Roundtable – Sickle Cell Patients

Sickle Cell Patients

All right, everybody, I’d like to introduce you to our guest lecturer, and hopefully soon up and becoming associate medical director in the future, I hope. Dr. Hefferlin was teaching, taught a lecture for us here a while back called It’s a Baby, and we enjoyed that one, and we asked her to come back and do another one. So my understanding is tonight you’re going to do one over sickle cell, correct? Correct.

Hi, everybody. All right. Yeah.

Do you want to share your screen with us? Absolutely. And this is a topic it doesn’t matter if you’re an EMT student, a paramedic student, an AEMT student, it really doesn’t matter, because there are portions of this that will apply to you no matter what I am sure. So that having been said, thank you for coming back to us, Dr. Hefferlin, and if you’d like to finish introducing, kind of telling us your background, because you know I’m not as familiar with your background, so I don’t normally go through the whole dog and pony show thing, and I’ll let you do it until I learn a little bit more about you.

Sure. So I am currently an emergency medicine fellow at Christa St. Michael’s in Texarkana working with Dr. Phillips, and that’s what I want. Before that, I did a family medicine residency in Corpus Christi, and before that, I was a paramedic in the Chicago suburbs, Joliet area, so that’s about it.

So that’s good enough. I mean, that’s quite a bit of background, but especially that paramedic part, you know, Dr. Phillips was a paramedic before he became a doc, Dr. Frame was a paramedic before he became a doc. I don’t think Dr. Eshelbach was, but he’s been involved from the medical director side for many, many years in EMS from that side and teaching and stuff, so all of our docs are just got some massive background that really helps you relate to our students better.

Yes, absolutely. So, all right, yeah, and doing that was how I kind of knew I wanted to end up in emergency medicine and definitely wanted to come back around and do some kind of EMS direction and give back to paramedics when I kind of got to this point. So here I am, happy to be here.

So today I’m talking about sickle cell disease and our agenda, we’re going to talk about what sickle cell is and then a couple of things, sickle cell pain crisis, how to deal with pain management and then complications that come along with sickle cell patients. So, sickle cell disease is actually a group of disorders and it causes your red blood cells to sickle. So you have your normal red blood cells are this kind of donut shaped, they’re a little flatter in the middle and plumper on the outsides.

Oops. Sorry, guys. There we go.

And then when cells sickle, they turn into this kind of crescent pointy shape and don’t function the way red blood cells are supposed to function. This is due to a variant of hemoglobin. So hemoglobin has different versions.

There’s the most common and normal ones in most adults is hemoglobin A and hemoglobin B. They form in four peptides come together and form one hemoglobin molecule. It’s usually two A’s and two B’s and then a globin and that’s where your oxygen carrying capacity comes from. Each one of those four parts of the molecule have a little nice little pocket where an oxygen sits.

The hemoglobin S, my understanding is it is able to carry oxygen, but once there’s a lack of oxygen, the hemoglobin S misshapes and begins to polymerize. So all the different hemoglobin molecules end up sticking together in one big long chain instead of nice individual molecules that can kind of move around and transfer oxygen back and forth. And then when they polymerize in those big long chains, they cause your red blood cells to turn into this sickle shape instead of a nice plump little kind of donut thing that we think of.

And then once these cells turn into the sickle shape, they die a lot easier and they get stuck in your small blood vessels and it causes pain and obstruction of those blood vessels. And then when they die sooner than they’re supposed to, you get hemolytic anemia. So all of which is no good.

So most of your patients, when you have a sickle cell patient coming in with sickle cell issues, will be coming in with a sickle cell pain crisis. So the protocols are fairly common. You want to check your airway and give them O2, check their vitals, check their mental status, give them pain management, get your IV going, give fluids, and then assess for potentially serious complications, which we will get to in a little bit, because there’s quite a few of them for these guys.

So sickle cell usually starts presenting in patients when they’re about five months of age. And that has to do with the hemoglobin transitioning from fetal hemoglobin to adult hemoglobin. And that’s when the presence of the S hemoglobin instead of the normal A and B kind of makes itself known.

