Hematologist-oncologist Robert Raphael, MD, director of the Pediatric Survivorship Program, lays out keys to protecting these patients’ physical as well as mental health. Reaching adulthood after cancer is more common than ever, but with risks that range from recurrence and heart disease to depression and unemployment, survivors need PCPs who know their histories and follow-up needs. Includes resources for designing appropriate care plans.
well welcome everybody. Thank you for joining in. And I'm just going to jump in without any further ado because we have a lot of material to cover here are the things I wanted to cover? I'm going to start with a sort of case presentation to get you thinking a little bit and I'll move into an overview of late effects of childhood cancer treatment and then we'll talk about what long term follow up looks like or should look like as we follow these survivors and we'll return to our case a little bit. And then I've got some slides with resources and references at the end and I have no relevant or even irrelevant financial disclosures to mention. So uh here's a scenario, just a little thought experiment but based on a real patient that we've seen in our survivorship clinic. Um Imagine You're seeing a 15 year old female coming to your office for the first time to establish care. You talked to her. She reports that she has a history of Leukemia at about age two. She also happens to have epilepsy that's controlled currently on medication and otherwise she's healthy. She has no acute concerns. So as a primary care provider, what are the things that you would like to know? Well, to start with? You'd like to know what were her relevant treatment exposures and what are the potential late effects that you should be looking out for as you perform a history and physical exam. What are the important things that you should be focusing on and be certain not to miss and what surveillance testing or imaging or laboratory studies are required and then how should this patient be followed? Moving forward. So to start in talking about late effects and survivorship issues, we always want to start by sort of touting our great success. Currently we are justifiably proud in um our greater than 80% survival rate for childhood cancer. Overall That translates to over 420,000 childhood cancer survivors currently living in the US and that number is probably outdated And that's up to 101 in 640 adults up to the age of 40. You can see in this graph here the tremendous success and how things have changed over time to a different treatment eras. And I don't know if my cursor shows up. You can see the blue line at the bottom is uh Children treated in the 1970s, the 1980s, the later 1980s, the 1990s. And on up through 2005 um with the improved success with each successive generation of clinical trial protocols. And then here on the right the survival rates for the different major forms of childhood cancer. The most common things that we see all of this success is laudable and wonderful but comes at a cost. And it's that cost that I'm going to spend a lot of this talk focusing on two thirds of childhood cancer survivors have some form of chronic late effects of their treatment. And a third of those late effects are considered severe or life threatening. In addition a quarter of survival survivors have significant psychosocial problems. 10% of them report persistent cancer related pain And the risk of death. 30 years after diagnosis is eight times higher than that of the general population. There's a cumulative prevalence of chronic medical conditions that is 95% by the age of 45 and survivors are between two and five times more likely to experience poor health, mental health concerns, functional impairment and activity limitations uh compared with age matched controls or siblings. So as we think about classifying late effects, we can put them into one of two broad categories. Right? The medical and psychosocial medical effects are things like second malignancies. Organ dysfunction like cardiac and pulmonary and renal disease, infertility, endocrine disorders and obesity and diabetes, musculoskeletal and physical defects, impairments and growth neurologic problems, chronic pain and as I just mentioned, early death on the other side of the ledger the psychosocial problems including cognitive dysfunction, depression, anxiety. Ptsd and low self esteem problems in academic work and unemployment and time off work, substance abuse, interpersonal interpersonal difficulties, insurance problems and financial toxicity. How do we know what we know about childhood cancer survivors? A lot of the material I'm going to present today. Uh comes from the work of the childhood cancer survivor study or the C. C. S. S. This is the largest and most studied cohort of childhood cancer survivors. It covers over 4000 survivors who were less than 21 when they were diagnosed. These are patients who were treated between 1970 1986 and needed to have survived at least five years from their original diagnosis. And these patients were collected from 26 participating centers across the United States and Canada, of which U. C. S. F. Is one diagnoses included most of the most common childhood malignancies including leukemias, lymphomas, neuroblastoma, soft tissue sarcomas, bone tumors, brain tumors and Wilms, tumors. But it's not an exhaustive list and it doesn't include everything notably absent. For example would be germ cell tubes. The database includes a lot of details about diagnosis and treatment including detailed history about radiation