Sickle Cell Disease (SCD) Achieving Success Through Synergy

Transcription

1 Sickle Cell Disease (SCD) Achieving Success Through Synergy Webinar Series: Aligning Patients and Providers to Improve Pain Management for SCD Part 2. Adult SCD Patients in the Emergency Department Improving Patient and Provider Satisfaction Introduction Frances Richardson Slide 1: Good day everyone. This is Frances Richardson, atom Alliance Sickle Cell Project Lead. During the first presentation in our series, you heard from a sickle cell patient who shared her perspective and experiences related to living with SCD. Now, I would like to introduce you to today s clinical speaker and sickle cell disease expert, Dr. Sophie Lanzkron. Slide 2: Our Speaker Dr. Lanzkron will discuss Improving Patient and Provider Satisfaction with Care in the Emergency Department for Adults with Sickle Cell Disease. She will touch on the etiology of sickle cell disease, the epidemiology of sickle cell pain and the quality of ED care for sickle cell disease. Dr. Lanzkron is an Associate Professor of Medicine and Oncology in the Division of Hematology at the Johns Hopkins University School of Medicine and the Director of the Sickle Cell Center for Adults at Johns Hopkins, which delivers state-of-the art, multidisciplinary care to over 500 patients. She is internationally recognized for her pioneering research on the optimal care and management of patients with sickle cell disease. She has served on the National Institutes of Health, Expert Panel in the Management of Sickle Cell Disease and is an NIH and PCORI-funded researcher. Her research focus is on improving the quality of care provided to this historically underserved population. Dr. Lanzkron is considered an expert in health services research in sickle cell disease. Welcome Dr. Lanzkron Dr. Sophie Lanzkron Slide 3: Thank you, Frances. So I m going to talk a little bit today about how to improve both the patients and the providers experience of care and provision of care within the emergency department for adults with Sickle Cell Disease.

2 Slide 4: Case JP I m going to start with a case, this is a 23-year old with Hemoglobin SS Disease who presents reporting typical vaso-occlusive type crisis pains in his arms and legs, he was last hospitalized just two weeks ago, his medications include Hydromorphone 4 milligrams every four to 6 hours as needed and folic acid, a milligram a day. He s talking to his mom but when you walk into the room; when he sees you he or she curls into a ball and starts to complain of 8 out of 10 pain. Background Sickle-Cell Anemia Slide 5: Sickle Cell Anemia So I m going to back up a little bit before we delve into this gentleman s life and talk a little bit about what Sickle Cell Anemia is. Just to refresh your memory, it s a single-based substitution of a valine for a glutamine at the sixth amino acid of the gene encoded for the hemoglobin beta chain. It affects approximately 100,000 Americans, and compared to the general population, in its most severe form still decreases life expectancy by about years. There are at least four distinct origins in Africa of this gene and one in Southeast Asia. The reason that the gene mutation survived is because it provides a profound survival advantage against Malaria. It s probably the most protective thing against Malaria that exists; so, it plays an important role in survival in Africa. Slide 6: Hydrophobic Pocket This is a picture of the two hemoglobin molecules, one who s normally has a hydrophobic pocket, but what is not normal is the valine sticking out of the other hemoglobin molecule. Slide 7: Polymer Formation And what happens, under the right clinical conditions, is that the hemoglobin molecules begin to stick together and develop this polymer. Slide 8: Intracellular Polymer Content Intracellular Polymer Content Results in Rigidity, Changes in Cell Shape and Membrane Distortion. As you can see how in this picture, in A, there s a normal shaped red cell - a biconcaved disc with hemoglobin that appears to be floating inside the cell - but as we progress to E, we see the development of polymers within the red cells until you get to E where we get this abnormally shaped red cell, a sickle-shaped red cell, that we think breaks up in the circulation that is removed by the reticular endothelial systems. In D you can see the red cell that is getting to be sickle shaped, we think that potentially this cell is reversibly sickled, that it can be turned back to its normal shape. Slide 9: Consequences of Polymerization: Vaso-occlusion This picture shows what we were all taught in medical school about what causes a vasoocclusive crisis. This abnormally shaped red cells that occlude the microvasculature [technical inaudibility] and areas [technical inaudibility] that cannot get blood so there s areas of hypoxia and that causes pain.

