Welcome to our presentation today regarding the rehabilitation medicine interventions offered for childhood cancer during acute care and also into survivorship. I would like to introduce my colleagues, Dr. Christian Niedzwiecki from Texas Children's Hospital and Baylor College of Medicine, and Dr. Matthew Haas as a pediatric rehabilitation medicine fellow at Cincinnati Children's Hospital, who will join me today in presenting this topic to you. The care for children diagnosed with and surviving from a cancer diagnosis benefits greatly from an interdisciplinary team approach across the continuum of care. While advancements in tumor diagnostics and treatment interventions have led to increased survivorship, medical and functional late effects due to the cancer itself or secondary to the interventions utilized to treat, manage, or prevent progression have increased and include conditions that span physical, psychological, and social function. As such, there is an increased need for expertise in pediatric rehabilitation medicine in the care for these children, adolescents, and young adults, both during and following cancer treatments in order to minimize morbidity and optimize quality of life. In this talk today, our goal is to provide you with an understanding and appreciation for the role of the pediatric physiatrist in the care of children diagnosed with and undergoing treatment for cancer, as well as for the management of the growing number of survivors from this population. Because of the impressive survivorship as a whole, this talk will also be beneficial to our colleagues who manage young adults, as most of these issues will have good carryover in that age range as well. For this talk, we have deliberately chosen central nervous system tumors as our primary tumor of which to provide examples throughout our discussion, as this is the most common solid tumor occurring in the pediatric population. We will provide insight today about the specifics about epidemiology of occurrence as well as survivorship in the pediatric cancer population, and then have some discussion about the basic principles of cancer rehabilitation and how they apply to the pediatric population as a whole. When we discuss important features of the rehabilitation and management of CNS tumors, we're going to talk a little bit about survivorship issues and late effects within this population, and lastly, provide some helpful insight into building a presence within a cancer treatment and survivorship program within the field of pediatric physiatry. Gaining an understanding about childhood cancer epidemiology is really essential for the pediatric rehabilitation physician, both from the perspective of understanding the incidence rates of the types of cancers that we have expertise for and to offer, but also to guide us in the timeliness of our interventions and recommendations. While cancers diagnosed among those under the age of 19 represent less than 1% of all cancers diagnosed in the United States, the high proportion of survivors among this population represents individuals at high risk of experiencing serious, disabling, and life-threatening acute, chronic, and late adverse side effects of cancer and its therapies. The National Cancer Institute Surveillance Epidemiology and End Results, or SEER, program represents the primary source of population-based data on cancer incidents in the United States. The SEER program typically reports cancer incidents according to age-specific groupings consisting of five-year intervals. The annual incidence of cancer in children less than 19 years of age is currently 176 cases per million population, which translates to an estimated 13,000 newly diagnosed cancers per year. Over the past four decades, there has been a modest yet continuous increase in the incidence of childhood cancers in the United States. Reasons posited for this observed increase have included improvements in the diagnoses and cancer registration, the ability to diagnose some tumors at an earlier age, and changes in possible underlying environmental risk factors. Survival rates for children diagnosed with cancer continue to improve. The estimated proportion surviving five years from diagnosis increased from 58.1% to 83.8% for those diagnosed over the last five decades. Children with cancers in childhood necessitate increased involvement of the pediatric physiatrist and rehabilitation team, and the annual incidence of these cancer types are demonstrated in the figure that I've shown. In children aged 0 to 14 years, noted in blue, acute lymphoblastic leukemia, or ALL, is the most frequently occurring cancer type. And central nervous system cancers are the most common solid tumors in this age group. In adolescents aged 15 to 19 years of age, noted in gold on this slide, the most frequently diagnosed cancer is Hodgkin lymphoma. However, CNS tumors remain a highly occurring tumor type in this age group as well. In addition, malignant bone tumors, including osteosarcoma and Ewing sarcoma, amongst others, occur at a higher frequency in the older adolescents than in the younger children. Currently, the overall five-year survival rate for children and adolescents with cancer diagnoses now exceeds 83%, as demonstrated in the first group on this slide. Although more than 83% of children diagnosed with cancer now survive for more than five years after diagnosis, survival rates vary greatly depending on the cancer type and stage. As you can see in the figure, the prognosis is particularly good for children less than 15 years of age with a diagnosis of acute lymphoblastic leukemia, or ALL, with a 91% five-year survival. CNS tumors overall demonstrate a five-year survival rate of greater than 73%. Adolescent populations have less optimistic five-year survival rates, including Ewing sarcoma in the 15- to 19-year age range with a 67% five-year survival. The success reflected in this growing population of survivors of childhood