doses and fields and chemotherapy treatments. The subjects fill out extensive health questionnaires at the time of enrollment. There's also a random sampling of nearest age living siblings included for comparisons. A number of follow up questionnaires have have followed as well as an expanded cohort for more recently treated survivors up through the 1990s. Now the C. C. S. S. Isn't everything. It's not the be all and end all. So other groups of course study childhood cancer survivors as well including the Children's oncology group ST jude's uh and a number of others in the United States and in europe. I'll spend a few a few slides talking about some of these broad categories of late effects now. And I'll start with early mortality. There is an 18% mortality rate at the time of 30 years from the original diagnosis. As you'll see, the most common cause of death Is from recurrence or progressive disease. That accounts for almost 60% of this early mortality. Outside of that second malignant nia plasm account for another 18.5% and cardiovascular diseases another almost 7%. As you can imagine, there's a transition in the cause of death over time with recurrence and progressive disease, accounting for most of the early late mortality. Uh, and these other conditions accounting for much of the later late mortality. Risk factors for death by 2nd Malignancy included exposure, radiation therapy, higher doses of alka weighting agents. And the top aside, risk factors for cardiovascular death include cardiac radiation and higher doses of anti recycling. None of which is surprising. A little bit of good news here is that if you look out over time, there's been a reduction in the 15 year mortality among the expanded see CSS cohort through the 19 nineties. From 12.4% in the earlier cohort listed uh, graft in blue here in this Kaplan Meier curve Down to six At that 15 year mark. Among the later Cohort. A lot of that reduction in mortality has come from reduced um, death by disease progression or recurrence, as you can see here in this graph and among those remaining, there's also been a reduction in mortality from second malignancies from cardiac and also from pulmonary causes. And those improvements are associated with reductions over time, as our therapies have changed with less radiation and fewer patients receiving radiation as well as reduced anthro cycling exposures. Moving on now to talk about morbidity and chronic disease and I'm going to spend a few slides detailing this because I think it's important and gives you a nice kind of snapshot. As I said in my introductory slide, survivors are 2.5 times more likely than their match sibling controls to report having adverse general health. Um, that's 11% versus 5% at a mean age of about 27 years. There are also three times more likely to report activity limitations and five times more likely to report functional impairments. Overall, 62% of survivors report having at least one chronic condition at a mean age again of about 27 years. And of those over a quarter are considered severe or possibly life threatening. In addition, almost a quarter report having at least three chronic health conditions. The relative risk for having chronic diseases 3.3 times out of their sibling controls and eight times that of their sibling controls for a grade three or four more serious conditions. The cumulative incidence of chronic disease is almost 75% At at the 30 year mark from the time of their diagnosis and that's still 42% if you only look at the more serious grade three and four chronic diseases. To give you an idea of what chronic diseases I'm talking about. This table is a snapshot of certain highlighted categories including major joint replacement, congestive heart failure, second malignancy, cognitive dysfunction, coronary artery disease, strokes, renal failure et cetera. You can see that in terms of absolute numbers out of over 10,000 survivors in this C CSS cohort The percentage reporting each of these individual conditions is not all that high. 1.6%. 1.6%. But as you compare them to their siblings where the numbers are so low that translates into a relative risk that's quite significant. 54% relative risk for major joint replacement. 15%. Relative risk for a second malignancy. This series of curves illustrates the increased cumulative incidents over time for chronic health conditions. Overall in this C. C. S. S. Cohort but it's broken down by different cancer diagnoses. Um For this in the upper left is total survivors and then you've got leukemias and brain tumors and Hodgkin's and non hodgkin's lymphomas etcetera. You can see how it differs across these different diagnostic categories. You can also see how it increases over time from earlier to later up to 30 years off therapy or post diagnosis. The blue lines are all grades and then the red lines highlight the more severe grade 3 to 5 um chronic health conditions. This is a study not from C. C. S. S. Now but from the ST jude lifetime cohort of over 3000 adult survivors of childhood cancer. And their methodology was a little bit different. But the out but the result is not all that different Here. They report a cumulative incidence of chronic health conditions by the age of 50. Now of Basically 100%. There's an average of 17 chronic conditions that have accumulated by the time these survivors reached the age of 50. And that compares to nine average chronic chronic conditions for their control cohort. And so this is illustrated here in in this graph with the controls in the dotted line and the survivors in the blue and the red