3 Slide 10: Cellular Adhesion We now know that the development of a vaso-occlusive crisis is much more complicated than that just an abnormally shaped red cell. We know that there is increased expression of the adhesion molecule both on the surface of red cells, especially on reticulocytes, they re called express reticulocytes, as well as an increased expression of adhesion molecules on the surface and the lining of the vessel wall. And this very sticky environment, we think, plays a role in the development of a vaso-occlusive crisis. And new therapies are now being used and studied that decrease adhesion molecule expression with the hope that it will help decrease vaso-occlusive crisis events and also shorten vaso-occlusive crisis events and those are currently under study. Slide 11: Regardless of what the pathophysiologic mechanism is, this is what it looks like. I m showing you a child who is about 4 to 6 months of age who is presenting probably for this first time with their first SC complication. This baby has Dactylitis which is they present with swollen hands and swollen feet and nobody would question whether or not this baby is uncomfortable because there is an obvious and objective measure that there is something going on with this child. Chronic Pain Management Adults with SCD Slide 12: Epidemiology of Sickle Cell Disease Pain I want to talk a little bit about this pain experience because what we see in a child is not what we see in an adult. Often, an adult will not have an objective measure of crisis. Sometimes they ll have a swollen joint, but, more often than not, there isn t anything that you can look at or see that tells you a patient is having a painful event. So, when we think about pain, which is the complication that drives the majority of sickle cell patients to interact with the healthcare system, we think of two types of pain in sort of a general kind of way. The first is the typical, classic vaso-occlusive crisis, something that has a beginning, a middle, and an end. On average, these can last about a week or so, they tend to peak in pain at Day 4, although there s lots of variability there. And then the other type of pain is chronic pain. Now we used to think that this chronic pain was due to the accumulation of organ and tissue damage over time, but honestly, we don t really know what causes the chronic pain. There is probably some aspect of central sensitization, so that there is plasticity of the neuro system that causes ongoing pain despite a painful stimulus, and so, chronic pain is complex and poorly understood but is a major contributor to the pain experience for people and especially adults with Sickle Cell Disease. Slide 13: This data that I m about to show you is from a nice study from Wally Smith from the Annuls of Internal Medicine from He took about 230 patients, most of whom were adults, and had them keep a daily diaries for up to 6 months. Slide 14: Breakdown of Diary Days And this graph nicely shows the pain experience of adults with Sickle Cell Disease. You can see here at the bottom of the graph that 45.5% of the 30,000 diary days that were collected in the study, in which patients did not report having any pain. Then above that is the 38.3% of those 30,000 days in which patients complained of pain without crisis, which is chronic-type pain, with

4 an average pain score of 3.9 on a scale of 0 to 10. The 12.7% days are the days in which patients reported having crisis-like pain, with an average pain score of 5, in which they did not seek out care for the pain. So none of these prior days patients sought out care for their pain. But at the tippy-tippy top of this graph is the 3.5% of days in which patients reported either vaso-occlusive crisis-type pain or chronic pain for which they sought out care for their pain. And so what this graph really represents and what we should learn from it is that patients with Sickle Cell Disease live with a lot of pain and they don t always come in for care. So, when you have a patient sitting in front of you in pain with Sickle Cell Disease you have to step back and say, Why today?, because it s likely that this patient lives with chronic pain or frequent episodes of pain but on this day has shown up for acute care. Emergency Care for Adults with SCD Slide 15: Quality of ED Care for SCD I want to talk a little bit about what we know about the current state of the quality of emergency room care for people with Sickle Cell Disease. It is quite clear, from both the patients and providers perspective, that there is a lot of dissatisfaction in the quality of pain management. Patients with Sickle Cell Disease report not having enough involvement in decisions about their own care and that providers do not demonstrate respect, trust and compassion. And there is some basis for this belief because studies have demonstrated that providers hold highly negative attitudes towards people with Sickle Cell Disease, and that they are strongly predisposed to suspect addiction in patients presenting for vaso-occlusive crisis care. Slide 16: Quality of ED Care US In another study looking at a retrospective cohort of adult emergency room patients, comparing individuals with Sickle Cell Disease to those with renal colic, the median time analgesic dose was 80 minutes for patients with Sickle Cell Disease and 50 minutes for patients with renal colic, and this was despite the fact that patients with Sickle Cell Disease reported higher pain scores on their arrival when compared with the renal colic patients and were more frequently assigned a higher triage priority. Case Study SCD Well Day vs. Crisis Slide 17: Case Presentation Back to our case of this 23 year old male; these are actually lab data from a very reliable patient of mine and there are two sets of labs here. One is from a visit that the patient made for a study that he was participating on for a well day and the other is a day in which he presented to the emergency department visit with a vaso-occlusive crisis. So if you look at these lab data you can see that the white count was a little higher on the December visit; maybe the baseline is 4.6 and now he s got an infection; it s hard to know from just looking at this data and not knowing the patient. Hemoglobins are about the same, hematocrit is about the same, retic is about the same and the message here is that you can t use lab data to tell when a patient is having a vasoocclusive crisis; it s quite clear that there is no lab data that confirms this diagnosis.

