Sickle Cell Disease— Look Behind the Pain Video Introduction
Slide 1. Hi. I’m Dr. Patricia Adams-Graves, the Director of the Diggs-Kraus Sickle Cell Center, and Professor of Medicine at the University of Tennessee Health Science Center. I’m going to be talking about Sickle Cell Disease and ‘Look Behind the Pain’.
Objectives Slide 2. The objectives are to define Sickle Cell Disease in order of its severity, it’s incidence, the top four manifestations of Sickle Cell Disease, approach to acute and chronic pain treatment, approach to the difficult patient, become aware of associated complications, as well as, maybe some solutions of developing collaborative efforts between us hematologists, primary care doctors, and emergency room providers.
Defining Sickle Cell Incidence of Sickle Cell Disease Slide 3. The incidence of Sickle Cell Disease is that one in every 400 African Americans is diagnosed with Sickle Cell Disease. But, what’s profound is there are millions and millions of people living with diabetes, cancer, and heart disease, but it is estimated that only 100,000 African Americans live with Sickle Cell Disease. For the other ethnic groups in the Mediterranean areas, the incidence in America is really unknown. The fact that there are only 100,000 living with Sickle Cell in America makes this disorder in America a rare blood disorder. However in Africa there are millions and millions of individuals living with Sickle Cell as you see here with diabetes and cancer in America, so there are a lot of knowledge gaps due to this being a rare condition in America.
Genetic Pathogenesis Slide 4. The genetic pathogenesis is due to a point mutation in the gene that codes for hemoglobin. We do have a picture of that so you can see the valine is substituted for glutamic acid for Sickle hemoglobin SS, whereas Lysine is a substitution for Beta Sickle C disease, and it causes profound manifestations just from that little single mistake in the gene that codes for hemoglobin. It’s important because hemoglobin is the protein that carries oxygen all over the body. Each red cell is made up of 70% hemoglobin, so that one mistake is actually profound, and that’s why Sickle Cell conditions are so difficult for individuals living with that condition.
More Holistic View Slide 5. Now, I did go over the genetic pathogenesis as we’ve known it over the past 100 years, however, there’s a more holistic view of the pathogenesis, which is that the Sickle Cell and their abnormal contents are very, very sticky on the outside, and when they die early in the body, they
release a lot of these inflammatory cytokines, the color pigment we call bilirubin that makes their eyes yellow, and these cells interacting with the vascular endothelial lining inside the vessel cells cause sluggish blood, vascular occlusion, inflammation. Now, what’s interesting is we have patients in the hospital that say, ‘I feel so much better, the pain crisis is over,” just to go in the next day and they say, “Now wait a minute, I’m worse, I’m not feeling right.” Well, there’s such a thing called “reperfusion injury”, where the vascular tree opens back up suddenly but that explosive flow against the vessel wall causes this inflammatory response and blood flow resumes. That’s called a ‘reperfusion injury’. These oxygen radicals that are formed are very toxic to the vessel wall itself and ultimately can result in a vasculopathy, which we now know is problematic for individuals living with Sickle Cell and I do have the references to this research on the slide.
Severe Hemoglobin Variants Slide 6. There are four types of severe hemoglobin variants. The most severe is Homozygous SS. And then, the next one to that, which has at least an equal clinical presentation, is Hemoglobin S Beta Thalassemia Zero, where there is no normal working adult A hemoglobin. The next most severe type is the Hemoglobin S Beta Plus where there is just a little tiny bit of working normal hemoglobin and, then lastly, the Sickle hemoglobin SC, which is a compound heterozygote, and these individuals all have pain. It’s important for us as hematologists to know what type of Sickle Cell we’re dealing with and, for the Emergency Room, it might not be so clear because pain is pain. However, I would encourage you to try to, at least, look at this a little deeper because they do vary in their clinical presentations somewhat.