lines up here. This same ST jude lifetime cohort just recently published this article looking at an earlier time point the cumulative burden of disabling conditions at key moments of transition for this these survivors. So they looked as a snapshot at age 18 when a lot of patients may be transitioning from oncology follow up to the primary care arena And also at age 26 when if they haven't already, their insurance is gonna switch and they're going to have to make a transition from the pediatric follow up realm to the adult follow up realm. So this uh graph in a is at the 18 year mark and then the one below is at the 26 year mark. And it's broken down here on the left are the controls. Um and you can see that at age 18 an average, uh there's an average of 22 conditions per 100 individuals compared with only 3.5 for controls. That increases up to 40 conditions per 100 individuals vs 5.7 for the controls. Again, listed here on the left, these are also broken down by the different major disease categories. Um and you can see that the types of cumulative um that the types of chronic health conditions which are color coded here vary as you might expect based on the original diagnosis. So I'll highlight things that jump out at me among the bone tumors. Not surprisingly, and yellow here, there is a preponderance of musculoskeletal as well as neurologic problems and among the central nervous system or brain tumor patients. Again, not surprisingly, there is a lot of neurologic late effects just to give you kind of a snapshot of what we're looking at even among a relatively young group of survivors. A little bit of good news buried in here. This is back to some see CSS data now, looking at the 20 year incidents of more severe grade 3 to 5 chronic conditions. It is lower for the more recently treated Patients. So you can see here in patients in the blue are those treated in the 70s and the red is in the 80s and in the green More recently in the 19, I'm sorry, I've got that totally backwards degrees in the 70s, the red is in the 80s, the blue is in the 90s as compared with their uh sibling cohorts with a rate of only 4.6%. The more recently treated survivors have gone down from 33% for the older cohort down to 27% for the more recently treated survivors. In the 19 nineties, we've seen a decreased incidence of endocrine open these of second malignancies of musculoskeletal and gastrointestinal problems. Um but at the same time there's actually been a higher incidence of some diagnoses for more recently treated patients as the treatment intensity has increased for certain diagnoses. For example, Medalla blast oma and neuroblastoma. So the increased survival rate for those diagnoses once again has come at a cost. I'm going to move on and talk about a different concept that a lot of pediatricians are not too aware of and honestly I wasn't too aware of until I started delving into the survivorship literature. And that's this concept of frailty. So frailty is defined as a state of reduced physiologic reserve that increases susceptibility to chronic disease and disability. Typically it has a gradual onset and is associated with aging lifestyle choices such as smoking or physical in activity as well as acute insults like illness or injury can affect the trajectory of the decline, frailty often precedes the onset of chronic illness and disability. And so it may offer identifying frailty may offer an opportunity for lifestyle modification to alter the trajectory. So this is diagrammed here schematically, as you can see here is the dotted line is the borderline between frailty and healthy function and here is your physiologic capacity and then on the X axis is age. So here's our survivor who has the acute insult of a childhood cancer and the resulting treatment. And then you see this inexorable decline in physiologic capacity toward the line of frailty. Well, maybe if we can alter this trajectory here by changing our lifestyle choices, we can change the slope of this line and delay the progression to the point when frailty and all the associated problems might ensue frailty is defined in a in a few different ways. The most common is this set of Fried's criteria. So just to give you a sense of how researchers study this, uh the criteria include shrinking, that is uh unintentional weight loss of greater than £10 in the past year. Self reported exhaustion weakness as assessed by grip strength, slowness as assessed by a standardized walk test and then low physical activity levels. So this has been studying in a few different survivorship settings. The ST jude lifetime cohort um which evaluated patients at a median of 33.6 years. So these are not old individuals. These are young adults reported frailty and almost 13% of males and 31 a half percent of females. This compares with 9.9% of frailty at 65 at age 65 In the general population. So this group of survivors is more frail than the average 65 year old. They also found an increased risk of new chronic health conditions with the relative risk of 2.2 as well as of mortality, with the relative risk of 2.6 among the frail survivors in their cohort compared with those survivors who did not qualify as frail. In another, more recent analysis by the sea CSS frailty was reported again in 6.5% of survivors, compared with 2.2% in matched siblings with the highest prevalence in pay in survivors of central nervous system and of bone tumors. Additional risk factors they identified were prior exposure to radiation, particularly brain and abdominal or pelvic, as well as high doses platinum amputation