cancers is offset, however, by the adverse health outcomes that they experienced related to cancer and its treatment, which we're going to share in today's talk. With this clear evidence of increasing incidence and high overall survival for a variety of oncologic diagnoses, it's reasonable to expect that pediatric rehabilitation medicine providers will have increasing involvement in their care. As such, it is important for these providers to have an understanding of cancer rehabilitation principles with which to approach these complex patients. An initial approach within cancer rehabilitation was put forth by J.H. Dietz in 1996 and is now often referred to as Dietz classification. Under this model, the rehabilitation interventions would fall into four categories, ranging from preventative, which focuses on early intervention and education to reduce anticipated disability, restorative, which aims to assist return to premorbid function without substantial residual disability, supportive, including care for those who have sustained permanent impairments and may not return to their premorbid or baseline function, as well as palliative, which emphasizes patient comfort, caregiver education, and equipment recommendation. Although the Dietz model and the interventions associated with each class can and does or can and do still apply to cancer rehabilitation efforts, many patients fall into multiple classes at the same time or transition between classes as there is disease progression. As such, an updated approach that focuses on cancer stage can be helpful for understanding and demonstrating the important role of cancer rehabilitation across all stages of oncologic care. Early on after initial diagnosis, it is important to understand the overall treatment plan, which may include additional conversations and relationship building with medical, radiation, and surgical oncology colleagues to create a well-informed plan to address and anticipate initial and acute functional impairments. This treatment plan includes types and frequency of therapy services, as well as appropriate interventions, including pretreatment, rehabilitation, strengthening, ADL training, and evaluation for adaptive equipment, as well as orthotics. A common example of acute impairments related to initial treatment would be postoperative cerebellar mutism syndrome, which can have a variety of other names in the literature, as well as clinical practice, including pediatric postoperative posterior fossa syndrome, the hallmark of which is diminished speech, up to and including mutism, emotional dysregulation, as well as ataxia and oromotor dysfunction. These symptoms typically arise within two days after surgical resection of a posterior fossa tumor, with midline tumors having particularly high symptom frequency. Our understanding of the pathophysiology includes interruption of the dentate nuclei, as well as efferent projections, which causes cerebrocerebellar diascesis, and all of the typical findings. Early identification is important to assess for potential interventions, including pharmacotherapy, specifically with bromocriptine. An important aspect of the posterior fossa syndrome, an umbrella term that includes cerebellar mutism, as you can see in the core of this diagram, is that there are varying levels of impairment with neurocognitive, especially behavioral, neurologic, especially motoric, as well as linguistic function impairments, and it's important to recognize this. Particularly within posterior fossa syndrome recognition is understanding the neurocognitive impact for patients with posterior fossa syndrome. And this 2010 study by Wells et al. included a retrospective review of 28 children with medulloblastoma who were one year out from treatment, and included neurocognitive data for 12 patients, approximately half of whom had been diagnosed with cerebellar mutism, which was the term that was used at the time, and is, again, an entity within posterior fossa syndrome. Based on this neurocognitive data, the mean IQ of those patients with cerebellar mutism was 16 points lower compared to those without cerebellar mutism syndrome, with a trend toward significance and a p-value of 0.07. Additionally, they found a full 60% of patients with cerebellar mutism syndrome were receiving special educational services compared to 29% of those without posterior fossa syndrome, or cerebellar mutism syndrome. Another example of early treatment effects includes chemotherapy-induced peripheral neuropathy. Included are two examples that highlight potential interventions. Vincristine, as we know, is an alkylating agent that causes a length-dependent sensory motor polyneuropathy. In children, motoric impairment and pain complaints predominate. An assessment of ambulation, as well as fine motor tasks, is an important aspect of early recognition of vincristine-induced peripheral neuropathy, which is dose-dependent and is possible to halt symptoms with disintunation or dose reduction. An important aspect of care for these patients would include stretching recommendations as well as evaluations for necessary orthotics. In contrast, cisplatin primary affects the dorsal root ganglion, resulting in primary sensory neuropathy or ganglionopathy, which has less motoric impairment and a non-length-dependent distribution. Primary assessment for patients receiving cisplatin would include impaired proprioception, which coupled with the ototoxic-related vestibulopathy found with cisplatin can contribute to significant balance impairments as well as ataxia that may be amenable to more proprioceptive feedback through gait training as well as brace use to provide proprioceptive input to a limb. Patients will hopefully have a positive response to the initial oncologic treatment, after which comes surveillance for a routine assessment to determine whether a change in treatment is needed. This is an appropriate time to shift the focus to educational and