The broadcast is now starting. All attendees are in listen-only mode. Good afternoon, I am Ian Schreier and it is my pleasure to welcome you to ASPMN's ninth year series of webinars that focus on the use of opioid therapies for the treatment of pain, opioid dependence and on the safe use of opioids in the treatment of chronic pain. This series is one of the many resources made available by the Prescribers Clinical Support System. This is a program that is funded by the Substance Abuse and Mental Health Services Administration. It is a collaborative project led by the American Academy of Addiction Psychiatry and a number of professional organizations. On the last slide, you will find a list of these organizations. You are able to obtain nursing continuing education credits for this presentation. You need to participate through the question and answer period. Detailed instructions concerning the CE credits will be given at the end of the presentation. 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The slides will also be available through the PCSS website in the near future. And that website is www.pcssnow.org. Today's presentation is When Too Much Is Too Much, Reducing Opioid Use Through Multimodal Pain Management for Young People with Sickle Cell Disease. Our speaker today is Jim DeMasi. He has had the privilege of working with young people with hematological and oncological problems for the past 25 years. He especially enjoys taking care of patients with sickle cell disease given their challenges and unique pathophysiology. Jim has worked in managing chronic pain and he continues to work closely with his colleagues at Children's Health in Dallas, trying to find novel approaches for caring for these children. Jim received his BSN from the University of Texas and an MSN from UCLA. He received his post-master's certificate in acute care from the University of Texas. Welcome, Jim. Hi, Anne. Thank you for that nice introduction. And I really appreciate all of you coming today to discuss this very unique population that's near and dear to my heart. Can you guys hear me okay? Anne, can you hear me? Yes, we can hear you fine, Jim. Thank you, Anne. Uh-oh, hold on one second. Now I've got some slide issues here. Give me one sec. There we go. Great. So again, I just wanna thank all of you for coming today and say good morning to those of us out West as well. I'm here in Texas where it's icy and snowing today. I am gonna discuss sickle cell disease. And at the end of the activity, I'm hoping that you all have a better understanding of the Five A's tool, which is how we determine if opioids are working or not in young people with sickle cell disease. I'm gonna discuss the etiologies for worsening pain and function in adolescents with sickle cell disease. Some ethical considerations, assumptions, and biases we see pretty often in our population and how that pain management's impacted what we do for that chronic pain patient in our multimodal approach at Children's Hospital and then end it with a case study in the interest of time. You know, I put these slides together to just basically talk for about 40 minutes or so. So we have some, and I hope to learn from you all today. I could tell you I'm not a substance abuse expert by any means, but I hope I can learn from you all as well today because we do have some substance abuse problems sometimes. As far as disclosures, I have no financial or personal disclosures. This slide I put here is what red blood cells look like. They should be round, they should be concave, they should be carrying oxygen. And for kids with sickle cell disease, their cells are just not round. And I like to use the analogy with the young sickle cell disease patient that your cells in your body, your red cells, should be round like wheels on a car. And for reasons that we don't always know the reasons why, your wheels are not round. And so it makes things sometimes difficult. And that's just something we try to keep developmentally appropriate so they understand. So as far as the epidemiology of chronic pain in young kids, we know that by age 18, more than half of those patients experience pain more than half the day. So it's 15 out of 30 days a month. And so that's a lot. And then by, you know, in that same age group, about 30% experience pain on all of the days. So really a lot of people with sickle cell disease adolescents, only about 15% are pain-free on greater than 15 days, if you look at that statistic there. The true prevalence of chronic pain is likely underappreciated because most patients manage their pain at home. They're not in the hospital. Contrary to what we think, a lot of these kids are just, you know, kind of managing things with their nurse and doctor in an outpatient clinic setting. These young kids will often seek acute care for their underlying chronic condition. It's difficult to differentiate between like a vaso-occlusive crisis versus, you know, just their chronic pain condition. And sickle cell disease, unfortunately, has no lab marker to help with its distinction. So if you look at a child with sickle cell disease or a person with sickle cell disease, you cannot, there's no differentiation. We look at hemoglobin, space-line hemoglobin and retic counts, but really that's just, you know, kind of a benchmark. Chronic pain is often associated with comorbid psychiatric illnesses such as depression, anxiety. And a lot of times there's challenges to getting good sleep with pain. Some kids will have somatic symptoms. Chronic fatigue will often impair their quality of life. And so it's our job to help tease some of those things out. And so at Children's Health, we have about 400 hematology referrals a year. And by, with that number, that's a lot of hematology referrals. We are one of the busiest centers in the United States. And so about 10 to 15%, and even, I think it's about 20% actually is the number are sickle cell disease patients. And we've identified with our large group of patients about 20 to 30 kids a year, which we consider a very small number of patients, but they are frequently admitted for pain that is refractory to standard treatment with poor outcomes. Whatever we do for these kids, their pain is always, again, we're talking about that 95%, or I'm sorry, talking