and lung surgery. This is the good news in here is that the risk was partially attenuated by lifestyle factors. A brief slide here about second nia plasm, the incidence of second malignancy or second nia plasm, I should say, at 20 to 30 years from the time of original diagnosis ranges from 3 to 8% and that six times higher than that of the general population. There is a whopping, 43 fold increased risk of breast cancer following lung radiation. But there is even a four fold higher risk of breast cancer after chemotherapy without radiation As well as a 3.5 fold higher risk of Sarcomas after aunt recycling chemotherapies. The second nia plasm incidents by the time you reach the age of 40 to 45 is 16.3% and that's over twice the risk of the general population at that age. A big chunk of that comes from non melanoma skin cancers. So canceling patients again to be on the lookout for skin cancers and to practice proper, you know, some safety with sunscreens and avoiding exposures is important. Cardiovascular disease. I will highlight among the various organ late effects because it is the leading cause of non cancer morbidity and mortality with the risk of eight times higher than that of age matched siblings. Risk factors for this include an three cycling exposure. With higher doses being riskier as well as radiation to the heart. If you look closely enough, over 50% of patients exposed to one or both. Risk factors and they are additive by the way, We'll have signs of cardiac damage within 5-10 years after exposure and those signs of damage tend to increase over time. But most of that damage at least early on is subclinical more about that later. But now I want to talk a little bit about infertility. Uncle fertility could really be an entire talk in and of itself. But I've dedicated just one measly slide to it to tell you that survivors are less likely than their siblings to have been pregnant or to have sired a pregnancy. And that's apparently the official term. Uh with a rate of 38% versus 62% in the general population or in their in their matched age. Sibling controls in the C. C. S. S. Risk factors include radiation exposure as well as calculating agents particularly in higher doses. And this is an area where dose as well as age of exposure matters. And it's also an area where the sex of the patient matters because male fertility turns out to be more sensitive to chemotherapy than does female fertility. There's a 46% rate of infertility among male survivors in the C. CSS cohort versus 17.5% for their brothers. 37% of them reported having fathered a child versus almost 70% of their similarly aged siblings. Female survivors in the same cohort were 1.5 times more likely to report infertility than were their siblings. Two thirds of them ultimately did report having achieved a pregnancy. But they also said that it took a longer time for them to become pregnant compared with their siblings, premature ovarian failure is a separate but related issue um affecting 11% of female survivors in the C. C. S. S. Cohort. This is at a median age of 30 about 32 24 years from the time of diagnosis. Think about that for a minute that's highly significant. This is long before um uh menopause or or ovarian failure should be an issue. And so um uh impaired fertility among younger um uh female survivors when they have relevant risk factors is also an important thing to watch out for pediatricians. So as the recently deceased Ron Popeo would have said, but wait, there's more, there are other late effects that I'm not even going to have time to get into talking about. And these slides just go through the titles of some selected articles from the recent survivorship literature to give you a taste of some of these other potential organ toxicities. Musculoskeletal late effects, performance limitations, um chronic pain, endocrine disorders, obesity and metabolic syndrome, nervous system complications after stem cell transplant, peripheral neuropathy, ocular late effects or eye problems, hearing loss, dental problems, pulmonary problems have had a biliary problems, renal toxicity, urinary bladder. The list goes on and on. But rather than go on and on with that list, I want to switch gears and spend at least just this one slide talking about another big topic, musculoskeletal problems. Now, most survivors really are psycho socially well adjusted, but they are twice as likely as their siblings to report adverse mental health. They are at increased risk as a group for depression, anxiety, PTSD and suicidal ideation. They're at increased risk for delayed psycho social development. And they also report a lower rate of marriage or cohabitation of college graduation and of full time employment. They have a similar or actually slightly lower rate for risky behaviors like smoking alcohol and other substance abuse. The risk factors for some of these poor psychosocial outcomes include prominently cranial radiation and having had a central nervous system tumor and also the physical and medical late effects that I just mentioned. So the survivors who have the more who are more severely affected by these medical complications are the very same survivors more likely to be adversely affected with psychosocial problems. And this is not particularly surprising. Um I'll highlight as well that A. L. L. Survivors not all but A. L. L. Survivors who were treated even without cranial radiation but with a lot of intra fecal chemotherapy directly uh in their spinal fluid are still at increased risk for A. D. H. D. For learning problems for executive functioning