vocational assessments and interventions. Additionally, physiatric surveillance would include intermittent functional assessments, including gait, that may cue their oncologic care teams to early recurrence. Monitoring and intervening for longer-term cancer treatment effects, including delayed radiation therapy, fibrosis, and lymphedema management, will also have a larger role in this stage. Additionally, counseling patients and families on appropriate maintenance exercises to maintain their overall endurance and functional capabilities will be important at this stage. An example of a diagnosis with increased surveillance interventions includes low-grade gliomas. This graph, which includes data from over 4,000 patients with pediatric low-grade glioma retrieved from the SEER database from 1972 to 2008, shows that there's a high overall rate of survival up into 20 and 30 years from diagnosis. Additionally, you may notice that these survival curves separate out based on a history of radiation, which was found to be the greatest risk factor for death with multivariate analysis of overall survival, and will be an important part of monitoring for late effects, as will be discussed later. In line with assessments of educational and vocational needs, it's important to recognize the significant cognitive impairments as a result of primary malignancy or treatment effects, particularly radiation therapy. Later in this presentation, we'll see data of cognitive effects of radiation therapy, particularly with respect to age at time of radiation. Multiple studies have established the cognitive impairments of survivors of pediatric malignancies including a 2008 study by Butler et al. evaluating a randomized clinical trial of cognitive remediation program that included 161 survivors of childhood cancer ranging from ages 6 to 17 who were one year off treatment and had manifested attention deficit. This study demonstrated additional deficits as shown above, including verbal and performance IQ deficits, impairments in concentration, processing speed, working memory, and visual spatial functioning. Additionally, a 2009 publication by Journey et al. in the Journal of Clinical Oncology analyzed social outcomes in childhood cancer survivor cohort and found that childhood cancer survivors had high school graduation rates of approximately 80%, which is generally similar to their sibling comparison groups. However, survivors of childhood cancer were much more likely to require special education services at a rate of 19 times greater than their sibling comparison groups. Additionally, they found that survivors were slightly less likely to attend college. With recurrent surveillance, there is a chance to identify disease recurrence, which will involve a change in treatment plan, often with additional rounds of chemotherapy and radiation therapy, the cumulative effects of which may start to become more evident. Additionally, resumption of or increases in doses of steroids may also be implemented and require close monitoring. One clinical example of this within the stage of disease recurrence would be the recognition of myopathy, which we know can have an insidious onset of painless proximal muscle weakness. An important aspect of myopathy in these patients would be to identify whether the myopathy is truly steroid-induced versus critical illness, given the complexity of a lot of their care, and that important distinction is significant for their care in terms of determining whether a dose reduction would be necessary, because in the case of steroid-induced myopathies, it is typically reversible with cessation or reduction of the medication. Additionally, with myopathies, it's important to emphasize that resistance and endurance training may lessen, though not completely eliminate or reverse any muscular atrophy or weakness, though they may continue to have overall reduction in function. In certain situations, whether due to an aggressive tumor type or genetic profile or due to lack of response to standard treatment, there may come a time where cancer relocation must instead pivot more towards focus of patient comfort and independence. Similar to the Dietz classification of palliation, this stage of temporization and palliation really emphasizes patient independence as well as comfort. Oftentimes this will involve evaluations for durable medical equipment and assistive devices to help facilitate increased independence, along with additional caregiver education in terms of safe biomechanics for transfers and management of care needs, as well as more involved swallowing assessments to determine the appropriate nutrition and hydration plans outside of the hospital. For those who have cared for or known anyone with a diffuse-intrinsic pontine glioma will recognize that this is a cancer type that can typify the temporization stage of cancer relocation. As this Kaplan-Meier curve demonstrates, there is low overall survival for patients with diffuse-intrinsic pontine glioma, or DIPG, on the order of months to a handful of years at best. As such, it's important to recognize the overall prognosis in patients such as this and recognize that certain equipment needs will be better met with less customizable and more off-the-shelf options. For example, ordering a custom molded wheelchair that may not arrive to the family until the patient has passed away is something that should be avoided in this particular population. Hello, moving on to Pediatric Central Nervous System tumors, it's important to recognize why it's so important that we address these as a profession. So as we look forward, why are we talking about this, Dr. Pruitt and Dr. Haas have clearly defined that pediatric central nervous system tumors are the most common solid tumor and occur in about 32% of the tumors. And one of the biggest issues is that these tumors are a top reason for mortality in children. Another reason why we need to focus on this is because