about that group of kids who has pain on 95% of their days. So whether we give them a lot of opioids or we rotate opioids, or we give them IV versus PO, their pain is kind of always the same. Because of these frequent and prolonged admissions, they're not in school, their baseline functionality is not great. Their perception of not having their pain well-managed can escalate and cause some verbal abuse of staff. We've had some physical throwing of objects and calling security and then a resultant noncompliance with therapies, because again, there's just not a lot of buy-in to the plan. It's been made worse with the COVID-19 pandemic due to the reasons cited there. And again, just harping on this lack of objective method to verify pain makes things that much more difficult. So you can see here in the illustration in the lower right-hand corner, a healthy red cell. If the cells are those wheels I'm talking about, then blood flow is unrestricted and life is good. But when a patient has sickle cell disease, a lot of their cells in the circulatory system make a sickle-like appearance, kind of a half moon, and so they can get occluded. Effects function wherever blood circulates. And so there's a slide later on, I'll talk about this, but one of these things is pain. The five A's of analgesia and talking about the objective. So I got this slide off an Australian website and the five A's include activity, analgesia, adverse effects, aberrant behaviors, or affect. In the chronic pain clinic, we like to base things on functionality, not getting the pain score to zero out of 10. And so we ask a variety of questions during our appointments in acting about activity. Are you able to function? Are you able to go to school? Can you walk up and down the stairs? Can you participate in athletics? Are you able to drive? Are you able to do the things that all normal teenagers can? And again, I don't like to use the word normal versus abnormal, but I think you all know what I'm talking about there. Are you able to do what a teenager who does not have sickle cell disease can do? Are you able to sleep? Because if your pain is so bad, you're not able to sleep. You know, are you doing well in school? Are you able to pay attention to those types of things? Analgesia, does what we are doing, does it work for your pain or not? Adverse effects, you know, are you sedated? Are you constipated? A lot of the medicines that we use will cause the kids to be somnolent. And there's often undesired side effects, constipation, especially with opioids. SSRIs, certain medicines can cause weight gain. Lexapro, amitriptyline, some can cause you to lose weight. Topamax, for example. Aberrant behaviors, these are just behaviors that are out of the ordinary, which, you know, kind of clue in the provider if maybe there is a potential issue. And again, I know that most of you can teach what aberrant behaviors are, given the audience that we have here, but sometimes we, as parents and providers, have difficulty identifying these behaviors. And then affect, you'll see that as a recurring theme in my slide, that if you have a poor mood and untreated anxiety and depression, poor compliance, it can certainly lead to poor pain control. So as far as etiologies of worsening pain and deteriorating function, there's many things that go on and many things that play a role here. I'm gonna talk about these individually, but we also have coupled COVID-19. So we're in the world of COVID-19 and the pandemic has just made things worse for everyone. So you can imagine these patients that already have sometimes difficulties getting in and out of the healthcare system if there's no beds available and these types of things, how COVID-19 has affected things, health insurance and whatnot. Loss of resources, lost job, lost time, not able to maybe get admitted to the hospital, again, the bed situation has just been really bad in Dallas, although things are improving now, but the last two years for my wonderful patients here has just been difficult. The next one, provider biases and racial disparities. This has been an ongoing issue for the last 25 years. I've taken care of kids with sickle cell disease. I'm not gonna spend a whole lot of time on that. All my slides jammed up again, I'm sorry. Then practitioner biases and an opioid crisis. So with the opioid crisis, we kind of made a pendulum shift over the last 10 or 12 years in providing opioids to these kiddos, but that piece is slowly getting better. But again, it does factor into how that patient's pain experience has been. So as far as genotype within the environment and sickle cell disease, so dependent on the type of genotype that the patient has or mutation that the child has on their hemoglobin gene will affect their clinical features of their disease. We do know that the mutation for sickle cell disease occurs on chromosome 11. And again, depending on what type they have, often explains the symptoms that they present with. I can tell you from experience that sickle cell hemoglobin C, certainly sickle beta thalassemia and then hemoglobin SS are our biggest issues with pain. That's not to say that the others don't have an issue. Certainly sickle cell trait can cause some hematuria. And I've heard about some stress induced, high impact activities with kids who have sickle cell trait that cause pain. But for the most part, we're talking about hemoglobin SS, SC and beta thal, sometimes some beta zero thalassemia. The environment can affect also the piece with sickle cell disease and with the phenotype, that's the ever changing set of characteristics that result from the interaction of its genotype with the environment. And so the things I talk about here with parents are diet, temperature, again, mood, sleep. Are you sleeping well? Medications, viral infections, and then oxygen concentrations. And so, again, how that person with sickle cell disease interacts with their environment dictates how much pain they're gonna have moving forward. Again, I know the slide is busy here, but it's just to kind of give you some information. Sickle cell disease pain with age, it increases over time. And so if you look at infancy in the toddler columns there on the left, the big piece here is that they have the presence of fetal