So when you’re seeing these patients, even as 10-year-old children, teenagers, adults, they’ve been dealing with this pain for a really long time. So they have a high pain tolerance and they usually require narcotics and oftentimes quite a bit of them. So you’re going to want to treat appropriately and redose their pain if necessary, depending on the length of your transport and all that kind of stuff, what your protocols allow.

So the reason this is so painful is you have your normal red blood cells and this little donut shape can fold very nicely and get through smaller spots. The sickle cells don’t do that. They’re stickier than your regular blood cells and they’re just awkwardly shaped and kind of terrible.

So they clog up and cause occlusion. And what does occlusion cause? Ischemia. And as we all know, we think of ischemic events like ischemic limbs and heart attack.

Ischemia causes pain. So don’t think that they’re trying to fake it or paint drug seeking or anything like that. Sickle cell people really do hurt a lot.

Okay. So potentially serious complications. There’s like I said, quite a few of them.

Sickle cell patients are, because of all this occlusion that they have going on, are prone to things, acute chest syndrome, more prone to strokes, meningitis, septic arthritis, splenic sequestration, and more prone to shock. So and we’ll go into each one of those individually. So acute chest syndrome is classified with hypoxia, chest pain, and fever.

So for you guys, you want to give it O2, like I said, pain management, like we talked about. You’ll have various pain management protocols depending on where you end up in your system. Tylenol for fevers, if you’re allowed to give that.

The other things to look for is wheezing, cough, and respiratory distress. Obviously, respiratory distress, you go through that algorithm, you increase the O2 as necessary if you have to intubate them, try to avoid it. But if you have to, you have to.

With the wheezing, you want to treat them as if you’re treating for asthma. They are much, much more prone to have reactive airway disease and wheezing and asthma-type symptoms. Much more so, up to tenfold more so than your everyday population because of these sickle cell occlusion that causes chronic inflammation and reactive airway type of issues.

And stroke, again, they’re more prone to stroke. So make sure you’re getting a good neurologic function exam on your patients, NIH stroke scale. And then, you know, if you’re finding neurologic deficits, follow your appropriate stroke protocol and go to the nearest stroke center.

A lot of dealing with sickle cell is, you know, having these things on your radar and knowing what to, you know, look for and treat. It’s not necessarily like a sickle cell stroke is going to be different than any other day stroke, but you just want to have a higher suspicion of these things in these patients. Meningitis, same thing.

Do what you would normally do for a meningitis patient, but you just want to be a little more wary that this could be happening in your sickle cells. So if they have headache, if they’re altered and they have a fever, same thing. Go through your altered mental status algorithm.

Tylenol for fevers and pain management for, you know, their headaches and the pain that’s going on. Septic arthritis, if you have fever and severe pain in a single joint. Oftentimes a septic joint will be very red hot, inflamed, angry, but not always.

So, again, Tylenol for fevers if you’re allowed, pain management, you know, get them to the closest appropriate facility. Septic arthritis can be very dangerous and generally requires surgery. And much like any other kind of infection, inflamed area, don’t stick a needle through the infection area, it’ll just make things worse.

Next, splenic sequestration. So this usually happens in the younger sickle cell patients when they’re children. It generally happens over fairly, I don’t want to say frequently, but it happens.

The more often it happens in these young children, the spleen often, what we call auto-infarct, so it keeps getting these occlusive episodes and eventually the spleen itself just kind of dies off and then they no longer have any splenic function. And so adults often don’t have any functioning spleen in them and therefore don’t have splenic sequestration problems because their spleen is effectively dead and gone by that time. So in younger patients, left upper quadrant abdominal pain, splenomegaly, hypotension, and tachycardia are kind of some of the signs you want to be looking for.

So pain management, if your vitals allow, because remember, you’re going to have some hypertension, so you don’t want to drop the pressure even worse. IV fluids and, you know, as normal, travel to the appropriate facility. And shock, again, you want to take them to the closest appropriate facility and treat as your normal shock protocols.

It’s not different shock than a regular patient, but, you know, they are much more prone to going into shock. And, you know, a couple additional pearls and here’s why we’re much more prone to going into shock. So because of their anemia, their hemolytic anemia due to the sickling, they tolerate blood loss more poorly than your normal patient.