our expertise as physiatrists lends itself to managing complications of brain injury on multiple levels. So when do these tumors occur and what patients should we be expecting? What this slide shows is that our median diagnosis age of all brain and central nervous system tumors is about eight years of age. So it's important to recognize what stage the family is going to be in and where they are. An eight-year-old is typically in the early elementary stage of development and school will be a very important part for the families as we move forward discussing. The importance of where the tumor is and how we describe the tumor is extremely important in dealing with this type of population. This slide is to show that there's multiple ways of looking at central nervous system tumors. It can be histologic, it can be topographic. The importance is that we marry these two together and certain areas will have certain parts involved in the clinical presentation and understanding of where they are. So there are multiple ways of looking at this. One of the more clinically important ways of looking at this from a rehabilitation perspective is infertentorial versus supratentorial tumors and the presentation signs and symptoms that will come along with those. Typically, your symptoms and signs are going to be due to increased endocranial pressure. This is most commonly in the posterior fossa tumors and they can have some specific or non-specific ways. So for the presentation, your specific ways are usually our focal neurological deficits, which we're pretty familiar with. Our non-specific ways are increased academic issues, academic decline, fatigue, headache, and certainly agitation as the endocranial pressure increases. In pediatrics, we have the additional concern that infants aren't able to tell us what's going on. We have to see what they're doing and so there are quite a few ways of looking at this and looking for this in children and infants. Bulging fontanelles, large head circumference, sun-setting eyes that lead us to an understanding that there's increased endocranial pressure. So in looking at this from a clinical perspective, those are general signs and looking at signs that actually talk to where the tumor is, certainly infertentorial, which is in blue in the child. You will get complaints of clumsiness, ataxia from what we see, and some cranial nerve findings, difficulty with swallowing, and that sort of thing. In a supertentorial area, we're looking at our typical hemiparesis. We're looking at sensation deficits, visual complaints, and certainly behavioral changes depending on where the tumor is located. Seizures can be in all areas depending on how irritated the brain is. However, it seems to be more associated with our supertentorial tumors. So now that we've reviewed kind of why we're talking about this and why it's important, I want to talk a little bit about some important types of brain tumors and their prognosis. This is going to lead us to how we manage the patient, whether it's in an inpatient setting, whether it's an outpatient setting, and the approach, the clinical approach which we are going to take as care providers for these children. Are we looking at a recovery standpoint or are we looking at a palliative standpoint as Dr. Haas previously discussed? So on one side of the slide here, we have a listing of the number of types of brain tumors and central nervous system tumors and how they're broken down. We are not going to talk about every single one of these. We're going to talk about a few. So the first most common type is the gliomas. The gliomas have an extremely large range of grades or of severity as shown here on this slide on the pilot under the pilot cystic astrocytoma grades 1 & 2. This would suggest that it is less cellular and that it is absolutely something that may be treatable versus the far side of the slide under the glioblastoma grade 4 where you can see the hypercellularity that is very infiltrative and aggressive. So in looking at the gliomas because they are the most common in our astrocytoma subgroup in our low grade, we get about a 78% of 8-year survival rate. I'm sorry, 96% survival rate and progression-free of 78%. Again, usually these are superior posterior fossa tumors and the extent of the resection and the extent of treatment is usually gross total resection and with minimal to no radiation or chemotherapy, though for some types they certainly are. These tumors are mostly located in the cerebellar regions and it's more associated with the posterior fossa syndrome which Dr. Haas talked about earlier. One of the more aggressive tumors and the most common malignant tumor in children is going to be an embryonal tumor called medulloblastoma. There's been a lot of research that's gone into medulloblastoma specifically because of its poor prognosis and the prognosis is dependent on staging actually genetic testing of the tumor type and the age of the patient. Unfortunately, this type of tumor disseminates early throughout the system and can be a significant issue. So the range of survival is actually 30 to 80 percent and so your average risk is going to be about 78% whereas your poor risk is going to be in the 30% range of 5 to 10-year survival rate. Earlier, I mentioned molecular subgrouping or genetics and how they play into these pieces and how aggressive we are with our treatment. So some wonderful studies that have been done have truly looked at the prognosis and which subgroups require more aggressive and less aggressive treatment to increase your survival rate. So as a whole, those were two of the most common tumor types as a whole. Survival rate is in the 73 to 74 percent range for all central nervous system tumors and that is remarkable. However, what that indicates is that there will likely be while there is a decrease in mortality, there is a going to be a group of folks that have a high morbidity which is where our profession comes