hemoglobin. And so there's not a lot of sickling that occurs. However, you can have some of the splenic sequestration that you see with sickle cell disease and some of the vascular occlusion, some of the tissue is scheming. But for the most part, the inflammation, pain, some of that maladaptive neuroplasticity that occurs with pain occurs later in life. We do know that pain crises are more frequent and chronic pain occurs more likely with age. And so, again, the kids that I'm talking about here, yes, we see the five-year-olds. Yes, we see the eight-year-old. But for the most part, I'm talking about teenagers and above. Pain can be in one or more location. It could certainly be nonspecific and diffuse. And I often will hear the chief complaint of it's everywhere. Patients require an increased dose and duration of opioids. And let me just tell you, I don't know how things are in your centers, but we run the gamut on how we administer opioids. There is no policy on opioids, except to say we try to start with an oral route, usually with an NSAID and an oral, either oxycodone, HiSET for the kids who can't take pills, or Norco. And we schedule that. But things get kind of out of control quickly. If a patient is really uncomfortable, they'll use some IV medication quickly. And then 12 hours later, a morphine's not working, let's do Dilaudid. And then we have them on a PCA, and then the kid's on oxycodone plus Norco, and then we have to come in and try to clean things up. So again, things just kind of get out of hand quickly sometimes. Kids become increasingly refractory to opioids. They have a concept of opioid-induced hyperalgesia, which I'll talk about in a second. The pain burden increases with age, and is often associated with school absenteeism. So if we're not, and the other thing too, is as these kids get older, if we're not able to reverse some of these behaviors, we do know that they go on as adults to have chronic pain. This slide illustrates the multiple issues and clinical pathologic findings that we can see in kids with sickle cell disease. And this is not only my frequent admission patient, but this could be my one to two admissions a year. However, in my experience in pain management, when they call us in pain management, most of my kids are not just the one and done. I call them the one and done sickle cell disease admission, the kid who comes in maybe once or twice a year at the most. Again, my kids are coming in monthly, is this population that I'm talking about for purposes of our talk today. And so a lot of these kids will have ischemic, inflammatory, neuropathic complaints, and we try to differentiate with that with the way we ask the questions, with imaging and so forth. We have a lot of kids with bony infarcts, AVN and leg ulcers. We know that, again, there's not always an obvious anatomic source. We'll get pan imaging on a lot of these patients and we really even can't, we can't find a ton of things physiologically wrong with them. We do know MRI changes do show up in the brain when there is central nervous system issues and we'll sometimes see it. We have some kids with migraine problems and we have some certain, well, again, this is an inclusive process, but like transcranial Doppler imaging and so forth, but that's another topic. Hydroxyurea is more effective for acute pain crises. A lot of our kids cannot undergo a bone marrow transplant, but the only potential cure for sickle cell disease is a bone marrow transplant. And in our center, we try to find a matched sibling BMT. I know we're doing more unrelated donor transplants, but again, that's not without risk either. There's a higher risk of transplant related mortality. And again, if you're not able to take care of these issues, and again, a lot of these things here that, this is physiologic. I'm talking about chronic pain in the setting of, we can't always find things here that respond to our therapies. So factors associated with the risk of developing chronic pain, this slide kind of reads from left to right. And again, we know that basal occlusive pain or that sickling that occurs with those half-moons in those blood vessels, are recurrent episodes of hypoxic and ischemic reperfusion injury that can cause damage to bones and tissues. This chronic process results in inflammation, tissue damage, and then this repetitive nociceptive injury. And so you have receptors all over your body called nociceptors where you feel pain. And so if these are constantly being exposed to pain and so forth, there's a process that goes on called central sensitization, and there's an increased responsiveness of nociceptors to normal pain and non-painful stimuli. And so, you know, again, and for those who are better experts with brain piece than I, we call it like a maladaptive neuroplasticity where there's some hypersensitivity to pain both which can cause allodynia and hyperalgesia. And so you have hyperalgesia, which is an increased pain perception that's not in proportion to what's going on with the noxious stimuli. So these patients have this increased high, high pain, and it might not be the most, like I said, it's an increased pain perception. But to me, it's pain, so we're just going to treat the pain. Allodynia occurs, you know, you have pain from a stimulus that does not cause pain such as light touch, pressure, heat, or cold. So I've had patients, you know, explain this as if I rubbed a feather on the body or certain parts, it's extremely painful, it burns, and don't do that. I've had patients who can't wear certain clothes because the material from the clothes causes so much pain, so it's really hard to see. Opioid-induced hyperalgesia, this is a paradoxic reaction. We think to chronic opioids and the long-term opioid that you're giving to help with the pain actually can cause some generalized pain. And in neuropathy, we just see a lot of pain caused by damage and heightened, you know, response within the nervous system. The central sensitization also occurs, we think, when we stop the opioids too quickly. And, you know, again, these kids are coming in for long periods of time, they're given large amounts of opioid, and then they, you know, basically stopped, kind of cold turkey, and then they're sent home, and then they wonder why four or