5 Slide 18: Diagnosing a Painful Crisis So how do you make a diagnosis of a painful crisis? And this is the thing that awkwardly [inaudible] the most difficulties for both the patient and the provider because the answer is there is no lab data, no radiographic findings, no vital signs that tells you that a patient is having a vaso-occlusive crisis. There just isn t any objective measure. And the gold standard for determining whether or not a patient with Sickle Cell Disease is having pain is when they tell you they have pain. And again, this is why this is a struggle, if there were some lab tests you could do, it would probably make it easier for patients and providers. Providers would feel more comfortable using opioids if they had a measure of vaso-occlusive crisis, but there isn t one and we all need to be able to adapt to that fact because it isn t going to change. Treating Vaso-Occlusive Crisis Slide 19: Evidence Base for the Treatment of VOC So what is the evidence-base for the treatment of a vaso-occlusive crisis? Again, another reason that providers struggle is that there aren t really good evidence to tell us what to do when a patient presents with a vaso-occlusive crisis; there just isn t an evidence base. Some pediatric providers got together using a Delphi methodology to come out with some recommendations about what could be done for children who present with vaso-occlusive crisis. And what they published was that children with Sickle Cell Disease with an acute pain episode should receive a parenteral analgesic within 60 minutes of registration or 30 minutes of triage. Their initial pain assessment should be documented using an age-appropriate pain scale, and the assessment should be repeated within 30 minutes of the first dose of the analgesia. I show you this because these recommendations have now infiltrated into the NHLBI Guidelines, and so a lot of other literature and things that you ll read that this is becoming the standard of care for not just children but adults, too, when they present for acute pain. Slide 20: What is the Best Way to Treat the Pain We do know that there are some ways to improve outcomes when treating patients for pain. The initial report of how to do this came from Dr. Benjamin who was working at the Bronx Comprehensive Sickle Cell Center and published her experience using a day hospital. So, instead of patients going to the emergency department for the management of their vaso-occlusive crisis, they went to a day hospital. In this day hospital, they used a specific assessment and treatment protocol and they were able to control pain in 90% of patients, they decreased hospital admissions by 40%, and decreased the average length of stays for those hospital visits, as well. And Dr. Benjamin feels that the key to the success of this was rapid assessment and treatment of pain, and rapid re-assessment of pain throughout the time period. Slide 21: What is the Best Way to Treat the Pain An additional study out of an emergency room in Chicago, investigators used a clinical pathway and the five basic features of their clinical pathway were to identify tolerant patients, those patients who were on MS Contin 60mg three times a day are not going to get a dose of a mg of IV Morphine to try and control their pain, so they identified tolerant patients, they used patientcontrolled analgesia, they used adjuvants such as Acetaminophen and Ibuprofen, and they were able to achieve pain relief 80% faster and a decrease in length of stay by 50%.