into play. So when we're looking at this and the things that we need to be aware of as clinicians that manage children with these types of injuries or deficits, we have to understand that surgery is going to be a mainstay. Whether it's a gross total resection or a subtotal resection is part of what we need to know as we move forward with the families and move forward with them in a way that is most appropriate for their child. Chemotherapy is changing regularly. There are many, many study groups and enrollments of children into different study groups and the oncologic profession is doing an amazing job at this. What we need to be aware of as clinicians is some of the top ones, some of the top drugs that are utilized and how they affect us. Two of them were discussed earlier by Dr. Haas. Here is one nice representation of some of the more important ones and where they affect affectionately called chemo man. This came out of Tulane and is very nice. There are various versions of this all over the place. I will not belabor this at this time but I do recommend that if you're going to be treating children that are receiving chemo that you be very familiar with some of the side effects of the more common drugs. The next component that goes along with this is radiation therapy. Most radiation therapy in children has moved towards proton therapy and there is a significant difference and morbidity mortality associated with photon versus therapy as we're looking at radiation therapy. As is described here on the image, proton therapy is a much more directed therapy and does not usually affect areas past the tumor whereas photon therapy or typical radiation therapy does. What we have to be aware of is that while children may not show initial effects within the first couple weeks, there will come a time during this for most patients around the third to fourth week of treatment that fatigue causes significant problems with participation in therapies and goals of treatment may change at that point. We need to be very acutely aware of that. So what can we do as a physiatrist in dealing with children? Certainly we can deal with focal motor deficits and ataxia. Some of the biggest issues that we have are dysphagia. It's isolated whether it's from cranial nerve deficit or whether it's due to mucositis from chemotherapy. These are aspects of the management that we have to be aware of because a dehydrated child is at very high risk for just about everything. They certainly have apraxia and sensory deficits. The cranial nerve deficits is going to affect them in a visual motor way as well as in a headache way which needs to be addressed either with patching or glasses or an understanding of where the child's going to be when it comes to visual motor integration. And so some of the aspects that we are able to do things with certainly spasticity management which most physiatrists are very familiar with and then different modalities. So constraint-induced movement therapy, partial weighted ambulation, FES. In our picture here we have FES showing the ability to extend the wrist while playing in Wii game which is quite a bit of fun for the children as most of them are very interested in video games these days. And then we certainly have cognitive vestibular and visual rehabilitation therapies. As Dr. Haas alluded to earlier, I think it's very important to understand that when you are looking at the orthotic and equipment needs and even therapy needs of the child with cancer, it is important to understand what you're shooting for and what the prognosis is of the type of tumor that you're dealing with. I think that at some point you will even have to address this in the middle of treatment depending on how the child is reacting to the chemotherapy that has been prescribed or is being given and the type of radiation therapy and the timing of that radiation therapy and where the child is. So in some cases it's important to plan for that fatigue that will occur in the future. In some cases it's important to look at the idea that in palliating situations we do not want to be delivering equipment after the child has passed and our goals in discussing this with our teams have to be focused and as leaders of the rehabilitation team we need to be able to express this to our team that the goals of therapy may be palliation. The goals of therapy may be to get home and enjoy what we have left. Other goals may be having the conversation of lack of return to 100% of what we were before this illness which is very familiar to all rehabilitation experts. At this point I will pass it along. Currently the overall five-year survival rate for children and adolescents with cancer diagnoses as you know as we've discussed has increased significantly as a result of the advancements in diagnostics and treatment options that are available for these patients. Children often tolerate and manage the acute side effects of treatment relatively well but the utilization of multimodal cancer therapy at an early age may result in complications that may not become apparent until years later as the child matures. Referred to as late effects, these late occurring or chronic outcomes persist or develop greater than five years from the cancer diagnoses. Late effects vary with the primary cancer type, its location, its treatment and potential or genetic or underlying medical conditions. Many of these late effects develop slowly and are often not evident at the end of treatment. Medical effects of these treatments are common and can impact almost every organ system. All survivors are at risk for experiencing the effects of cancer and its treatments on psychological and emotional functioning as well. The Institute of Medicine has recognized over the years the need for a lifelong surveillance plan for cancer survivors that identifies the risk based on the therapeutic exposures that the patient had as a result of treatment, the genetic predispositions they