five days they come back into the hospital having pain. And so we're really trying to change that because it's, you know, it's very much unrecognized by providers, patients, parents are like, oh, we're better, I want to go home, and then they just stop the medicines, and then five, seven days, they're later. And one of the ways in pediatrics we've helped reduce that is by sending kids home on methadone tapers. As far as phenotypes, I talked about that earlier, there's certain pre-existing, again, characteristics that we see, female, although I don't see that in my practice, mine's generally 50-50, boys and girls, genetics, early life trauma, that we do see. So if a child, you know, victim of abuse, I've had a few kids be in car accidents, for example. I've had one kid who witnessed the murder of a relative at a young age, and so that kid comes in a lot, and it's still talking about that, even 10 years after the fact, it's really quite sad. mood disturbances within the family, you know, again, poor sleeping, fatigue, catastrophizers, we talk about this with psychology, and then multimodal, I'll talk about that here in a few minutes, and then exposure to stress, or what is that person's behavioral response to pain or stress. So OIH, hyperalgesia, or OIH in sickle cell disease, on this slide, I'm not going to talk, but again, paradoxic reaction to chronic opioids, but again, we, in this study here from this 2016 paper from pain, the group had higher rates of clinical pain, so pain scores were higher, you know, again, they were admitted more for vaso-occlusive crises, they were found to have higher rates of anxiety, their mood was not good, and, you know, that was based on mood questionnaires, and I didn't, I don't exactly know how things were scored, but there were higher rates of anxiety and depression, and then also, you know, MDD as well, major depressive disorder, and then sleep, and that keeps coming up, if kids don't sleep, it just makes your pain worse. So pseudo-addiction is a niatrogenic syndrome of abnormal behavior developing as a consequence of inadequate pain management. This niatrogenic is, you know, a result of what we do to the kiddos, and we give them large amounts of, you know, analgesics to help meet the pain stimulus, but then it really doesn't work very well, and then we'll, you know, escalate those, that amount based on what the patient's asking for, the parent trying to reduce the pain, but again, we're just kind of, it just doesn't work, and so, you know, it's like, okay, so we're going to give them 20 milligrams a day of morphine, you know, for this, what's your score, 10 out of 10, then we give them 40 the following day, what are you, 10 out of 10, and then 100 the following day, 10 out of 10, and so this inadequacy of pain management then leads to anger and frustration, and you can see this whole cyclical little illustration here of, you know, and just mistrust between the patient and the healthcare team, and so, and again, I always find that the same things are trying to be done to fix the problem, which is just, let's just dump more opioid into the child and see if that works. Twenty-five years ago when I started, that's exactly what we did, and these kids would be, number one, overly sedated, they would spend four to seven days in the hospital, they would, you know, not be able to, you know, work with physical therapy or get out of bed as well because, you know, they would just be so over sedated, and that just piece did not, it just didn't work out, and so we do things a lot different now in 2022 to try to help with that piece, and again, I think it's paramount to help, you know, minimize some of these issues. With sickle cell disease, I often hear that these kids, you know, are chemical copers and they're, you know, just dealing with life with drugs and they're all substance abuse, you know, potential legal, you name it, they have, and I will often present this slide to nurses, doctors, parents, and just say, you know, this is not the case. Maybe the kid is going through some withdrawal, maybe, certainly they have problems with tolerance, they're needing more drug to get the same desired effect, large amounts, you know, again, longer because they're in the hospital, maybe, maybe not the psychological problems related to use, but as far as craving and legal problems and neglecting major roles, I would say with the exception of not going to school, we really need to be careful when we talk about that, and you know, I would love to hear from some of the adult hematology people in the group, you know, do you guys have these problems with substance abuse, substance use disorders? So, you know, again, I was telling you, we send kids home with methadone calendars and tapers, and we've had a lot of pushback with that, and people think that, well, what are you going to do? Send these kids home with methadone and oxycodone for some breakthrough pain and gabapentin and, you know, this and that, and isn't that dangerous? Well, certainly, it's a concern, but, and I hear this a lot, overdose with sickle cell disease, that's pretty common, right? And it's just not, it's just a lot of, again, a lot of it is practitioner bias and people just not understanding this, and this slide basically is just illustrating that overdose deaths with people with sickle cell disease who are on chronic steroids is low. And in fact, and this is one paper, but I found a few that other people who are on opioids for other conditions, they have a higher, greater chance of overdosing compared to the child or person with sickle cell disease. I also love the fact that people get opioid, well, I don't love the fact, but the fact that people get opioids from migraines is just mind-boggling to me. The opioid epidemic and sickle cell disease dealt by association, so there is a negative attitude towards sickle cell disease and a fear of catering to an addiction. And this predates the opioid crisis, even in the 90s and early 2000s, there's this issue. They, a lot of people think that patients are, you know, addicted to, as a result of just undertreatment of their pain, and again, they're tolerant, but they're not addicted. It's just something that we always have to try to kind of, you know, re-educate. We do know that