problems. As well as issues with processing speed memory and I. Q. When you test them with neuropsychiatric testing, all of these survivors would be recommended to receive screening neuropsychiatric testing. But it's a resource unfortunately that's all too limited in our community on the basis of uh poor reimbursement and limited insurance coverage. But it's something we would love to do more often than we can do. So looking at all of those late effects together. How do we decide what it is that a given survivor is at risk for. So the things we should be considering and thinking about and taking into account are the type of cancer that that survivor had. As I showed you in some of those previous slides, the treatment exposures that they received here. The difference is the distinctions and the details matter. So, knowing about the particular chemotherapy agents as well as the doses that they received radiation therapy. Again, knowing what area was radiated to what dose and what modality was used, protons versus photons versus breaky therapy etcetera among patients who received a stem cell transplant. Again, what was the conditioning regimen? They received? What was the source of their stem cells? And did they experienced graft versus host disease with which carries with it its own set of potential late effects for patients who had surgery. Again, the type of surgery, the location and their body of the surgery. Um, for patients who received blood products, calculating the total volume of blood cells they were exposed to as well as the era in which they were treated for infectious risks like hepatitis C. Are things to consider as well as for the risk of transfusion allow iron overload and the things that come with it. The age of the patient matters both the age of diagnosis and treatment as well as the age by the time a follow up to think about what are the things they are at greatest risk for it at that stage of their life, the sex of the patient matters, particularly for things as I mentioned like fertility. Um as well as the patient's genetics, This is an area of rapidly expanding research to help to try to better understand what factors the patients carry with them that may affect their risk for late effects. So this is kind of the heart of my talk. And the thing that I want to emphasize to you is why long term follow up matters matters for a lot of reasons. As I hope I just emphasized and and demonstrated to you these medical and psychosocial late effects are really and truly significant. What we hope is that long term follow up when properly done may give us an opportunity to identify problems early and therefore an opportunity to intervene and perhaps reduce the risks or to affect the trajectory of decline patients therefore need to understand the risks. Many may have no problems or symptoms for years. They need to know to avoid additional risks and they need to know when something is wrong. Patients need to understand and know their history. The details of their cancer treatment are complex, but they do matter and because the risk of their late effects does depend on their particular treatment history, but at the same time it's very difficult for them to keep track of medical records. Health care providers need information that's you guys diagnosis and treatment history or key for you to understand what's going on and how to best follow and manage these patients so that you can understand what are their current and then their potential late effects and there is really a need for communication between both the pediatric oncologists, the primary care provider and other specialists, specialists who may be involved or become involved in those patients care. We all need more research because cancer treatment is constantly evolving. You've seen the differences in risks among patients treated through those different eras of treatment and recommendations need to change to reflect our new knowledge. They are constantly changing and being updated. And I'll speak in a moment about the Children's oncology group late effects guidelines which are always being revised and updated as time goes on. And also it's worth mentioning that we have all these new agents now, these new ways of treating Things that we that didn't exist five and 10 and 20 years ago, the story has yet to be written about the late effects of those new agents because they haven't been around long enough. Also, Children grow up and so there is inevitably a need to transition from the pediatric to the adult health care setting as well as to transition from the realm of shell of oncology follow up to the primary care arena for their subsequent follow up because these patients are not going to keep seeing an oncologist, especially not a pediatric oncologist for the rest of their lives. And as they grow up, these Children who are no longer Children need to start taking responsibility for their personal health. So because of all of that In 2003, the Institute of Medicine set out a landmark report with a set of recommendations to improve care and quality of life for childhood cancer survivors. They called for the development of evidence-based clinical practice guidelines for us to define a set of minimum standards and for the establishment of programs in all pediatric oncology centers and then to evaluate the different models of care. They called to improve awareness of late effects among survivors and their families and to improve education and training for both specialists and for primary care providers, which is part of why I'm giving this talk. They