They’re already anemic. And, you know, any loss in ability to decrease oxygenation causes cells to sickle, which causes their pain, causes an even decreased ability to continue oxygenation and et cetera. And then not just, you know, your meningitis and your septic arthritis, sickle cell patients are more prone to infections in general.

And so I think that’s a very short lecture today. So if we have lots of questions, I can definitely go through and answer some more stuff. And I have a question.

Yes. So whenever we’re doing pain management for people with sickle cell, a lot of the medications that we give are usually intravenous. I’ve noticed that a lot of the times I try to do IVs on patients that have sickle cell, it’s near impossible to find a vein.

And I usually find myself just blind sticking and hoping for the best and trying to get something. Is there a specific, like I know we can, there’s certain medical, we can get fentanyl like intra or not. Yeah.

Yeah. Fentanyl intranasal and stuff like that. Would that be the better route for it if unable to get anything or.

Yeah, definitely. They are, like you said, usually hard to stick. And, you know, however, you can get the pain medication to them.

You know, if you have protocols for intranasal or IM pain medications, then, yeah, definitely go ahead and do that. Don’t hold back pain management from them because you can’t get the IV. I’m going to make a shameless plug for my presentation coming up on Tuesday or Monday, and that’s actually going to be about pain management and some guidelines that have recently come out.

But I would tell you that, yeah, IIN is definitely. I think we lost you mid-sentence, Doc. That was me for a second.

Dr. Phillips, we can’t. There we go. Oh, there you are.

Same thing with the headset again. What I would say is that IIN is definitely a good option in these patients. Obviously, IIN or intramuscular is not necessarily preferred.

But if it’s your only option, then certainly you can do that. The other option would be certainly intraosseous. If you really need to get a line on somebody, just follow the protocol.

Be sure and give them some lidocaine as soon as you start that because the insertion itself is not as painful as pushing fluids into that bone is. So in the situation to where you deem that IO would be necessary, how far into a sickle cell crisis would you deem that if you can’t get IV, you’re like, all right, now I have to IO this? Well, definitely if they’re in shock. That’s obvious.

I think that it depends on if you have intranasal available in your protocols, then I think that you can certainly redose them intranasally. Keep in mind, and I’m sure that Dr. Hefferlin was going to bring this up, these patients can require pretty massive doses of opioids because they’re opioid resistant. They have had these usually their entire life, and so they get them on a regular basis.

Many of them have prescriptions for high-powered oral pain medications that they take on an ongoing basis. Don’t be afraid of going ahead and giving them more. Certainly, you’ve got to monitor the respiratory status.

Of course, and they’re not going to be calling you until those high-dose orals that they get aren’t working. I actually have a friend who is a radiology tech that has sickle cell disease. If Jane or anyone would like to hear from that perspective, she said that she is always willing to come and speak to anybody about sickle cell disease because she actually has that.

Now, Dr. Hefferlin, I didn’t hear you bring it up. There’s actually two different forms of this sickle cell disease and sickle cell trait. I don’t know if you can elaborate on that a little bit.

Sure. Sickle cell, that’s kind of going back to the hemoglobin S and going into genetics. Sickle cell trait is when you have one gene for the hemoglobin S, and sickle cell disease is when you have two, I believe.

Sickle cell trait, you have much less of the hemoglobin S in your system. It is possible to have sickle cell trait and still cause yourselves to sickle and get into a pain crisis, although it’s much less common. Generally, sickle cell trait is considered a benign carrier.

It usually doesn’t manifest itself in these patients, but it is possible, I was reading. They then in turn pass it on to the next generation. If two trait people get together and their children could possibly have sickle cell disease if they both pass on that hemoglobin S gene, and then the child ends up with the two hemoglobin S’s.

The sickle cell disease is the patient that has the two hemoglobin S genes, and so they create this hemoglobin S instead of the hemoglobin B. Like I was saying, a normal molecule of functioning hemoglobin is two A’s and two B’s, so you end up with two A’s and two S’s that then misshape and turn into these sickled cells. Could you also comment about oxygen therapy for these patients? I did. You want to give them oxygen.