racial and ethnic minorities tend to be undertreated for pain historically anyway, so that's a problem. Two-thirds of nurses believe kids with, or I should say, patients with sickle cell disease are opioid abusers. I hear this a lot, especially in the ER, where over half, and again, this is a study from Pete's Blood and Cancer, so over half for ER, about a fourth of hematologists, and I just hear it in the way that people speak and the words they use, but I'm kind of sensitive to hearing it, and so I'm like, well, hold on, you know, and again, a lot of it is just trying to, you know, explain that it's not that simple. As practitioners, we should at least try to learn about the early signs of misuse. I could tell you in our population, however, hematology and oncology, misuse is low. You know, of the items here, cited lower-level behaviors, we do see sometimes people asking for early refills. You know, we see people asking for medicines by name and route, and that raises a lot of red flags with providers and says things like, well, who knows the dose of their medication, the route, and this and that? I could tell you, I know all the routes and the medicine and doses of my medications, so it's brought up a lot. I'm like, okay, so what? He knows he's on, you know, 10q6 of oxycodone PRM. Well, they're watching the clock, and they're doing this. He must be an addict. I'm like, look, again, when you're hurting, you hurt, and so you have a tendency to, you know, to do those types of things, so using medicines for non-pain purposes, you know, we get a lot of, well, I was tired and sleepy, and so I just took, you know, this oxycodone to help me sleep. You know, I said, well, look, that's not the reason, that's not the way the medicine, let's try to get you something else that's going to help with your sleep. I bolded here, though, again, is risk of aberrant behavior is, again, behavior is, again, for untreated mental health. We see that a fair amount. So, again, I just, again, it's really important that we have psychology, see our patients. It's important that we ask about anxiety, depression, you know, certainly PTSD, panics, you know, again, our kids in, with sickle cell disease, hematoma kids are at risk of the same thing that our non-HEMOC kids are at risk for. This slide just illustrates treatment decisions can be influenced by bias and emotion, you know, and again, I just, I ask these questions a lot during the talk, you know, are the kid, are the patient preferences for pain treatment given the highest priority versus what's best for the parent or practitioner or what have you? Does the patient benefit from these treatment decisions? Are the things I'm doing help? Am I decreasing harm and reducing harm? You know, am I, you know, am I being respectful of my patient without discrimination? These are comments here. I just want you guys to see that I have heard the, again, I'm not going to go over all of them, but the first one is an African-American girl who had a splenectomy and just was given oral pain meds after surgery, and she asked me this question after I saw her because she was in terrible pain afterward. I hear a lot about, you know, faking it, malingering and all that kind of stuff. Again, I just, again, it's just an awareness. God knows what else they're using. I'm so tired of taking care of these people. What does that mean? Especially in our current environment, you know, words are important. I know I have about five minutes here, so I'm going to move quickly here. So Children's Standard of Care, so it is a multi-modal, multidisciplinary group. We have Friday Clinic. It's a four-hour appointment, so we, and I'll tell you about who's there, does promote self-management, and we're all about empowerment and motivational interviewing, and our goal is not to get your pain to zero, but our goal is to maximize function, and sometimes it's 15 things they can't do, and we talk about two to four things because it gets overwhelming, and then we have follow-up appointments, but I could tell you our multi-modal plan and our multi-modal treatment and management has really been successful. When we have the 30 and 60 and 90-day questionnaire, it's usually at least 55 to 70 percent saying this has been really helpful. We spend a lot of time, again, time is the key there, educating the child and the family about pain and their pain problem, and again, we really focus on one or two things, pain, sleep, maybe anxiety, depression. You know, hey, you can't go to school all day for eight hours, but what about if going, you know, for four hours? We base goals, they're very measurable goals, you know, if it's the middle of the summer, why don't we get you back to school by the start of the new year? If it's March when they come in, we're like, hey, why don't we have a goal of having you return to school in the fall? I mean, everything is very objective and developmentally appropriate. The family is certainly involved, the parents are encouraged to come, there's a physician, there's the NP or PA. We have three nurse practitioners, one PA, and then certainly our nursing staff is just wonderful. Psychology meets with the patient, physical therapy meets with the patient, so every person or every subspecialty meets with the patient. First, we get the history with the nurse and the scribe, and then there's the physician who goes in and gets kind of the, you know, that piece that I just talked about. Psychology meets with them, social work's only brought in kind of PRN or is needed if there's a problem identified. Man, I, you know, I can't help them with this pain because I have a transportation issue or so forth. Physical therapy, you know, where is that pain and what are some exercises we can do? And we'll often, you know, get them an HEP, which is a home exercise plan, or these are things you can do, or they will just do physical therapy either in the outpatient setting or they'll come to the home. Our team approach is individualized, and it's a rehab focus with active patient participation. You know, the first thing we normally do in this bullet point four is acknowledge patient family concerns and fears and understand that we're listening to you. You know, we're not trying to minimize, we're not trying to, we don't have our