have, health-related behaviors as well as comorbid health conditions for cancer survivors across the spectrum of ages. So in result of this and a result to this, the Children's Oncology Group or COG developed risk-based exposure related guidelines, the long-term follow-up guidelines for survivors of childhood, adolescent and young adult cancers for follow-up of care patients who have completed treatment for pediatric cancers. The COG, as Dr. Niedzwiecki mentioned, is an international clinical trials group devoted exclusively to childhood and adolescent cancer research and they put together a number of trials that our patients are on for treatment of their cancer diagnoses. The guidelines that I talk about can be downloaded by anyone at the following address, www.survivorshipguidelines.org. I'm going to take you through the navigation of these guidelines and demonstrate some examples for you. So if you enter survivorshipguidelines.org into your navigation bar, you're taken to the homepage for the long-term follow-up guidelines for survivors and childhood, adolescent, and young adult cancers. If you click on where the blue arrow is, COG long-term follow-up guidelines, it's going to take you to the guidelines content, which are on our next slide. And this is the introduction page. And as you scroll down, you're going to come across the contents page, which nicely breaks down late effects by a number of different categories, including late effects that are due to any cancer experience that the patient has had. Chemotherapy, specifically by agent. So you can look at the specific agent that a patient was treated with and look to see which long or late-term effects are associated with that particular chemotherapy agent. Radiation, surgery, bone marrow transplant, the list really goes on. It's a really, really nice guideline to follow. So for example, if we wanted to assess the late effects from radiation exposure, we could look at the specific guideline, which for cognition and cognition effects due to radiation. If we click on this and look at the information that's provided, it's going to give us some really good information about the potential late effects associated from radiation that affect cognition. The recommended periodic evaluation for this condition, as well as some further recommendations to consider for the management of this particular recommendation, as well as some up-to-date references to consider and to look at to further update your knowledge in that particular area. Many of the survivorship issues outlined in these guidelines are directly related to function and should involve the experience of a physiatrist in the evaluation assessment and treatment recommendations. Some examples that benefit from physiatric expertise include the areas that are listed here, but there are certainly many more areas outside the scope of CNS tumors. So there are plenty of late effects related to long bone sarcomas, for example, and amputation or limb sparing surgeries. I'll go into some detail about some of the late effects that are related to CNS, since that's the topic we chose to center our discussion about over the next slides. Many posterior fossa tumors are complicated by hydrocephalus and the accompanied increased intracranial pressure at the time of diagnosis, which often precipitates some of the symptoms that are eliciting the workup, such as frequent emesis and headaches, but also can include a delay in milestone achievement in cognitive and vision deficits as well. At the time of diagnosis, a number of these patients will require CSF diversion with an EVD at the time of surgical resection of their tumor. A number of these children will have successful removal of their EVD within days to weeks postoperatively, but a number will also require placement of a VP shunt for ongoing CSF diversion. One study that was conducted at Duke University evaluated the relationship between shunted hydrocephalus and neurocognitive functioning in a group of 35 survivors with a diagnosis of medulloblastoma. Of these survivors, who were at least one year post-treatment, 10 required VP shunt placement for hydrocephalus in addition to tumor resection itself. So the results from their study revealed that participants with shunted hydrocephalus compared to the patients who didn't receive a VP shunt had significantly lower IQs, had lower intellectual functioning, had lower academic skills in writing and math, and impairments in visual motor abilities. This is just an example of the COGS long-term follow-up guidelines for survivors, which provides a description of the potential late effects in cognitive function related to neurosurgical interventions, including hydrocephalus, which we've just discussed. So these recommendations are specific in regards to the recommended periodic evaluation for cognitive function for those patients who have had a neurosurgical intervention for hydrocephalus, which in this case is an annual assessment of the educational and vocational progress, as well as the screening evaluations that are recommended, which include a referral for formal neuropsych evaluations at baseline to entry into survivorship. Although many of these children have had one or more tests, neuropsychological tests, during the period between diagnosis and entry into survivorship. And then the recommendation is to have periodic follow-up with neuropsychological evaluations as clinically indicated. There are additional considerations for interventions also provided within this guideline. As survival rates for children diagnosed with posterior fossa brain tumors have improved significantly over the past few decades, long-term functional assessments have become priorities as well. Many outcome measurement tools are instituted to evaluate specific areas of functioning and include a number of different measures. So we have the six-minute walk test, the TUG or the timed up and go test, the Brunex-Ozuretsky test