The protocols say nasal cannula oxygen for these patients. You want to start them on oxygen. This is pulling back memories from way back in the day.

At some point, I remember them saying that in vitro, they were able to reverse sickling by high doses of oxygen, but if I remember correctly, they have shown that in vivo, you can’t reverse the sickling. However, because sickling is caused by lack of oxygenation, I think the theory is by providing them with oxygen, you’ll prevent further sickling of cells. Also, especially if they’re hypoxic and having acute chest syndrome and that kind of stuff, you really need to keep them as well oxygenated as possible.

Exactly. Do you shoot for the same pulse oximetry levels that you would for any patient, 94 to 99%, or do you shoot the upper end of that? That was actually the question I was about to ask as well. The answer is, ordinarily, with the protocols that we’ve come out with lately, and there’s another good lecture as well from the BMJ infographic about oxygenation, sickle cell patients are not included in that particular restriction.

We’re actually shooting to just keep people above 93%. In general, if they’re 94 or higher, we just kind of leave it alone. These people, because they already have impaired oxygen delivery due to the sickle cell not releasing the oxygen as efficiently, we want to try to increase the oxygen that may be dissolved in the serum.

In these folks, it is actually recommended that we go ahead and give them supplemental oxygen, even if they’re already sitting at 98 or 99%. There’s a question over here. When it comes to trauma in these patients, for hemorrhaging, I assume she meant hemorrhagic shock, does sickle cell complicate matters more or differ your treatment than it would be in a patient with calcium cell or with a department that carry blood products on the end rods? In trauma, it doesn’t necessarily alter your treatment.

You’re going to do the same things that you would with a non-sickle patient. However, like I was saying, a less amount of blood loss will cause more problems for the patient. They’re going to go into shock more easily.

They’re going to tolerate that blood loss less well. I don’t want to say that. They will not do as well losing the same amount of blood as someone else.

So what seems like minor traumas or doesn’t seem like too much of a blood loss in a sickle cell patient can cause more damage and more systemic problems than your average Joe Schmo. So from that aspect, you want to be more on alert for treating the shock and being wary of being prepared to deal with those consequences and to give blood if necessary. As far as transfusing blood, the hemoglobin does not – let me check if that’s right.

They do require blood transfusions occasionally. And I don’t think being sickle cell affects their ability to receive transfusions of like Oneg for trauma blood because the hemoglobin is not one of the antibodies that reacts and that causes transfusion reactions. So they can and should receive blood if it’s called for and may likely need it sooner rather than later than some other patients.

Yeah, I agree. I would have to actually look at our transfusion protocol. But I believe that actually sickle cell patients may actually have a higher hemoglobin level where they meet the criteria to receive a transfusion.

And that has to do with the fact that their cells do not release the oxygen as efficiently. And so we’re talking about that there is tissue level damage that is not going to be apparent until later on. And so we want to try to keep in mind that pressure is really an important thing.

You want to keep that MAP higher in them if possible just so that they are delivering oxygen and other vital needs to all of the cells and be in tune to provide fluids a lot earlier than you might others. Excellent question, Nicole. Fun fact, it’s not entirely relevant.

They found that people with sickle trait and sickle cell are protected against malaria, which is why they think sickle cell has kind of evolved and lasted as long as it has in patients because there is some protective value to it. That’s interesting. I have a question or something to see if you can elaborate more on why.

And I’m sure I understand why, but I think it may be vague for the class. Why they’re more prone to meningitis. So they are more prone to infections in general.

I’m not sure that I know the exact mechanism of infections being more prone. They’re especially more prone to encapsulated bacteria because of the autosplenectomy. Gotcha.

Yes. And even if they haven’t totally infarcted their spleen, they still have impaired splenic function. That makes sense.

Samantha asks, I have been told that persons living with sickle cell reduces life expectancy. If so, why is that? So along with having all of these micro ischemic events, the sickling also causes damage to the lining of the blood vessels, which increases plaque formation. And so they’re more prone to things like, you know, heart disease and the risk factors for all of these hypertension and all that kind of stuff is higher.