6 Slide 22: Treating Acute SCD Pain So these are the published NHLBI Guidelines that you can find online, on recommendations for how to treat pain in the emergency department setting and there s actually a nice algorithm within the NHLBI Guidelines. The basic tenants of this are rapid clinical assessment, recognizing tolerance and giving an appropriate first dose of opioid, aggressively managing patients, re-assessing them, and involving the patient in the decision about what the appropriate treatment is. Asking a patient what dose typically works for them is an appropriate first step. I ll tell you, in a patient who I don t know and they come in and tell me, I need 8 mg of IV Hydromorphone to control my pain, I m not going to give them 8 mg of IV Hydromorphone to control their pain because that s a very high dose and it would worry me since I don t know the patient, so I would give a lower, more appropriate dose, but then I d go back in 15 minutes, in 30 minutes and see how the pain is doing and how the patient is doing in managing their pain and give additional doses, as necessary. Slide 23: Guideline Consensus Recommendations This is again, recommendations from the Guidelines, and one of the things that the Guidelines recommend is using individualized prescribing protocol, so, having patient-specific treatment plans. I m going to discuss that a little bit more in a minute. Adult SCD Patients and Opioids Slide 24: Patients and Opioid Misuse But now I want to talk about this issue of patients and the concerns for opioid misuse. So there are a lot of clinician fears about contributing to or causing addiction to opioids and this is a recognized barrier to the delivery of high quality pain management. But studies have shown that the prevalence of substance abuse and addiction among people with Sickle Cell Disease is actually lower than or maybe some studies showing the same as that found in the general population, but studies again have shown that clinician attitudes and physicians tend to overestimate the prevalence of addiction in the Sickle Cell disease population and these attitudes contribute to patients reports that acute visits for their pain are often dehumanizing experiences. Slide 25: High Risk Behaviors This is one study demonstrating the prevalence of narcotic use, smoking, bad behaviors in a group of young people with Sickle Cell Disease compared to a group of young people without Sickle Cell Disease and this is from North Carolina. You can see here that the actual number of percentage of these children who are using alcohol, smoking cigarettes, using marijuana or cocaine, is actually significantly lower than children who do not have Sickle Cell Disease. Slide 26: High Utilizers or Frequent Flyers And then there are the high utilizers or what s called the frequent flyers not a great term, but there is a known subset of people with Sickle Cell Disease that are known to contribute disproportionately to ED utilization. There is some suggestion that the high-utilizer population does have an increased prevalence of substance abuse; yet, not everyone who is a high utilizer has substance abuse issues. Some of the high utilization comes from poor disease management, those other social issues. But what we also know and what is really important to understand

7 about the high utilizer population is that they have been shown to have more severe disease, they are at more risk for morbidity, more risk for mortality, and we have to keep that in mind when we see them in the emergency room. So everyone struggles with this patient population, if you talk to providers who take care of a lot of folks with Sickle Cell Disease, they ll all report that it s about 10-20% of their patient population but they represent over 50% of the ED visits, over 50% of hospital bed days, but only 24% of office visits. Interventions to Improve Care Slide 27: Provider Attitudes Going back to provider attitudes, which I think are driven somewhat by this high utilizer population, it s been shown that, compared to hematologists, ED providers have more negative attitudes towards people with Sickle Cell Disease. Pediatric ED providers have more positive attitudes, while adult ED providers have more negative attitudes towards people with Sickle Cell Disease. Importantly, ED providers with the highest level of negative attitudes toward people with Sickle Cell were less amenable to adhering to recommended pain management strategies - this is really a barrier to providing care. There are interventions that have been done to try to improve provider attitudes. We have used a video intervention to improve ED provider attitudes, and the reference is there. Slide 28: Interventions to Improve ED Experience: Attitudes The video shows people with Sickle Cell Disease who are living their normal lives and talking about their experiences of care. Much as Dominique just said. What I tell people is my experience with patients with Sickle Cell Disease is very different than what happens in the Emergency Room and even what happens in the hospital, because I get to see the folks living with Sickle Cell Disease on good days, when they re not suffering and in pain, and they re an amazing group of people living with what is a really bad, debilitating disease. So I get a lot of satisfaction from my time seeing patients in clinic because I really admire the people who live with this disease and the video really takes that message home and it behooves people to take a little bit of time out to see folks with Sickle Cell Disease when aren t at their worst, when they re not in severe and excruciating pain. Slide 29: Interventions to Improve Care Patient Specific Treatment Plans Another intervention to try to improve the quality of care is the use of patient specific treatment plans. Ideally these plans are developed by patients hematologists or their primary care physicians but I know that there are lots of emergency room physicians who see a lot of patients who don t have a hematologist or a primary care physician. But the use of these patient-specific treatment plans are really useful. The most common plans that we develop are the ones that we include for our patients who maybe come a couple of times a year and we want to make sure they get an appropriate dose as soon as they get to the emergency room and that is based on what prior dose has worked for them and what dose of opioids they typically take at home. But, we also have our far less used plans or more infrequently used plans and these are plans that limit opioid exposure and these are developed for patients sometimes who come a lot, who we feel are coming for managing chronic pain, so it s not that the patient isn t in pain, they have chronic pain. And the appropriate management of chronic pain is not the use of IV opioids, and so we