Major SCD Manifestations Slide 7. The major manifestation, as a result of this point mutation that they’re born with, is vascular occlusion that I alluded to already, which is an interruption of normal blood flow. Wherever there’s a vessel, it could have an interruption of blood flow. It could be a leg, an arm, it could be in an organ, and it could be in the brain. The next manifestation is anemia. Anemia is a result of a shortened red cell life span, or hemolysis as we know it in the medical community. It’s both intravascular as well as extravascular hemolysis, and therefore, Sickle S cells live about 14 days whereas normal red cells about 121 days. That’s why they’re anemic, however, the SC folks, their cells live about 45-50 days, so they’re kind of right in the middle. The next manifestation that we need to be aware of is the inability to fight encapsulated organisms. Those types of infections render very catastrophic, when fever occurs. That’s why we tell them to go to the E.R. if there’s a fever over 101. Sickle Cell patients can’t fight encapsulated organisms such as Klebsiella. I actually have a patient who had an amputation below the knee due to Klebsiella sepsis; she could not fight and we actually had to administer antibodies, IV IG to her and after a three month course of fighting Klebsiella, an encapsulated organism, became catastrophic for her and she is now using prosthesis as well as in a wheelchair. The fourth manifestation that’s a combination of the vascular occlusion, anemia, and the infection is baseline inflammation. This vasculopathy. One would ask, “Well, why don’t we use
steroids?” It’s been done in studies, however, when you wean the steroids, the Sickle Cell reoccurs in a vengeance and they have the worst pain crisis ever, so we try to stay away from that unless there’s a co-morbid state such as Lupus or Rheumatoid Arthritis that requires steroids.
Identifying Sickle Cell in the ER Steady State Levels Slide 8. Now, after alluding to the anemia as a manifestation, what the emergency room doctor should know is that there’s variability in the steady state levels of the hemoglobin whereby Sickle SS homozygous individuals can go down as low as 5 grams. It’s a little scary when they get down that low, but a lot of times they are asymptomatic as low as 5 . So if you look at the chart I have provided, the range of hemoglobins for an SS individual is 5-10; S Beta Zero Thal is 6-11; SC can actually be normal as well as S Beta Plus Thalassemia. There is confusion with those individuals because when they present and they are not anemic, you might want to think they’re not actually here for Sickle Cell but may be a trait. However, there are compound heterozygotes and they just happen to have higher hematocrits. But if you look at their retic counts, and they retic count is usually elevated as well as a slight elevation in their bilirubin, they have real Sickle Cell Disease. Slide 9. Now there was a study in the 1970s that looked at 4,000 Sickle Cell patients. These patients, from both up to the age of 48 months, demonstrated that they are all born with Sickle Cell Disease and manifest by the age of six months. At six months, they may have sequestration crisis where their liver and their spleen sequesters out those abnormal cells that converted from their hemoglobin F or the baby hemoglobin they are born with, over to adult hemoglobin. That is the extravascular hemolysis that occurs and if these babies do this, it’s like having an internal bleed. They need a blood transfusion; if they don’t get the blood transfusion, they could die. Now, what’s important on the adult side is people with Hemoglobin Beta Thal Plus and Hemoglobin SC, they could still do this but the hemoglobin homozygotes, they do not do this because they infarct their spleen and they do not have the ability to sequester those red cells in adulthood. They also can develop dactylitis in the digits and life-long anemia. This diagram demonstrates that, by the age of six months or so, pain becomes the primary manifestation. You’re talking about a two year old. So, all individuals have life-long pain. They are in pain intermittently all of their life, and so I need the emergency room doctors to be mindful that yes, they’re grown now, they survived childhood from sepsis and all these other childhood complications and strokes, but you still have to remember they were born with pain.
Acute and Chronic Pain Treatment Pain Severity—Most Common Presentation Slide 10. So, the most common presentation is pain, as you saw on the previous slide. Pain is the cardinal feature of Sickle Cell Disease in all sub-types and what gets on all of our nerves is it is “unpredictable”. So, we are going, “Are you kidding? Certainly it’s not that bad.” But, it is. It is really that bad. I happen to have Sickle Cell Disease in my family, so I’ve been in the house seeing two or three cousins suffering and it’s no joke. The pain episodes may signal a life-
threatening complication and that’s why the emergency room docs need to take this very, very seriously. In an acute episode, what is important here is an acute pain episode precedes 22% of Sickle Cell deaths. I think that takes a pause. And it is also associated with those complications that you need to be aware of.