of motor proficiency, as well as the Canadian Occupational Performance Measure, or the COPM. It's helpful to have these outcome measures for assessing and reassessing areas that challenge the patient, and also to help us properly institute treatment programming for these patients. A study that was conducted at the Hospital for Sick Children evaluated 30 patients with posterior fossa tumors at a mean time of six years from diagnosis using the Brunex-Ozuretsky test of motor proficiency. As a group, significantly decreased functioning compared with norms was observed in balance and running speed and agility. Specifically in balance, 70% of the participants performed below or well below average. It's really important to keep in mind that these deficits, which are often identified at the time of diagnosis and presentation, continue to be present long-term and demonstrate the need for ongoing intervention from the physiatrist and therapy teams. This is one of the prime examples that I provide in discussing the benefit to having a pediatric physiatrist involved in the acute oncology clinics, as well as in survivorship clinics as well. Even with multidisciplinary team involved in the care of the patient, function and independence really rarely rise to the top of the many, many medical issues that are addressed in these clinics. So having a pediatric physiatrist involved in caring and focusing on function is really important. There's really great benefits in having a provider with interest and expertise in mobility deficits and ADL function and cognition to really guide the patient and family to optimal independent functioning. One last area of survivorship for patients with CNS tumors that I'm going to chat about is cerebrovascular complications, which can include development of stroke, moyamoya, occlusive cerebrovasculopathy, or even cavernomas. Alterations in cerebrovasculature may occur as a consequence of radiation therapy. And development of arterial disease is really a late phenomenon. Therefore, stroke as a result of cranial radiation therapy typically occurs years after treatment initiation. And the phenomenon is really most common in children irradiated before five years of age, in the setting of neurofibromatosis or NF1. Those patients typically have optic gliomas at the time of diagnosis. And so we're doing a lot of radiation around, or we typically in the past, I should say, did a lot of radiation around their vasculature, just above their internal carotids and into their frontal area of their brain. The median time to recognition of moyamoya syndrome is three to four years post-therapy, really putting children and adolescents at considerable risk for stroke at a relatively young age. And this, again, is the children's oncology slide, which talks, again, about what the recommended evaluations are and assessments and screening and so forth for monitoring for any radiation effects that could lead to cerebrovascular issues. So the COGS current long-term follow-up guidelines recommend that survivors of childhood and young adult cancers who received radiation treatment have annual neurologic exams and pursual of neuroimaging for any neurologic concerns that are identified. But as we know, often these events occur more urgently and outside of the survivorship clinic setting. It's also recommended that patients with additional late effects that might arise as a result of treatment and impact the risk for stroke, such as hypertension, diabetes, and obesity, receive instruction and counseling to reduce those added risks. Now I'm going to move on to pass the talk on to Dr. Haas to talk a little bit about the role of the pediatric rehabilitation medicine within cancer rehab. As we've hopefully demonstrated, the childhood and young adult cancer population has substantial need for physiatric expertise, both in terms of volume as well as the degree of functional impairment. And in order to understand how pediatric rehabilitation medicine is poised to provide this care, I think it's important to assess kind of the role of those providers. When we evaluate the current state of pediatric rehabilitation medicine nationally, there are on the order of a few hundred pediatric rehabilitation medicine physicians. Additionally, there's approximately over 30 trainees in combined residency program, as well as fellowship training that will become part of that workforce. But the majority of practices lie within academic centers, specifically centers that have oncologic care. Most of these practices end up having a few number of physiatrists combined with some non-physician providers, such as nurse practitioners or physician assistants. And typically based on overall prevalence of other diagnoses that cause significant disability, most providers end up having a bulk of their clinical practice devoted to diagnoses such as cerebral palsy, traumatic brain injuries, myelomeningocele, and are not able to focus as much on cancer populations. This is obviously dependent on overall practice size, where larger groups are able to have providers focus on more subspecialty populations, as well as overall practice locations. For example, within a center of excellence for certain diagnosis that may lend to a greater percentage of their clinical time spent to that population. Understanding how new trainees in pediatric rehabilitation medicine are trained to address the needs of pediatric cancer populations, we must first look at the ACGME requirements for fellowship training, which are shown here. Specifically looking at the core pediatric rehabilitation diagnostic categories of which cancer is not included in the most recent revision from July of 2020. Additionally, looking at the American Board of PM&R subspecialty examination blueprint, which is an indication of the subspecialty examination content questions, shows that roughly 5% of questions highlighted here by the blue arrow section D, 5% would be related to, should