And so they are, you know, in general, more prone to getting more of these things that cause death earlier, I think, is a big part of it. And then, you know, being anemic, being less protected and with, you know, this autosplenectomy and all of that kind of stuff, they’re just much more prone to getting all those terrible things that make people not live as long. I guess earlier we’d spoke on IM versus IM versus IO.

And to kind of scale this down, I guess would be the best way to go at it. You’re in Texarkana. I’ve worked out of the Longview, Texas area.

Transport times in and around where you are can be anywhere from 10 minutes to 45 minutes to flight for an hour and a half, things of that nature. Sickle cell crisis, primarily focusing on IO. Unless we’re real, real deep into this, I mean, like full-on sepsis, bad, this has gone very far downhill.

What’s the justification in going IO with this sickle cell patient, depending on where you’re at? I mean, per my thought, unless we’re really bad off, am I going to IO a sickle cell patient when, say, I have a 15 minute or less transport strictly for pain management? What’s your thoughts on that for how we escalate this? That is a good question. And I mean, if you have intranasal or intramuscular something available, I would say probably not. But I don’t know, Dr. Phillips could probably answer that better than I can.

What I would tell you is that I would reserve IO for somebody that is in shock or near shock. And especially along transport time. I believe that if you’re 15 minutes from us, the intranasal would probably be very appropriate.

And once again, Tuesday, we’re going to discuss a lot of those same issues because it’s already been, the science on it has been thoroughly reviewed, at least to date. And of course, those things change. But there are not really, in my mind, a great justification to put an IO in somebody if you’re, you know, 10, 15 minutes from the hospital.

And you can get them there quickly and you have other methods to deliver pain control. Certainly, you know, worry about, you know, if they’re volume depleted or something, that’s a different matter. If you have somebody that their map is 60, then certainly put the IO in, even if you’re, you know, 10 minutes from the hospital.

Just go ahead and get some of those fluids started. But yeah, that’s an excellent question, Kyle. Thank you.

Thank you. Can I jump in here for a second and correct me if I’m wrong? Also, one of the things you have to keep in mind, and it’s also in the pain management class to a degree, would be environmental effects on your patient. You know, you get out in here and these patients, you’re going to have them out in the cold in the wintertime, trying to get them to the box.

Maybe the back of the box is not warm enough or your heater’s out or not working effectively in the back of your box. That’s going to make their pain tremendously worse due to the vasoconstriction from the cold. So you’ve got these sickle cells that are occluding these vessels anyway, and maybe a little bit of blood is getting by.

So maybe simple things, not just oxygen, not just, you know, but the simple things over and above or even before some of these drastic measures for pain management meds might be, you know, just as simple as keeping them, wrapping them in blankets if it’s cold and turning up the heat in the back of that box during cold weather and not letting, and even on the opposite end of the spectrum is, you know, we’re hot and sweaty because we’ve been jumping in and out of the Amos. We’ve got that wonderful sub-zero air conditioner in the box that’s blowing crazy too right on them. That’ll do the same thing.

So keep in mind, sometimes simple comfort measures can go a long way as well. Absolutely. And cold stress can actually precipitate a crisis in somebody that’s not in crisis.

So that’s an excellent point. One other quick thing, the sickle cell trait, definitely those people can have crisis. While we think of it as, you know, relatively benign, they can and do go into crisis.

And they will have sickle cells on their peripheral smear if we do those in the hospital. So, you know, keep in mind that just because they have trait, you know, don’t think that they’re faking any of the things. And I would tell you that in this, you’ll hear it again on Monday, is that if you, if you think that somebody is having pain, err on the side of giving them pain medicine, because, you know, I would much rather that I give somebody pain medicine.

And, well, I’ve gotten fooled. So what? I really don’t care. And I’ve been in that club that has said, I’m not going to let anybody fool me.

You know, they’re just drug seeking. Well, you know, it’s much better to err on the side of going ahead and giving somebody some relief from their pain than it is to make somebody suffer needlessly. Right.

And Samantha has another question over here and understand her wording. It might be a little bit different than ours. She says she’s never had a patient with sickle cell persons who are living with sickle cell.