Look Behind the Pain Slide 11. So, my central message to emergency room providers is, while I’m sympathetic about the triage that you do and they may present with pain and they’re whiney and there’s no respiratory distress, it looks pretty uncomplicated, is to try to look behind the pain. What I’d like to relay in my 30 years of practice is that, oftentimes, if there’s something bad going on behind the pain, there is a 72 hour delay from E.R. presentation, unless they sat at home longer and they come in with the presentation, there is a 72 hour window where they could really do something catastrophic that’s associated with a severe complication.
Disease Complications Manifestations of Severe Variants—Infection Slide 12. So let’s just go over some of these complications. First, the manifestation of what we call the splenic infarction, probably by as early as the age of seven, that renders them with the inability to fight infection such as Asplenia condition that causes reduced immunity, and therefore, when they develop a pneumonia or blood infection – if it’s one of those organisms – they cannot fight. So we do know that Salmonella, Neisseria Meningitidis, Klebsiella, pneumonia, are some of them, and the pneumococcus, that used to kill the children before they could even reach five years of age. We now have those children on prophylactic penicillin so we have this growing population of adults that you’re going to be met with now in the emergency rooms. So you can look at these pictures; now one of the distinctions…just yesterday I had one of my patients who is mentally challenged, she came in and she wasn’t very forthright due to her mental condition. She had actually presented with a pain crisis – that’s all she could describe. So they treated her appropriately and sent her out, but she came straight up to my clinic when we opened saying that she was still not right, and it was her left foot that was really hurting where the ER said they got her to zero pain. Well the pain reoccurred, just to find out that her left foot was swollen all the way up her ankle and she has an ankle bone infarct. And, in the picture you’ll see where we talk about osteomyelitis. That is less frequent in America. That happens in the Third World where the water is not clean, Salmonella osteomyelitis, but bone infarction occurs and the C-reactive protein, which is an inflammation marker, is often elevated, sometimes slightly, and if that’s the case then that person deserves a closer watch because that is extremely painful and it hangs around two to four weeks. Now, I’m not saying the person should be in the hospital for two to four weeks for it; I’m just saying that it’s only diagnosed through an MRI. But if you have any kind of sense that a bone infarction is going on, then you know, the nonsteroidal anti-inflammatory agents along with an opiate is prudent.
Vascular Occlusion Slide 13. The spectrum of its evolution is that first you have an interruption of blood flow, wherever it is. Then you get tissue ischemia, inflammation, infarction, damage to the tissues, and then this pain is there, wherever that occurs. Now, what’s dangerous is if it’s in the muscle then its uncomplicated pain. But if it’s in the organ, then it could cause organ failure. Also if it’s in the muscle, and maybe even some bone infarction, in the ribs, that person is not going to be able to treat their pain at home. If they could treat their pain at home, the vast majority of them would stay at home. Now, entertain me. I am not talking about the 5% of patients who are deviants. I am talking about the 95 – 97% of the good people. So give them the benefit of the doubt when I say, ‘Hey. Believe the patient,’ even when you don’t see anything because a lot of them are younger than a lot of the patients that you see and they look good on the outside, but believe me, they are not looking good on the inside.
Major Pain Syndromes in SCD Slide 14. Now, there is a multitude of pain syndromes associated with Sickle Cell Patients. They are categorized as acute and chronic, and sometimes acute on chronic. Now, I’ve got the list there and I’m not going to belabor them but I want you to look at them. And the Top 4 or 5 in the acute side for the ER is, of course, the Sickle Cell Chest Syndrome. Sickle Cell Chest Syndrome encompasses “a syndrome”. It encompasses not only a new infiltrate on chest x-ray. The hypoxemia syndrome it could represent a fat emboli syndrome; it could represent an impending lung infarction. It could depend on a sternal bone infarction in the chest with hypoventilation and difficulty breathing because of the pain in the bone and have nothing to do with the lung parenchyma so chest x-ray followed by a C.T. scan is prudent. Cholecystitis, abdominal pain, right upper quadrant, or they could have that vague abdominal crisis that causes pain without gall stones, stroke evolution and then the uncomplicated pain syndrome and then painful erection – all of those things are horribly painful and I would let you read the chronic ones but I would take it pretty seriously to look behind the pain.