be expected to be related to cancer, which shows that overall there may be limited training and exposure to cancer rehabilitation concepts, as well as populations within these trainees. Of those who do encounter childhood and young adult cancer populations with high frequency, this is typically during the inpatient domain, arena of delivery. And with that, there are significant challenges and barriers to these patients participating in that level of therapy. Primarily includes timing of the therapy intervention. Prime example of that would be prehabilitation, which is contingent on a referral and knowledge from the oncologic teams of the value and utility of having rehabilitation medicine consulted early, as well as the adequate resources in order to achieve that intervention, as well as understanding the timing of what comes next. Additionally, need to understand any specific precautions or post-operative management concerns that would limit participation, such as in a sarcoma patient who has a restriction and weight bearing precautions, waiting until those precautions are lifted in order to get the most out of their therapy time. Additionally, in terms of the interplay of other therapies, it may be important to talk with your referring providers about different options to mitigate certain side effects, such as nausea or vomiting or fatigue that may limit the participation in therapy to less than three hours of therapy per day, which we know is required by insurance. Additionally, you may need to have special consideration for side effects related to treatment that may impact sort of activity restrictions, such as anemia or thrombocytopenia that would limit the ability to do more resistance type training exercises, or any restrictions to participation that would include things like neutropenia and not allowing patients to be in group settings or being limited to kind of in-room therapies, which obviously, as we know, has an impact on the overall opportunity for improvement. Additionally, having an understanding of unexpected transfers back to the acute care side, which would obviously interrupt their rehabilitation course, for example, fever in a patient with neutropenia that would require IV antibiotics. Additionally, there may be a need to consider insurance benefits for the patient. This includes understanding that the treatment course of disease may involve more significant functional impairment later on and trying to balance the needs at that point with what you're currently dealing with. That may be a factor. And lastly, there really needs to be a high amount of patient and family as well as buy-in from the team, both your providers, your referrers, as well as your rehabilitation team, as Dr. Niedzwiecki had mentioned. I think it's important to have appropriate expectations set with the family in terms of the frequency of therapy, the intensity of therapy, so that they know what they're in for. And with that, talking with the referrers to kind of have a dialogue about the priorities in terms of timing. And so if there's a one-week interval between chemotherapy cycles and their rhetoric is that they want to allow the patient to recover and kind of rest up for their next round, kind of breaking down that thought process and trying to allow for more interventions that may ultimately improve their overall tolerance of that therapy as well as overall recovery is important. And then more specifically, as Dr. Niedzwiecki had mentioned, making sure that the team has buy-in, especially in those cases of kind of more temporization or palliation, where the focus is much more on caregiver training and DME acquisition, which is oftentimes very different than what the team is focused on. Beyond acute inpatient rehab, there exists significant opportunities to expand into outpatient rehabilitation. A prime example of that would be kind of follow-up after an acute inpatient rehab stay, which most providers typically end up doing. High-quality care of shared patients with our oncology colleagues will also result in additional outpatient referrals for more specific opportunities of physiatric expertise in terms of gait impairments, as well as spasticity interventions. And with that, then you may have opportunities to have greater integration within a more comprehensive oncology clinic, as we have here in Cincinnati. That in turn will allow you to interface with a lot more of your oncology colleagues and allow for opportunity to expand beyond the oncologic diagnoses that have typically high functional impairments and ultimately result in inpatient rehab needs, such as neuro-oncology and in-tube diagnoses that ultimately have higher overall survival, such as leukemia and lymphomas, as previously discussed. And this final slide shows some specific roles that pediatric rehabilitation medicine can play within comprehensive clinic settings, both inpatient as well as outpatient. Those who practice pediatric rehabilitation or rehabilitation medicine in general will not find any of this all too surprising, but I think it helps to capture a lot of important information that can be conveyed to oncology colleagues. And in my personal experience, I'm currently interviewing for faculty positions and a particular interest in cancer rehab. My conversations with the oncology teams has been that they're trying to navigate on their own and are really looking for a partner in this care and recognize the importance and the value that pediatric rehab medicine provides in the overall spectrum of oncology care. So with that, we'd just like to thank you for joining our talk today and listening in, and certainly reach out to any of us with any questions that you may have regarding our presentation. Thank you.

pediatric rehabilitation medicine

cancer care

interdisciplinary team approach

pediatric physiatrists

central nervous system tumors

late effects

cancer rehabilitation

long-term effects

quality of life