own agenda. You tell me what you need from us, and I think a lot of that is just kind of that team multidisciplinary approach that all of us in our group is very good at. And again, you know, I know it's time limiting today, but if you have any questions of how we do things, just please call or email me. Lastly is a case study. Again, you all could read here, so, but I had a 17-year-old deaf female kiddo with sickle cell disease, and she relocated, I think, from northern California, and she was followed in our service 2019 to the present. Ten admissions, eight ED visits. We get a lot of calls from this kiddo. She does have AVN of her hips, right knee issues as well, long bones back. Wasn't going to school, wasn't hanging out with her friends, had a new dog that she said was young and dumb, kept keeping her up at night, anxiety and depression from being concerned that she's not going to, you know, get better. She has a dad that's also deaf, some aberrant behaviors going on, noncompliance, but, you know, and you can kind of see there, she was having issues with sedation, constipation. Family was angry, but I don't know if they were angry. It's just kind of an expressive piece of, you know, just being, you know, hearing challenged, but we were able to get an interpreter, and we hired an interpreter, and man, that helped a lot. The other thing I wanted to talk about, we were doing high-dose morphine, hydromorphone, PCA, methadone, you know, her MME, man, it was a lot. It was a lot, but no matter what we did, it just did not work. I think we finally started using some ketamine, and that kind of settled her down. We did a care conference. We're the family and a large group of all of us with these sub specialties here. We did talk to our friends in adolescent addictive psychiatry, because we were worried about a substance abuse problem. She was taking medicines for what they weren't prescribed. She was testing positive for marijuana, and so, and again, this revolving door admission. She kept going home, and then she'd be readmitted. We'd give her more medicines. We would stop them quickly. We might or might not do methadone. We might or might not do some ketamine. I think we even did some low-dose Presidex on her, and again, this whole central sensitization piece was just going on with her, and we just could not get things under control. You know, and again, I'm just kind of the messenger here, but we did determine that she did not have an opioid misuse disorder, but that her chronic exposure to chronic opioids did attribute to a lot of her central sensitization, and that the risks of opioids did outweigh the benefits. And so we put her on a 14-day, which I think I ended up changing to 21 days of methadone. And then we agreed as a group to do an opioid washout period. And again, please, if I'm misspeaking here, I would love for this washout period. We decided that we were gonna do 45 days with no opioids, and we were gonna just do opioid-sparing medications only. And not only did it work, she did come back to the hospital for like three months. She did go back on hydroxyurea. She was compliant with her medicines. We put her on gabapentin. We were doing drug levels on that. We put her back on her vitamin D. She no longer tested positive for any drugs of abuse, with the, again, exception of the methadone. Let's see, what else did we do here? Oh, and then we began transitioning to the adult program, which we do early, which is very good. That way the kids don't fall through the cracks when they turn 18 years of age. She remained off opioids for a period of three months, didn't come back to the hospital. She was very compliant with her medications. She only gets typically tramadol, which is a PO, kind of moderate opioid. She does have significant social anxiety disorder. She did agree to go on an SSRI. She is really doing well. Her and her dog are friends now. We ended up talking to her about getting a crate for her dog, which is like, oh yeah, that's smart. So again, wrapping up here, it's upstream management. So we call these unique disease patients. So whenever they come into the hospital, we identify them. Typically they have to come in four to five times a year, and we'll add them to our list. And so when they come in, we have a treatment order set that gets flagged, we get consulted, and we try to do consistent pain management for these kiddos. We are big in the schedules, focusing on function. You gotta be out of bed. Just because you're in the hospital doesn't mean you're in a hotel. Children's health, you gotta get up, you gotta do PT, you gotta do something. And I think that's kept the kids out of the hospital a lot because they're held accountable. Hospital's just not as fun as it used to be. Medication management, opioid sparing medications, such as SSRIs and things like that. A lot of kids were non-compliant with their hydroxyurea, so I just reach out to our hematology cohorts, and I'm like, can we get this started, or at least get the conversation started? And if we do identify a problem, sometimes I just call Dr. Atkinson in addiction, psychiatry addiction medicine, and he's just so wonderful, and it will go through criteria and stuff, because I am not an expert on that. We do a lot of trout fishing, this is my friend Molly, she's our PA in Oklahoma. So schedules are big, and you can see it's not a schedule of fun. Real quick, drugs that we use, so hydroxyurea, we do know that using it and being compliant decreases the frequency of phasal occlusive events, acute chest syndrome. Again, improves quality of life, decreased morbidity and mortality, decreased opioid consumption during crises, and again, this is huge, decreased length of hospital stay by two days on average. So again, a lot of the chronic pain kids that I follow are here for five to seven days, but three to five when they're on hydroxyurea, and that's pretty square on. Gabapentin, if your kiddos are not on gabapentin, this targets, this is an opioid sparing pathway, this targets the gabapentin pathway, really works well in helping, especially nociceptive and neuropathic components, it has some anxiolytic properties as well, so it's a real good drug that we use a lot of. If gabapentin doesn't work, kid doesn't tolerate it, whatever, we use