. Can you speak on that at all?

Francois Bethoux, MD:

The baclofen pump is a wonderful treatment that is extremely potent to decrease spasticity, particularly in the legs. And it's true that both on the pediatric and adult side, we've evolved to implant these pumps in individuals who are able to walk, because we know now not to be afraid of making them lose the ability to walk because we thought we would cause too much weakness, too much relaxation. And if the legs get kind of like wet noodles, right, well then it's difficult to walk on them. But we've realized that if we're careful in selecting the good candidates, we can do that. And so, part of the transition is to help former children, who are now young adults, who've had a baclofen pump for many years, transition to adult care. But in some cases actually, we've had adults with cerebral palsy get their first baclofen pump as adults because their specificity had evolved to be bothersome at that particular age.

Glen Stevens, DO, PhD:

And why don't you comment briefly about Botox use in individuals with CP?

Francois Bethoux, MD:

Botulinum toxin therapy is also widely used in CP. One of the nice aspects of this therapy is that it can be customized because we inject specific muscles and so we can really customize the number of muscles that are injected to the person's needs, as opposed to, say, the baclofen pump that relaxes every muscle from the waist down. And so, it is very much customized, can address the upper extremities as well. It doesn't require surgery, requires injections, which, nobody looks forward to getting injections. And in the pediatric world there often are done under conscious sedation or applying some local anesthetic over the skin. And we try to transition with, again, still providing the same level of comfort if we can. And again, sometimes we start the treatment as adults. Again, this is very specific for spasticity, but spasticity affects many people with cerebral palsy.

Glen Stevens, DO, PhD:

So not every caregivers are near a large academic center, a lot of people will be in very small related areas. Are there good places for providers and patients alike to find resources?

Francois Bethoux, MD:

There are wonderful community resources. In our area, the most well known is United Cerebral Palsy. And they help a lot of children, families and adults with cerebral palsy, find the right providers. They also provide some services, particularly rehabilitation services, but they also serve to facilitate their insertion into the community, their remaining in the community. That's one example. I like to say that any provider can take care of someone with cerebral palsy. There's no special degree to have. Actually, or right now, there's not really special training that is designed specifically for cerebral palsy. If there's good listening skills, a desire to help and access to either specialized providers or good literature when needed, are plenty to address this.

And what we're trying to promote is a model where there may be a cerebral palsy medical home, if you wish, which could be in a big academic center, realizing it's not close to everyone. But then we know that there are some physicians, some advanced practice providers who have some experience and a keen interest in helping people with cerebral palsy, then we can connect with them and direct patients and families towards them, for example. The same goes with surgeons and et cetera. So that's the way we realize that the services we may provide as a home for cerebral palsy are not the end of it. It may actually just be the beginning. We may see people once and then they will be referred to a provider or their existing provider, while be provided with some resources. And then we can continue in the promotion of telehealth during the pandemic. If there's any sort of aligning to this pandemic, it's the fact that we've all gotten comfortable to providing telehealth services. Then we can stay in touch with these individuals remotely and they can get their care in person with their own providers in their community.

Glen Stevens, DO, PhD:

Yeah. I think that's an excellent point in probably telehealth for certain types of disorders where there may not be specialists in the area, allows the patient to touch base with you so that then you can communicate with them as to what you feel or who you feel they should see. And then they can look for that person in their community. It always just reminds me that, once again, that we just need to listen to our patients, right? What is the patient telling us? What do they want, not what we think they want.

Francois Bethoux, MD:

Exactly.

Glen Stevens, DO, PhD:

But what is it that they really want. What about research in the area for spasticity in general or cerebral palsy? Anything going on?

Francois Bethoux, MD:

There is actually, well, there's two ways to see it, not enough research. We often bemoan in many fields, especially in certain disorders that there is not enough research. But actually, when I did my own search on Dr. Google, I was pleased to find more research going on than I thought. First, there is research about the needs of those with cerebral palsy, particularly adults with cerebral palsy. There's a fairly large literature on children with CP, but a smaller literature on adults. And we find themes that, to me, evoke missed opportunities. One, the good news is life expectancy for those with cerebral palsy has increased, but generally, overall remains shorter than for those without cerebral palsy. We also find that what we call again, comorbidities, right, all these conditions that anybody can acquire as an adult, high blood pressure, obesity, heart disease, add to the risk of dying prematurely, but also decrease the quality of life with those with cerebral palsy. And not surprisingly, we also find again, health inequities with minorities.