Manifestations of Severe Variants – Vaso-occlusion Slide 15. This is a picture, on the next slide that shows, from the NIH, a blood vessel that’s been sort of cut in a longitudinal fashion to look at red cells flowing nicely through the blood vessel. And then on the right panel the sickle cells jamming up blood vessels – and that is just showing only the red cells – there are no white cells shown to illustrate that these people actually have interruption of blood flow and these are red cell clots. And you think of a PE or a DVT, these are a little different and require a different type of treatment and approach. Anticoagulation does not necessarily help these types of occlusive events. On the bottom left is a lung vessel that is actually a subsegmental lung vessel, if someone comes in with pleuritic chest pain or a low O2 sat, they will be experiencing, oftentimes, vascular occlusion in lung vessels. And, I want to give this pearl to you, “Can you really see that on a chest x-ray?” I had an ER doc call me and say, “My patient’s O2 sat is 90% when it is normally 100% and they have a little chest pain, so the chest x-ray is clear and we’re going to send them home.” I don’t advise that. I advise to observe that person. That person could be having a vascular occlusive disease in the subsegmental areas
of the lung, and it’s below the resolution, even sometimes of the CT, and I will do earlier blood transfusion in that individual and I think it warrants at least a hematology consult as we have hypoxemia syndrome and I’m going to talk about later. Above that slide is obviously hemorrhagic stroke that we are seeing in the adult patients as well as with the children.
Lumbar Spine SCD Crisis Slide 16. The next slide shows that ‘back pain’. Oftentimes you can’t see anything, but this is unfortunately an autopsy of a person who died and they were also were in back pain. In the Diggs Collection, which I have in my office, represents 1,000 autopsies that Dr. Diggs did over 60 years, this is one of those pictures that shows the bone infarct in the spine that you can’t see, and that’s extremely painful. Bone infarcts can happen in the long bones, just like someone who has a pelvic fracture, and cause fat emboli to embolize into the lungs, so I wanted to actually illustrate that by that picture of that pathological spine vertebral body bone infarct.
Manifestations of Severe Variants – Vaso-occlusion continued Slide 17. Next slide just shows leg ulcerations. Like I said, wherever there is occlusive interruption of the blood flow in the skin, you’ll see breakdown of leg ulcers. About 70% of Jamaicans have it, but in America, in our area we have about 15% of our population have leg ulcers and they rarely occur before the age of 10. My point is, is that is a manifestation that represents chronic pain. So, if someone comes in with a pain crisis and it’s in their arm, they’re likely on a pain medicine that increases their tolerance so they can tell you, most of the time, the dose of the pain medicine that works for them. It’s not drug seeking, but just pay attention: if they already have a leg ulcer and they’re already on pain medicine, then they do require higher doses of pain medicine than the normal population. The middle panel shows osteonecrosis of the bone and the far right upper shows bilateral hip replacements that were required from that AVN from panels ABCD. The lower left represents hepatic crisis. The sinusoids are occluded with sickle cells and the middle panel is mostly Hemoglobin SC, so if they get a retinal disease and they could potentially go blind, that panel on the lower right just represents how their blood looks – it’s not your typical sickle cell shape - its half sickle and half sea crystal.