a lot of pregabalin as well. Ketamine, so a lot of times we'll do methadone, which is an NMDA receptor antagonist, and so they'll go from ketamine to methadone, we'll send them home on a methadone taper. Ketamine works really well, you could turn it off, a half-life is quick, and we have a ketamine intractable pain policy that we wrote, and it works really well. Decisions to use the medication vary, and I can talk to you about that. Presidex, we do, so again, Presidex, which is a selective alpha-2 adrenergic agonist, works nice in central nervous system to help kind of slow things down, and decrease opioid requirements, again, we usually use this in conjunction with some of our other analgesics, really makes kids sleep, it really mirrors natural sleep, well, great, great medication, if our nursing staff lets us use it. So they get concerned about using these things on the floor. Methadone, methadone, methadone's a big medication we use, we use a lot of Lexapro, which is an SSRI, we use a lot of Prozac, SNRIs, such as duloxetine to help with chronic pain, we use Efexor to Pyramate, especially with migraine comorbidity, naloxone we use less so, because we just don't understand the medication, so subloxone is a big one that is in the adult population, and is a great paper for those of you who are interested in reading, we use no subloxone in pediatrics, and I would love to hear from you separately, or after this, about your experiences with it, and then chrysanthelizumab is a monoclonal antibody, I think in older teenagers that hematology uses. Thank you all so much for listening, I know this is a little unorthodox with the webinar, this is our team here at a function, here's me, and my attending Dr. Spain, wonderful, and this is our pain management team, our psychologists, some of our PA, NP, our licensed family therapists, my directors here, and that's all I have, thank you for letting me speak to you, and if anyone is stressed or anxious, I recommend going to Yellowstone, this is Montana, it's beautiful, and I'm gonna turn it back over to Ms. Anne. Thank you, Jim, for that presentation, very informative. We do have some time for questions, and you can still go ahead and ask those questions while I start talking about it. One of the things that some of the viewers wanted to know was about the methadone, how you actually do the tapering, do you start them on the methadone while they're in the hospital, and how fast is the tapering usually? Yeah, that's a great, great question, Anne. So typically, the methadone is used right around hospital day three to five, and it's generally about 72 to 96 hours after we have good morphine or hydromorphone equivalent data, and what we'll do is start them on equal analgesic dosing of the morphine or hydromorphone that they use from the previous 24 hours. The other way we do it is if maybe they're getting PRN, morphine, or we'll just start them at kind of not an arbitrary dose, but we'll start them at the methadone starting dose, which would be 0.1 milligram per kilo, PO, Q8 hours, and then we'll just wean by 2.5 milligrams per dose every day until off, and again, depending on the severity of pain, how much the morphine or hydromorphone they use, or even how much methadone they need to get pain relief, kind of dictates how long our taper's gonna be, anywhere from 14 to 28 days. Does that answer your question? Yeah, I think somebody just typed in the methadone is a slow taper to prevent withdrawal over a period of several weeks at their addiction clinic to months, depending on that situation. As a person from addiction clinic, so. Yeah, see, so that's, I really wanna, I don't know how to get ahold of that person, but can you please, can you give me a call? Well, what I'm gonna do is somebody asked for you to give your contact information, and if you could put your contact information, we'll put it up with the slides and the webinar, and then they could contact you. So Cecilia, go ahead and please contact Jim. Yeah, because. Have a conversation about that. I was gonna say, just because we do it that way doesn't necessarily mean it's the right thing, and that's why I, you know, should we do things 30 days? Should we do things longer? You know, because, I mean, we have kids who will be on that 21-day wean and go, this is what's keeping me out of the hospital. Why are you stopping me on this methadone? And I don't always have a good answer for them, and so I'd be interested to see what Cecilia or any of the other listeners have to say about that, because, yes, we have to, you know, monitor them more closely, and yes, but again, if it's gonna help them, what's the downside and drawback to that? And maybe, you know, I don't know, tolerance or maybe it's not a good idea. Right. There was another question, and I think it brought up something in mine, too, is that you talked to, about the case where you had the, young lady was taking some marijuana. So do you regularly do urine drug screens to find that out, or is that self-report, or? Yeah, so the answer, that's a good question, Anne. So the answer is no. We do not do a lot of drug urine screening. This was reported to me, and so, and again, we, because again, I don't know what I'm gonna do with the information. Yeah, so, yeah, I'm kind of at a loss of words what to say here, because, yeah, I mean, we're only gonna do the wean, typically for seven to 10 days. It just doesn't come up very often in pediatrics. Yeah. Yeah. Well, I wonder, you know, now, it just gives a thought to me that there are a lot of places in the country where marijuana is legal, but not necessarily for children, but they still could have much more access to it. So it would be a good question about how they were doing that and how it would affect moods. Yeah, the, we are doing heads, you know, assessments on all the patients, usually during, you know, our interview in the chronic pain clinic, and you'd be surprised how rarely that answer of positive for marijuana use comes up. It doesn't come up very often. For kids with sickle cell disease, but again, it could be, you know, maybe not being truthful or what have you, but. Right. Yeah, it's hard to know. My migraine patients in pain clinic, oh, yeah, they're all, there's a lot of use of marijuana, especially