Are they always in pain? They’re always saying what you’re saying is that they’re always in pain. What’s hurting them all the time? And that really depends. Some patients kind of they’ll be able to tell you, like, yeah, I always feel it in my legs and it’s really bad today.

Or some patients are, you know, will come in and, you know, they’re just going to have their areas that tend to hurt more. Some patients is just generalized pain everywhere. So and.

Also, a lot of these people will also along with having, you know, organ infarctions, they’ll also have bone infarctions. And so, you know, I would if if at some point, Jane, if you would like to, like I said, this this x-ray tech friend of mine, she has this and she has had to have a hip replacement in her 30s because of a vascular necrosis in her hip. And she has a vascular necrosis in the other hip.

And she’s also got some bone on bone changes in her knees because of her sickle cell. So these people do have chronic pain. And it’s usually all day, every day that is being managed chronically if they’re compliant.

Now, obviously, we have a lot of people that are not compliant with their with their pain management or with any care. And those are those are a different factor. But that’s an excellent question.

And so and it’s not a weird question at all. It’s actually a very good question. I’m glad that you brought that up because these people do have pain.

And I think unless we hear from somebody that actually has this trait or disease to kind of hear their perspective on it, not somebody that we’re kind of thinking is sketchy. I think that that’s something that is an excellent perspective to ask. I think that’s a great idea.

I think I would love to have her talk to her and see if she’s willing to shoot me an email. And maybe we can set something up where we can have a question and answer session with, you know, someone who has sickle cell and then let her pick their brain. And since she’s a healthcare provider, that would help us a lot because she’d understand what we’re after.

We have some more questions over here. So if Matt asks, so if they just have SC trait, are the symptoms going to be as bad as if they have the disease? They can be, yeah. They do have the sickle cells.

And like we were saying, they can go into crisis. So the symptoms can be, you know, every bit as painful and dangerous as your sickle cell disease. And Ashley says or asks, so with the spleen being damaged or non-functional, what does it do to your immune system since the spleen plays such an important part of the immune system? So, like Dr. Phillips was saying, particularly the encapsulated bacteria become more of a problem because those are the ones that the spleen deals with.

So, and I should know more about which bacteria are encapsulated versus not, but plays a role in meningitis and some other diseases caused by these bacteria. They’re more prone to them because they don’t have as much ability to fight against them with the spleen. And then I believe the spleen also plays a role in like filtering and like holding blood, like a reserve of blood.

So that’s also diminished. So I guess that also means that in our pre-hospital environment and emergency environment with sickle cell patients, we need to always keep a, like do a sepsis assessment. A high index of suspicion, no matter what’s going on, evaluate them as well for septic symptoms as well.

Correct. Yes. Absolutely.

So the main encapsulated bacteria that we worry about are strep pneumonia and haemophilus influenza. And those are the ones that can cause a really bad disease, especially the haemophilus influenza is implicated in a lot of bacterial meningitis. We don’t see it very often now because we give the HIV vaccine, haemophilus influenza B vaccine to kids.

And so, especially if you’ve got a younger patient that has sickle cell disease and they’re not vaccinated, they’re going to be much more prone to that. Dr. Phillips, two part question. Not to beat the I.O. horse to death, but what you just followed up on with the, with possible bone infarction and things being seen more in these patients.

I’m assuming, especially your elderly ones as this, as the disease has progressed or they’ve had it for longer and it’s been no longer. Do I have any contraindications or concerns with I.O., microfractures, infiltration, things of that nature being that bone deficiencies could be a side effect of sickle cell as a whole? Well, it’s not that they have hyper or hypocalcemia in those areas. It’s not a calcium problem.

It’s a problem with the vasculature. Now, keep in mind that we’re putting this into the bone marrow. And so this is actually where the blood is produced and it’s coming from the integral system.

So, no, it really is not a concern. Obviously, if they’ve had a joint replacement down there, you know, let’s say that they’ve had total knee replacement, then, you know, that’s probably not the best site for you to use because you may end up hitting the prosthesis, depending on how long the stem is. Yeah, that’s an excellent question.

Really is. Nicole has a good one. I’m sorry.