Relative Hypoxemia Syndrome Slide 18. Now, I mentioned earlier about the presentation of hypoxemia syndrome and I wanted to define it. Sickle Chest Syndrome is a new infiltrate on a chest x-ray accompanied by anything referable to the chest, with or without fever in adults. You don’t have to have fever to have chest syndrome in sickle cell individuals. The right panel, as you see, is hypoxemia syndrome, which is a clear chest x-ray with an O2 sat of less than 95% on room air, especially in a young person. Just like you’d want the baseline hemoglobin, you’d need the baseline oxygen saturation in anything referable to the chest. The A-a gradient most of you have on your calculators, medical calculations, it tells you if a person is shunting, that is a good test on room air. Now, when I get called and they say, ‘They’re 99% on 2L of Oxygen”, that is fake – that is with supplemental oxygen. I want to know what they’re doing on room air so I can make a clear assessment of their
body without any help. The A-a gradient, if it’s done on room air and it’s greater than 20, they’re shunting, that requires an admission in my view.
Collaborative Solutions PCMH Slide 19. Now, one of the things I need you to feel comfortable about is, here in Memphis, TN, we work really well with our ERs. The ERs all over the city call me randomly for whoever presents so that we can discuss the case. I can give a patient profile, I usually know a lot about them in my head, I usually know what they require. We have a patient-centered medical home, The Diggs-Kraus Sickle Cell Center, whereby we try to decompress the emergency room during the week days because we feel the emergency room is not the place for primary care for any kind of chronic disease, including Sickle Cell. So this panel just shows from 1999 to current, well, up to 2015, how we have shifted presentations of Sickle Cell patients from the ED over to our outpatient center and, even when we had a hospital unit, we still have the unit, but it’s not as specific because of bed crunches, but we do have trained nurses, both from the inpatient side as well as the outpatient and we can take care of them and make sure that those uncomplicated pain events are pulled away from the ED so the emergency room doctors can focus on more critical emergency situations like respiratory failure, heart attacks, any kind of organ failure – those things can also happen in the Sickle Cell patient. So, we do know that they will still require emergency room care but oftentimes the emergency room doctors, I’m sure, will get frustrated when they have a patient coming in with uncomplicated pain that we can treat as an outpatient.
Treatment Slide 20. So our treatment, overall, pain, of course with opiates, IV fluids, anemia, sometimes they need blood, if they are Jehovah Witness we give them erythropoietin and we try to couple that with hydroxyurea so the Sickle Cells that they make are more favorable and not as sticky. We, of course, fight infection with immunization in the preventive mode as well as with antibiotics, and the vascular occlusive events its case-by-case, which can include a whole spectrum of treatments including blood exchanges. Now, I want to switch over to that 5 – 6% of the difficult patients. And, when I say ‘difficult’ – the deviations, the people who actually sell their pain pills, or they are cocaine addicts, they have mental illness, or they may have traumatic brain injury like the girl who came in in crisis with the infarct in her foot, she really couldn’t express herself. She looks like she’s ok but she really is mentally challenged. People with co-morbid conditions, the disruptive patient who is the sociopath and what we call – I hate this term – “the frequent flyer”. Sometimes that person doesn’t have a patient-centered medical home, because we don’t have ‘frequent flyers’ anymore like we used to because of the medical home. We actually have reduced our 30 day readmit rates down to probably about 1%. Our two week readmit rate is the same, which used to be huge; it used to be 30%, so we have made good strides with our infusion suite and our patient-centered
medical home. But it’s taken…I’ve been there 30 years and I must say that that’s not something that just happened. We worked that over some years before we got that patient-centered medical home refined so that we are working together in tandem with the ER docs and the hospitalists to make sure these patients are moving to the right place, at the right time, for the right reason, and there are no real premature discharges. Because we’re closed on Saturday and Sunday, we make sure that if it’s Friday, we have to get a good plan going so they don’t bounce back to the hospital. So, when we have patients that present to the Diggs-Kraus Sickle Cell Center, our goal is to get them that first pain shot in less than an hour, if we could do it in 20 minutes. And then we sit them down and when I get back to them they are calm, they are in less pain, and I can have a meaningful conversation to get their history of present illness and of course, if possible, we do the IV fluids. So we’ll do an IM pain shot, give them a warm blanket, and then we’ll see. Reassess, reassess with our treatments and then we might disposition them after that and we may admit them, it just depends on what’s going on.