also called for dedication of government and private resources to ensure access to care for survivors. And I'm sorry to say that we're not all the way there yet. And there is still need for more investment and for a call to and they called for an increased research to prevent and to ameliorate and to study these late effects. This report served as a call to arms for the creation of survivorship clinics across the country. Overall, the goals of long term follow up can be summarized here right? Our goals, our education for the patient and family and for health care providers who are also involved for surveillance with appropriate screen but targeted screening tests and comprehensive history and physical at the time of your long term follow up assessment, coordination of care with communication between providers, documentation of that care and support for transition of care as well as support with with psychosocial services and with financial and insurance issues and continued research. These are the goals of our long term follow up clinic. The first set of goals here, I should have said get summarized in these two really important key documents the cancer treatment summary and the survivor care plan. These should be portable documents in the patient's medical record that the patient then can take with them as they transition across different places or between providers, especially in the primary care and the adult health care arena. Unfortunately of course there exists and barriers to proper long term follow up and a study. This is in 2000 and four evaluated patients and found that fewer percent of adult survivors of childhood cancer reported having cancer related follow up within the prior two years, Fewer than 20% reported having been counseled on risk reduction and screening tests. The risk factors for failure to follow up include things like increasing patient age, increased time from diagnosis patients race, lack of insurance, distance from a treatment center and socioeconomic status. All played a role. A few more recent studies found that this continues to be a problem. A. C. C. S. S. Study found poor adherence with the Children's oncology group that's called long term follow up. That's L. T. F. U. Uh surveillance guidelines. Um They looked at um what survivors in the C. C. S. S. Studied report reported in terms of compliance with screening recommendations for these particular things. And the results were pretty dismal. Only 12.5% reported complying with breast cancer screening guidelines. 37% with colon cancer screening guidelines, 22% with skin cancer screening guidelines. In less than half with cardiovascular screening. Only 27% of survivors and even fewer of their primary care providers actually had access to a survivorship care plan, possession of one of those survivorship care plans was associated with higher rates of compliance with those guidelines. We took a look at this within survivors seen in our own clinic here in Oakland and we found that just 41% of adolescent and young adult-age survivors who were seen with the intention of transitioning to primary care for their subsequent follow up. Actually successfully made that transition within a year and a half after their last follow up with us. There are a number of different models of survivorship care. I'm not going to go through the details of this but suffice it to say that there are as many different ways to structure a survivorship program as there are pediatric oncology centers and academic medical centers that treat childhood cancer around the country. All of them though should have a few key things in common. They should be multidisciplinary and ideally will include the involvement of a psychologist the social worker, some research and nursing staff as well as integration or at least accessibility of things like nutritionists, geneticists and endocrinologists to follow up for some of the common the most common survivorship issues, the goals of all of these problems. I listed in a previous slide, late effects, surveillance and management education for patients and caregivers in their physicians, psychosocial support and contribution to survivorship research. All patients who come through a long term follow up programs should receive the comprehensive treatment summary and a survivorship care plan. I mentioned this a little bit earlier, the Children's oncology Group of guidelines. This was developed by the C. O. G. In response to that landmark Institute of Medicine report in 2003 and they got on the ball quick. Later that same year. In 2003 they issued their first set of guidelines. These are evidence based graded recommendations Grouped by treatment exposure and organ system. They're designed for the surveillance of late effects in survivors at least two years from the end of their treatment. The most recent version is version five that came out in November of 2018 and these are continually being revised and updated with major updates happening about every 5, 4-5 years. These also include a set of what they call health links for patient and family education written in easy to understand language, there is a public website so you don't have to be a C O. G. Member to go to this website. You can google it, you can find it here survivorship guidelines dot org to integrate these or at least to make reference to them in your practice. This is a snapshot that didn't reproduce really well. It's just a screenshot from one of the pages from the cognitive effects guidelines to give you a sense of it. So this is from the chemotherapy section. There's