You had a second part. No, the second part of that is with the, with the viruses and infections you had mentioned, with the HIV and things of that nature, with COVID being such a prevalent thing in today’s society, is sickle cell in that high list of comorbidities that we’re seeing around COVID and things of that nature? Or has there been any research on that that you know of? You know, and really, I haven’t read any research papers where they talk about the incidence of it in those people. Certainly, they’re more prone to certain infections.

But honestly, I can’t say that I’ve seen more sickle cell patients with, with COVID-19. And Dr. Hefferlin, you certainly are seeing patients there too. I can’t say that there’s a higher prevalence.

And I think part of that is just simply the infectious mechanism of COVID-19. It’s not an overall infection. It attacks certain cells and primarily those cells are not going to be on the blood.

So I can’t say that I’ve seen an increased incidence. I don’t think I have either. And I, and I don’t know that I’ve seen more sickle cell patients having, you know, crisis or issues or anything like that, even.

No, in fact, I think, I think we’ve seen less and part of it is probably don’t want to come in for fear of contracting COVID-19. Right. Thank you.

So now, Nicole, are there medications that are commonly used in EMS that have contraindications specific to patients with sickle cell? Or ones that will worsen their pain or condition, for example, vasodilators or medications that can cause vasoconstriction? Basically a medication that you might think would be appropriate to give, but specifically you can’t to a patient without sickle cell. No, excuse me. The vasoconstriction could be problematic because like we’re talking about with the cold and causing those vessels to constrict and cause a crisis.

However, if you’re at the point where you’re giving someone vasoconstriction, it’s usually because they’re in shock and need that vasoconstriction to support their perfusion. And at that point, that’s more important than the potential additional pain that we’re going to cause is my thought. Because that, you know, that needing that perfusion, especially if it’s, you know, because we’re altered or in shock or, you know, not perfusing vital organs, that’s, you know, life threatening and that needs to be dealt with if, you know, if it doesn’t work with fluids and blood and that kind of stuff.

Nicole, there’s basically very few medications that are commonly used that can precipitate a sickle cell crisis. There are a few, but they’re not going to be in the back of your truck. Again, excellent question.

Really good questions. Kyle, push nose pressers and sickle cell crisis, question mark? If you need them, yes. Short answer.

Umang, I know you don’t need an MD roundtable and you bumped in late. Do you have any questions? Hey, sorry. I was on a call.

I’m in my clinical right now for actually in mineral wells. Actually, I don’t, I’m thinking about some questions, but I did have a patient today. You were talking about the pneumonia vaccine, correct? She did, I got her history and she did mention she had sickle cell trait, but she had a wide variety of issues.

She had, I’m sorry, I’m trying to remember all her, she had COPD, history of hyperlipidemia, history of hypertension, and she said she also had chronic pain. So this was actually earlier today. So I think it’s kind of funny that you’re talking about sickle cell, but she had sickle cell trait and she was a 67 year old woman.

But upon, she told me she had chest pain. So we put a 12 lead on and her SpO2 levels were at 92. So I didn’t give, we didn’t give any medication.

We just started an IV and her vital signs, her blood pressure was a little bit high. And she was complaining of chest pain or not chest pain, shortness of breath. So we just put her on oxygen and started her on, actually, yeah, we just gave her oxygen.

There’s not much we can do in that situation, especially if she has sickle cell trait. For people with sickle cell trait, are their SpO2s usually going to be like running a little bit lower than, even if they also have COPD, like are they going to, she says she’s normally at around like 86. So is that normal or is that something to be wary of? I’m sorry.

No, not at all. So that’s, the COPD definitely complicates things. I would guess that her COPD is what’s causing her SpO2 to be a little bit lower.

The sickle cells carry oxygen, but they don’t release it properly. So you don’t usually get low oxygenation unless there is like an acute chest crisis or something like that, like causing damage where the cells that are normal can’t oxygenate well, or there’s just so many of them that the tissues aren’t perfusing because your SpO2 is measuring the oxygen carrying capacity of your red blood cells. So, and that should be normal in your sickle cell patients, even if they’re sickling.

And so it’s not always the most reliable while they’re, not that it’s not reliable, it’s not, you know, they’re, you’re going to want to give them oxygen, even if their numbers look good. Okay. To give them that extra boost of oxygen in their system.