Presentation to Diggs-Kraus SCDE Subacute Unit 8 to 4:30 pm Slide 21. Upon presentation at the Diggs-Kraus Sickle Cell Center we have a sub-acute unit that is Monday through Friday, 8:00 until 4:30. They have to arrive just before 2:00 pm to get care. The first goal is to get that first pain shot in less than an hour of presentation. Then we do a brief history and physical because the patient’s more comfortable; I can actually get a meaningful history of present illness at that point; they are calm and I don’t worry about being tricked into giving pain medicine to people that are being fake or anything. I just go ahead and give them that shot, and we typically know our patients. They may get IV fluids. We might even give them a blood transfusion and warm blankets to warm their bodies because cold is actually a trigger for pain. Now upon disposition, we’ll decide whether they need to be admitted or they go to home, and maybe we’ll bring them back even the next day if we’re open.
Short Acting Opioids Slide 22. The American Pain Society Recommendations for Initiation of Pain Medicine was designed to look at that tolerance I discussed. Their bodies get used to pain medicine. They’ve been hurting all of their lives. These numbers are not for that virgin or that patient that has very infrequent, once a year pain. This is for people who have acute or chronic or are actively taking oral pain medicines at home. Morphine we start at 8 to 10 mgs and we continue it every two to three hours. Demerol, we have about four people left on that. We feel that’s not a safe and effective drug anymore and it has by-products like Meperidine, Normeperidine, which is not a good pain killer, but for those who really, truly cannot tolerate anything else but that and Fentanyl, we have a handful, and we limit them to about 30 doses in the inpatient side and in the outpatient side they might get three doses. We’re fading that out slowly but we do have a few stragglers and they’re in their 40’s and 50’s, so they’ve tolerated it all their lives and we’re not worried about them. So I don’t want you to think that we routinely use Demerol. Dilaudid, or Hydromorphone, we use that quite often, and it’s 0.5 – 2.0 mgs, depending on whether they’re a
virgin and what they tolerate, but 2.0 mg is a good start for severe pain. Of course we like PCA pumps. We don’t do them in the outpatient setting right now but our infusion suite may upgrade to that in the future. Right now, we just let the nurse give the shot and see what’s going on between the pain shots and go from there. While we are treating aggressively, we try not to over treat. That’s what the Pain Society recommends and that’s what we do.
Solutions Best Practice Pain Protocol Slide 23. We do have Best Practice Pain Protocols for our Institution and there’s a copy of what it looks like and, of course, with the electronic record – it’s enfolded in there – but I just wanted to give you a little peek that we do have a Pain Protocol as well as a (cont.) Slide 24. As well, we have a Blood Transfusion Protocol, especially for our pregnant patients. They are special and they are high risk, but we do have protocols that keep us in-check. The nursing staff…they don’t have a lot of wiggle room in that way, and it triggers questions to be asked if they’re confused about what they’re supposed to be doing.
The Difficult Patient Slide 25. Now, I want to switch over to that 5 – 6% of the difficult patients. And, when I say ‘difficult’ – the deviations, the people who actually sell their pain pills, or they are cocaine addicts, they have mental illness, or they may have traumatic brain injury like the girl who came in in crisis with the infarct in her foot, she really couldn’t express herself. She looks like she’s ok but she really is mentally challenged. People with co-morbid conditions, the disruptive patient who is the sociopath and what we call – I hate this term – “the frequent flyer”. Sometimes that person doesn’t have a patient-centered medical home, because we don’t have ‘frequent flyers’ anymore like we used to because of the medical home. We actually have reduced our 30 day readmit rates down to probably about 1%. Our two week readmit rate is the same, which used to be huge; it used to be 30%, so we have made good strides with our infusion suite and our patientcentered medical home. But it’s taken…I’ve been there 30 years and I must say that that’s not something that just happened. We worked that over some years before we got that patientcentered medical home refined so that we are working together in tandem with the ER docs and the hospitalists to make sure these patients are moving to the right place, at the right time, for the right reason, and there are no real premature discharges. Because we’re closed on Saturday and Sunday, we make sure that if it’s Friday, we have to get a good plan going so they don’t bounce back to the hospital.