also radiation and surgery sections. This is in the section on anthro cycling drugs focusing on cardiac toxicity here. You can see the different anthro cycling agents. They include a dose conversion because things like donna rubies center about half as cardio toxic as dark. So Robison and drugs like Mido's antron are 4 to 10 times more cardio toxic. So to standardize it you can do this um conversion. This shows you a little table here for how often patients are recommended to get a screening echocardiogram based on a combination of the radiation they may or may not have gotten and the cumulative dose of anthrax cycling's that they got because these C. O. G. Guidelines are a little complicated and sometimes difficult to implement in real life. The C. O. G. Collaborated with texas Children's hospital to create this website called passport for care. Initially this was designed for people who run survivorship programs to plug in the information for a given survivor and it then kind of spits out these nice little treatment summaries and survivorship care plans based on the C. O. G. Late effects guidelines. The and and they're updated in real time as the C. E. O. G. Updates their guidelines. They also created a side of the website of public site for the survivors themselves where these documents and the associated things like help links and other references are securely archived. They go in, they log in with a username and password and then they can access these and print them out anytime they want. So how does our program work at the UCSF Benioff Children's hospitals here in Oakland? Uh And also across the bay from where I am in SAn Francisco what do we do? We provide really a consultation service mainly for internal but also for external referrals as well as for patients self referrals. And we welcome those external referrals because they're very important. Most of the time though our patients are being continually followed for long term follow up by their own primary oncologist within our own institution. But at some point those primary oncologists will choose to refer them and send them our way for a special visit to our special survivorship clinic. Um And they often do that around the time that they're anticipating a need for a transition um but not exclusively and there's a variety of reasons that they may want to refer them earlier ideally we would love for all of our survivors to be referred and come through our program. What do we do when they come? They get a comprehensive history and physical. Of course they get seen by our multidisciplinary team which includes an oncologist, a social worker, a psychologist and a nutritionist all embedded within our clinic, all of whom independently see the patient. Um And uh we then can make referrals to other specialists as needed depending on the particular situation. We focus on education and then creating this documentation. Everybody leaves with some form of a cancer treatment summary and a survivorship care plan here in Oakland. We provide them access to the passport for care and its associated documents and in san Francisco. Doctor Goldsby has long since created his own version of what they call a passport, which is this little wallet sized card that summarizes all the relevant treatment exposures and history as well as the survivorship care plan. Uh and in development is going to be an iphone app version of that passport. So I'm excited to start implementing that. We also review and order uh order and review relevant screening tests. We provide coordination of care between any referrals that are needed primary care and follow up. And of course we're continually involved in various research projects. As I said, we often tend to focus on adolescent and young adult aged patients who are approaching the age when they may be ready to transition both from oncology to primary care. And also often because it happens around the same time from pediatric to the adult health care arena. But we don't mandate that they make that transition and we have no set age where we're going to kick the patients out. So after we see them in survivorship, these young adult patients may or may not feel ready to make that move. And if they don't, that's okay, they'll continue to get followed annually by their primary care provider as well as by their primary oncologist until they feel ready to make the transition. But by then we've set the stage. They're invited and welcome to come back periodically, perhaps every five years for some patients will come by when they're younger, more for the benefit of their patients of their parents and then again, come by later when they're older and more ready to start taking ownership for their own care and going to start making that transition one way or another, most patients will sooner or later graduate from pediatric oncology to follow up ultimately with their primary care provider. So that's where you guys come in. Primary care is essential to the proper long term follow up of our survivors, patients may also need to transition from the pediatric arena to the adult to adult oriented care. And so it's important for pediatricians to help facilitate that transition if it hasn't already happened to our oncology, survivors. Um, it's really important and I emphasize these in our survivorship clinic visits that are young adult survivors or adolescent survivors have their annual health care maintenance visits, which is otherwise all too easy right to neglect and not do it if you're feeling fine. But it's especially important for our survivors to stay