with anxiety and so forth. But that's a good point. I'm really glad about that. Can we change the topic a little bit so we can answer some of these questions? Somebody was curious to know about if you have used buprenorphine for pain control in this population. I have never used buprenorphine in our population, and we want to. We want to, but, you know, again, and let me just say some of the things I was talking about with ketamine and the Presidex and some of these novel approaches, these are new in our center. It's not to say they're not being done in your centers, but buprenorphine is not something we currently do, and I really want to study that. I just don't know really where to start. And I think that cohort of patients, that group, it might be a small number, but it's our next project. So bloxone and buprenorphine, but no is the answer to your question. Okay, and are you using PO ketamine, or is it strictly the IV ketamine while hospitalized? IV ketamine only. And we are talking about doing a study with intranasal ketamine right now. Okay, and you'll see when you, some people have put some more information in. They, and here is somebody from Johns Hopkins is telling you about that they use buprenorphine regularly. So that will be a good source for you. Yeah, and then I thought it was interesting. You know, it's just very hard to distinguish and your use of between addiction and pain that is not treated well, especially in a population that you talked about that has so much pain on an ongoing basis and being frustrated in how to deal with it. So it is good that you talked about how you consult the addiction and having a addictionologist or addiction physician that you have contact. It seems to me that that is a very important feature for any program because it is hard to evaluate. Yeah, thank you. And I appreciate that. I mean, I think sometimes we just don't know what we don't know. And I have to find someone who can help me with that. And yeah, so a lot of things, a lot of it's just all the polypharmacy and all the drugs on board. And then I find out, oh, you're not going to school. And then, oh, then it's just kind of that outlier. Oh, they are doing some of those aberrant behaviors that I listed on my slide. And I'm like, when it gets a little out of hand, I'll talk to my attending physician and we bring them up in noon conference. And I'm like, I think I'm gonna talk to addiction psychiatry because I don't know what else to do for this young person, right? I mean, yeah. And you talked at the beginning about, and this is one of the people said that, the behaviors that are out of control, those anger behaviors and so forth. And how is it that you handle those things? Is that mainly through your psychologists come in and help you with that, manage behaviors? That's a good question. Usually, the first thing I'll do is go into the room and it's a big old hornet's nest in there of just this and that, but go in there, first of all, and apologize to the patient. We're not doing you the best here and we are trying very hard. And then the second thing I'm gonna do is acknowledge the pain. That's the big thing. Because a lot of times you're just, and then thirdly, I am gonna call my psychology colleague and we're gonna try to do better. Usually though, by the time things have escalated to that point, there's usually a care conference. And so I'm talking to a social worker and we're trying to kind of put our heads together. At Children's Health in Dallas, the other thing we do is we round together. So, there's a unified front, there's no splitting between caregivers and so forth. And we're just trying to take care of that patient as best as we can. But psychology is very much involved. Like I was saying, during our multi-modal group, they spend almost an hour. And they assess for anxiety and depression and some of the, are they maybe doing some recreational use of drugs, what's going on in the home, those types of things. I mean, that stuff is very important. The other thing is we have a database on the computer that shares all this information because again, every time these patients leave and then get admitted, it's like we're reinventing the wheel each time because we just don't have a great, our EMR is terrific. It's just that we just don't know how to always use it. And so we're trying to improve that because it's always a miscommunication between other practitioners and other subspecialties or disagreements on how to manage pain. Right, right. And it's hard when it's not clear what to do. Well, we're out of time, Jim. And there are a lot of people who want to get in touch with you. And Kandace will send you that information. We'll also make sure that your contact information is available on the website for people to get in touch with you. But I thank you so much. So thank you everyone for participating in the webinar. Just as a reminder, we're able to provide continuing education for this session. At the end of the webinar, you will see an email with an evaluation form from ASPMN. You must complete this within 10 business days in order to get your nursing continuing education credits. And a link to retrieve your certification will be included at the end of that evaluation. Even if you are not applying for CEs, please take a few minutes to assess the evaluation and provide feedback on today's session. You will get a second evaluation and that will be from the PCSS organization. And please complete that second evaluation. That's for purpose of the SAMHSA grant. A reminder that today's presentation was recorded and will be posted tomorrow afternoon on the ASPMN website. And then in the near future on the prescribers clinical support system. And that URL again is www.pcsnow.org. And there's a calendar of upcoming events and helpful clinical information on there as well. And we hope you join us for some upcoming sessions. We have two more webinars coming up through ASPMN and you will find that information on our website and you'll be getting reminders soon. Thank you everyone. And thank you again, Jim. Thank you ma'am.

Jim DeMasi

chronic pain

pediatric patients

sickle cell disease

multimodal approaches

pain management

genotype

opioid crisis

individualized treatment plans

mental health concerns