8 will develop patient-specific treatment plans that limit exposure in those settings. The other setting in which we will limit exposure to patients where it is clear that opioid use, the risks far outweigh the benefits. If I have a patient who s been caught in the hospital with needles in both arms that we didn t put there, those folks, the risk of giving them opioids is tremendous and so we figure out a treatment plan that is appropriate for those patients that does not use opioids. Slide 30: Patient Specific Treatment Plans A really important point that if you are going to use these patient specific treatment plans that limit the dose of opioids: they have to be developed and agreed upon by a multidisciplinary team, that ideally includes the hematologist and a pain specialist, and if you don t have those, certainly an emergency room doctor, a social worker people who really know the patient to help develop a plan that is appropriate. The plans have to be justified with reasonable documentation, so you can t just decide that so and so, We re just going to do the same because we are tired of seeing so-n-so, there has to be a real reason to do this. The plans have to be communicated to the patient prior to their institution; you can t have a patient just show up in the emergency room and surprise them with We decided to change your treatment plan. And these treatment plans have to the exception and not the rule. Regardless of whatever the opioid plan is, every patient on every visit must have a complete assessment for their Sickle Cell complications, because remember, it s the high utilizers who have the more severe disease and you don t want to miss a pulmonary embolism, or line infection, or sepsis, and so each patient, regardless of how often they come, needs a complete assessment. The emergency room doctors are really good at these assessments, we just need to make sure they happen for every patient each time they come in. Slide 31: S.C.R.E.A.M. Protocol I want to share with you a collaboration that we ve done with our emergency department. We do have a Sickle Infusion Clinic, but it s not open 24 hours a day, and so we have worked really closely and developed a very close relationship with our emergency room and so that whatever treatment plans we do in our infusion clinic are the ones that are done in our infusion clinic, an ED champion participates in our multidisciplinary weekly patient meetings so that we can discuss patients that have problematic behaviors or the ones in which we are really worried about their assessments when they come in, and what to look and so it s a really close collaboration. But, on their own, the ED went a step further to try to improve the delivery of pain management for this patient population. So, at the time that they instituted this, the time to first dose of pain medication was 190 minutes so, far beyond that 60 minute goal, and we know from prior data that in a model controlling for patient acuity, for every 10 minute increase in time to first dose of pain medication, the relative risk of admission increased by 0.7%. The primary cause of this delay was the availability of an observation bed which is where we put all of our patients with Sickle Cell Disease. And in order to address this, the ED, led by an ED physician assistant, Stephanie Figuerao, developed a protocol to treat patients in the waiting area. Slide 32: S.C.R.E.A.M. Acute Pain Crisis Patient Adult ED Process Map/Workflow And this is just a process map, so the goal is to have triage identify Level 3 Sickle Cell patients who presented to the emergency room within 30 minutes. The physician assistant or advanced

9 practice provider then performed a rapid evaluation of the patient in the [observation room] and took the patient out of the waiting room and put them into a holding room to get them evaluated by the advanced practice provider. The goal is to do that within 60 minutes and then, the relief nurse would administer that first dose of pain medication. Slide 33: S.C.R.E.A.M But you can see from this graph that the red is before the institution of the S.C.R.E.A.M protocol and the green is afterwards. They really were able to decrease it not to 15 minutes yet, but we are really hoping to get there but to a 90 minute rate of time. And importantly, we already had a pretty low admission rate because we have an infusion clinic, which has been a very effective way of decreasing the admission rate, which on average across the nation is about 40% from the emergency room, but adding this piece also decreased the admission rate 22% to 15%. This rapid delivery is really a key in trying to decrease admissions. Thank you. Conclusion Frances Richardson (after Dr. Lanzkron speaks) Slide 34: Conclusion Just to recap, to improve care in the ED and to increase patient and provider satisfaction, you reminded us that this patient population lives with acute and chronic pain, which is often excruciating, and that very few sickle cell patients are addicted to opioids. You also recommended following the NHLBI guidelines and developing patient specific treatment plans. And finally, you encouraged us to consider or imagine the patient on a good day. Dr. Lanzkron, thank you so much for your insight and sharing with us today. Slide 35: Questions/Contact Information I want to remind everyone of how you can participate in this project. Please join our series of learning sessions, make patient education referrals, and share best practices and lessons learned. My contact information is noted on the slide. To learn more about the project, you may also visit our website at atomalliance.org or you can contact me directly at Please share your input with us by completing an evaluation survey.