on top of it. A history and physical turns out to be adequate screening for the vast majority of the potential late effects for our patients. And it can be targeted based on the particular exposures and risks and it should be documented and laid out in their survivorship care plan. Other screening studies will be based on their exposure and their risks with Echocardiograms, for example, every 2-5 years for those who were exposed to anthrax, cycling's or chest radiation or both Patients who had head or neck radiation need to get thyroid function tested annually. And patients who had Chester abdominal radiation will be eligible for enhanced early screening for breast and colon cancer starting at age 35 ish, depending on the particular situation. And of course, as I said, coordination of care from the primary care providers, survivorship unfortunately, still has a number of unmet needs. These clinics require a lot of time and resources and reimbursement from the patient visits. Just doesn't cover all those expenses, including things like services from the psychologists, the nutritionist, the social worker, the research and clinic coordinators and the time it takes for medical record review. All of these things are un or under reimbursed and institutional support to make up the gap is really inadequate. So most programs like ours rely on charitable contributions in order to survive both from individual donors, from organizations like the braddock Family Foundation, which has helped found and continue to support our braddock emotional support team and organizations like swim across America, which has graciously donated for years. Support from their events to Benny Off Children's hospitals, Oakland and here is a shameless plug to swim across America because their annual event is coming up. Their san Francisco Bay open water swim is coming up in early october, check out the website if you like to swim, you can register, you can join a team if you don't like to swim, you can donate to one of the teams. There are teams representing both the East Bay and the West Bay as well as particular individuals. Um and it's a tremendous event. If I've got time, I don't have time, I'd like to just circle back to our case. Um and then I'll quickly wrap up and allow a little bit of time for questions. So this 15 year old female who came to establish care who had leukemia at the age of two and that's about all you know about her except that she also has epilepsy. So what did you want to know? This is based on a real patient we saw in our survivorship clinic sort of a typical case she was diagnosed with B. A. L. L. At 21 months old. She was treated per this Children's cancer group old ish now study 1961 protocol. These are the total of all of her chemotherapy exposures. In addition she got prophylactic cranial radiation which was our standard of care at the time totaling 1200 centigrade. She remains in first remission. She completed therapy 11 years prior to her survivorship clinic visit the active late effects that we currently know about include clinical leuco encephalopathy with epilepsy as well as some cognitive deficits related to her radiation and or her in tropical chemotherapy. That's what she's got now. Here is a list of the things that she is at risk for in alphabetical order problems with her bladder resulting from cyclophosphamide exposure, cardiac problems from her aunt recycling exposure, dental problems from chemotherapy and childhood in general endocrine problems related to her cranial radiation. Hepatic problems related to some of the chemotherapy, musculoskeletal problems from steroid exposure, cataracts as well from steroid exposure, peripheral neuropathy from vin, Christine exposure, reproductive problems from uh cyclophosphamide and second malignancies from radiation and from some of the chemotherapy as well. Here's what her survivorship care plan calls for in a nutshell echocardiograms every five years. Annual assessment of her thyroid function baseline laboratories including a blood count metabolic panel vitamin D. And then you don't have to do an annual blood count forever and ever. And then you can do them as clinically indicated as well as a bone density test which is recommended by the Children's oncology group for screening for um osteoporosis or osteopenia. Though it's not a particularly strong recommendation of mine personally follow up should include annual history and physical with primary care. Annual follow up with pediatric oncology until this patient feels ready to make that transition as well as continued follow up with neurology, regular dental and eye exams. In conclusion. Well in conclusion cancer sucks and it continues to suck even after it's gone and done. Survival is insufficient. This is a quotation I stole from a wonderful novel by one of my favorite authors. Emily ST john Mandel. I think it encapsulates a lot about the survivorship field. It's not enough just to have survived. Long term follow up is important. No your patient's treatment, cancer treatment history and survivor care plan, refer them to our survivorship program if you don't have them screen them for relevant late effects as for the C. O. G. Guidelines. And thank you for listening and for doing so. Here is a link to some uh important resources including the C. O. G. Late effects guidelines and also including the swim across America website. This is our enormous team of hematologist oncologists and transplant specialists in san Francisco and in Oakland and in our satellite locations and here's how to refer a patient to UCSFF