So if somebody, say, is African American or with cerebral palsy, they will have a higher risk of mortality and a higher chance of having comorbidities. And unfortunately, some other research shows that these health needs are not really addressed in a preventative or curative manner. And so, some of the research is about demonstrating the needs so that we can get the right services, that's important. And then a lot more research to try and promote more discoveries, so there's advances in genetics. So now this group of disorders that I was talking about, sometimes can be actually identified as a genetic disorder. It's very important for knowledge first, but also some people with cerebral palsy will have a family. They need to know for their offsprings, if there is a risk of actually having some issues over time, for example.

There's research on stem cells, and stem cells make everybody involved with neurological disorders, dream, right, of fixing the nervous system, repairing the damage even years after it occurred. So the good news is there's more and more research and some promising results, particularly in animal models. We just need to be very patient because I don't think we've been able to harness in stem cells to the point that they can become routine care and we can promise that we can restore damage and restore function as well.

There's also research on the outcomes of spasticity management in cerebral palsy, to find who are the best candidates. What really are the outcomes? What we really provide to people who have cerebral palsy? And there's some more of the beaten path interventions that can be very low cost and very available in the community. I found a research study on the effects of dance on people with cerebral party. I'm a healthcare provider and we all know that there's a cost to being in healthcare and more and more a cost to patients, out of pocket costs. But there are community resources to help restore and maintain health that can be very engaging and also very easy to access and can be done in conjunction with the healthcare provider to make sure that everything goes safely.

Glen Stevens, DO, PhD:

I think that we're all guilty to some extent of microaggressions when we look at certain populations. I suspect that there's a number of patients that when they look at somebody with a cerebral palsy, they see the motor related problem, but they assume there's also a cognitive related problem associated with it. But it doesn't have to be. What's the instance of cognitive difficulties in patients with cerebral palsy?

Francois Bethoux, MD:

You're right that there are many assumptions. And particularly, if we see someone with a severe physical disability, who may not be able to speak even, and may have difficulty controlling their limbs or in a wheelchair, we may assume that their intellectual performance is very limited. And so in these cases, it's always good to ask. So the numbers vary because the degree of intellectual disability may vary greatly. And often actually, it's been assessed during childhood and we have a very good idea when people become adults of what it is. And so that allows opportunities such as special education, such as access to workshops for people with limited cognitive and physical abilities.

But we don't want it to be a stigma, right? That we stigmatize and we say, oh, there are special services for these people. And they're not like us, and I'm not going to be able to communicate with them. And low and behold, the person in the wheelchair in front of them may have a college degree and may actually have a high level IT job that they're doing. Maybe a researcher, maybe somebody who's very active in the community. And we will not realize that unless we give it an opportunity, unless we approach the person, ask some questions and listen carefully to the answers.

Glen Stevens, DO, PhD:

Well, Francois, I've really enjoyed our talk today. Any parting tips for anybody out there or areas that we've not covered, that you feel would be important?

Francois Bethoux, MD:

I'd say that do not underestimate all the needs that people with cerebral palsy have, but let's not underestimate all the opportunities we have that are not costly, not high tech, but may really change their lives. And it always pays off to keep an open mind and be attentive to someone else's needs, but also expectations from us and from life.

Glen Stevens, DO, PhD:

Excellent. Well, I think keeping an open mind is what we all need to do, and I really appreciate your sharing your insights with myself and our group today. And I wish you well in your practice.

Francois Bethoux, MD:

Thank you very much. I wish you well.

Conclusion: This concludes this episode of Neuro Pathways. You can find additional podcast episodes on our website, clevelandclinic.org/neuropodcast, or subscribe to the podcast on iTunes, Google Play, Spotify, or wherever you get your podcasts. And don't forget, you can access real-time updates from experts in Cleveland Clinic's Neurological Institute on our Consult QD website. That's consultqd.clevelandclinic.org/neuro, or follow us on Twitter @CleClinicMD, all one word. And thank you for listening.