Barriers to Effective Pain Management Slide 26. So what are these barriers to effective pain management? Usually it’s an incompatibility of patient and healthcare provider. Basically, if the healthcare provider literally sees a young person in their 20’s presenting with sickle cell pain, they really just don’t believe the person, they believe they are exaggerating. But I would share with you that I would rather err on the side of reducing suffering than worrying about being tricked on one pain shot that you
could give in a timely fashion, ok, to someone born with a pain syndrome. I feel that we need to believe the patient. Make sure that the patient knows that you believe them and I promise that you will get that pain under control, and if you’re tricked, we know who they are. So if you have someone who is a deviant call us and we will tell you and we have a way to manage them as well because they also have sickle cell disease so we want to make sure that we take care of them, as well. We worry too much about causing addiction. It’s difficult to cause addiction with someone with cancer pain, I mean, it took 100 years to get them respect. Sickle cell has been in the U.S. more than 100 years now; I think it’s time to start believing them as well, like we do our cancer patients and put them in that category, not worrying about it. You can’t cause addiction by giving somebody a pain shot for pain in a painful syndrome. I would say believe them, give them that first shot, then go back, call us, try to figure it out.
ER Triage Slide 27. Now the ER Triage I respect, the ESI, the Emergency Triage Score Index, may not predict the evolution of the Sickle Cell complication, and I really want to encourage that you look behind that pain and that 72 hour delay. I do talk to Dr. Shantay Smart, our ER Director, about our patients. We go back and forth about, “Well, they look pretty good now.” “Well, the 02 Sat is like 91%. Try some incentive spirometry, see if they improve.” “May be we should bring them in Observation.” There is nothing wrong with fast-tracking a Sickle Cell crisis into Observation to make sure they’re heading in the right direction. The C-reactive protein, the repeat of the lab, the escalation of pain, the evolution of chest syndrome does not necessarily happen at that moment of presentation to the emergency room by your ESI. So I would appeal to ER doctors to give the patient the benefit of the doubt that they actually might be in an evolution into something bad rather than, ‘Oh, they’re fine,’ and send them out because you’re under pressure to move people out. I’ve seen it so many times where the next day or two they’re in a bad way and we have to start all over and they’re worse; so it’s better to actually put them in observation if you know they are a reliable patient.
Attitudes of Physicians and Nurses Slide 28. This last study that showed the attitudes – it was a big survey that went across the US looking at attitudes of physicians and nurses – it’s funny. Some of the hematology doctors actually felt that Sickle Cell individuals were mostly drug addicts and drug seeking and, of course the nurses felt like if they followed the doctors’ pain order that they’re going to “cause addiction”. There’s no such thing – causing addiction for one pain shot. If they’re addicted, they’re addicted. And if anybody deserves to have that word attached to them it’s surely someone who’s had pain all their life that requires pain management and they have the right to have physiological dependence on opiates. We actually deal with that, too. We have deescalation of their pain medicine to make sure that there’s no rebound from that. I teach them about Opiate Withdrawal Syndrome. I teach the pregnant women about Fetal Abstinence
Syndrome so that they know that this pain they’re experiencing might be that they’re off of their pain medicine too soon because if they go home and their pain is resolved and they are one of the younger patients and they just stop their opiates and they’ve been on a PCA Dilaudid pump for five days, they may have opiate withdrawal, and that is a crisis and it hurts. So we actually teach our patients to look out for that and not to suddenly stop their opiates but to wean off and we don’t have a lot of that in our population anymore. So, believe the patient and then if you get their confidence you’ll probably get the truth. What they do is overrate their pain on the 10 Point Likert Scale for Pain Intensity, “I’m a 10.” They might be a 7, but they’re scared to tell you they’re a 7 because they’ll feel you’ll hold back and you won’t treat their pain. So if you give them the script and say, “Is this really the worst pain in your life? You look like you are better…and I promise you I won’t withhold care…” I’ll bet you they’ll take that down to a 6 or a 7 or whatever it is, and that’s still bad pain for them if that’s where they come to the ER. We have patients tolerating 3, 4, 5-level pain every day as they get older.
American Society of Hematology Slide 29. Now, one of the solutions I wanted to talk about is the American Society of Hematology in 2017 has this collaborative effort going on. We have a paucity of treatments since hydroxyurea in 1998, we have not had one additional FDA approved drug for Sickle Cell, but we do have a couple that are coming down the pike. One hits polymerization early and another one we’re going to participate in, helps the anemia. We’re so hopeful and praying that these other two drugs come along because I think that multi-modality treatment is going to be the future for the care of Sickle Cell, along with hydroxyurea, and they will hit the Sickle Cell vascular occlusive disease at this level. And so the Sickle Cell Care Collaboration that’s supported by the American College of Emergency Physicians, along with the American Society of Hematology – and I just left a meeting this year – working together towards one goal which is to get that first pain shot in a timely fashion. Dr. Tabby in Kansas, she wrote a paper looking at that first shot and she was overshooting her goal. She wanted to get that first shot in 20 minutes after presentation. Well that’s unreasonable. At least make it 20 minutes after registration. But of course the three ERs across the country that did this test showed that patients were hours and hours and hours waiting for that pain shot, and not only that, they were dispositioned before their pain was controlled, back home. And, while I know that emergency rooms can be sort of jammed up with other things and the nurse-patient ratio may not be favorable, some kind of way we need to get together with emergency room providers to figure out that first pain shot, may be sit them down somewhere where you can just watch them and not worry about them. But that is a barrier to proper pain care: the emergency room doctors feel like there is nobody to watch them. Our patients are usually not opiate naive and rarely… I haven’t seen a case of respiratory failure, but that’s the fear of nurses and that’s the fear of doctors. But in an opiate tolerant pain syndrome – that’s a rare occasion. I’ll bet you, if you really think about it, it hasn’t happened much with your patients with Sickle Cell in your ER. So my appeal is let’s work together, and if you’re close to a patient-centered medical home, there’s usually a doctor there like me that can talk to you to give you some guidance.
Summary Slide 30. In summary, I believe that Sickle Cell is a really rare and complicated condition. We worry too much about pain and addiction. We don’t like the patients telling us their dose of pain medicines. If they come in and say, ‘I need two of Dilaudid,’ you want to think they’re drugseeking. But if somebody told you they didn’t know their insulin dose for their diabetes you’ll be upset, so we need to get over it. We need to believe the patient. They know what their pain treatment entails, what the dosages are, and just believe them. And then, look behind the pain. Ask, ‘Why is this person having this crisis?’ It might be uncomplicated and you’re doing good. If I could follow them up for you in the next day or two, you could send them out and I’ll re-check them. But if there’s a weekend and you can’t follow them up, sometimes it’s just better to put them in Observation. Evaluate for the vaso-occlusive complications that could be life threatening, believe the patient, treat the pain, and hopefully after this discussion, you’ll feel comfortable to even call me. I’m available and I will have my card and my contact number for you. I will recommend a couple of resources for you. One of them is from the National Institutes of Health. It’s a pocket guide for clinicians for the Acute Complications of Sickle Cell Disease and its Management. You can pretty much go online and see all the acute complications and see what to do and how to evaluate. The American Pain Society I alluded to earlier is a little pamphlet that you can get and it will give you some ideas. I believe that if these are drug addicts and fake individuals, probably only about 5% of the population, please, please think about the 97% of good people, and all of this work is not done for drug addicts. Believe me: we are desperate to get more drugs available but currently there isn’t anything. Reduce suffering. Err on the side of reducing suffering rather than worrying about addiction. Thank you for your time. I hope this was informative and will stimulate you to approach Sickle Cell Disease in a different way.