But yeah. Um, because she’s, even though her SpO2 was, um, at her normal baseline, she was complaining of shortness of breath. And she was always like a little bit, she told me she had chest pain.

She had a normal sinus rhythm though. Um, so we just, we gave her very little oxygen. Um, but I was also kind of worried because she told me she had a history of pneumonia and she had some diminished sounds on the left side.

So I was wondering if there are any other interventions that you would recommend for a patient like that? Um, gosh, that’s tough because, you know, she does have a lot of kind of variable things going on. Let me ask one quick question. Yeah.

Did you get an end tidal CO2? Yeah. Uh, so her end tidal was hovering between 40 to like 47 throughout transport. So certainly, you know, the, since the, you, you, Dr. Heffernan hit the nail on the head, you’ve got a lot of diverse sort of pathology that’s working there.

And so, you know, in this particular case, monitoring your patient is going to be the number one thing. And that, that waveform cabinography is something that I wish we used more in the hospital. And we don’t.

And that’s bad on us. But, you know, this is a patient where you really need to say, okay, I need to deliver oxygen because she has sickle trait and I don’t want to put her into sickle crisis. And she’s saying that she’s short of breath and those patients, usually it’s because they are slightly hypoxic because it’s a hypoxic drive in COPD patients.

So let’s give her a little more, but we kind of got to watch her end tidal CO2 because if that starts to rise, we need to back off on the oxygen because obviously airway over, over circulation, A before C. But you kind of got to treat all of those things. I mean, it’s, it’s actually a very good case because there’s so many different things at play in that particular case there. Good job.

Kind of why I got apprehensive about, I mean, it was a short transport. That was a good thing. So that’s the only reason we, we started an IV, but we, I didn’t want to push fluid just because of the fear that she did have pneumonia.

Correct me if I’m wrong. If she has pneumonia, that could be fluid within the lungs. I don’t know if that could kind of cause like more issues in a pneumonia patient.

I don’t know if I’m saying, wording that correctly, but Well, think about it from instead of pneumonia. Think about it from an infectious perspective and what’s the worst infectious thing that we can have is sepsis. So, you know, you, we, we’ve had this discussion at the hospital with our nephrologist and also our intensive care doctors.

And their thought is that rather than hypoperfusing anybody, allowing their MAP to go too low, they would much rather that we go ahead and give them the fluid so that we keep the MAP up, even if that requires that we have to put them onto a ventilator and then dialyze them. Now, you’re not going to do that in the field, but if they need the fluids, give them the fluids. And so that would be first and foremost.

You agree, Dr. Hepperling? Absolutely. So, We can always take the fluid back off if we put in their lungs and like Dr. Phillips was saying, Pneumonia is more of an infectious process and You kind of think of it as fluid in the lungs. It’s not fluid like a CHF fluid backup of pulmonary edema in the lungs.

So, You know, you may, you also bring up an interesting point that you didn’t even realize that you brought up. Since I live in that area, mineral wells in that area. You’re not going to be able to do dialysis at PPGH.

So if you’re taking them to Palo Pinto, Yeah. Then they’re in, you know, once again, it would still be preferred that you perfuse the patient than anything else. And if they have to transfer the patient to a place that has dialysis, in that case, you’re talking to Actually Weatherford now has inpatient dialysis, then that would be preferred.

But this is where, regardless of where you are, you should know the capabilities of the hospital that you’re going to be transporting to. So that you can decide whether or not that truly is the most appropriate facility. I see.

Okay. Yeah, because we did take her to PPGH and she was put in the fast track unit due to possibility of infection. Her husband did have COVID.

So we were kind of, we let the hospital know about that, the hospital staff. And so we put her in the fast track unit. And so they immediately put her on the 12 lead also once we transferred her, but That’s what we suggested we did, but From what it seems like, correct me if I’m wrong, I’m thinking about sickle cell as like a potential clue, like in history taking.

So like Because there’s not much we can do in the pre-hospital setting besides try to manage symptoms, not symptoms, but like vital signs and manage the MAP, right? So like I’m thinking about Asking patients like, oh, do you have sickle cell trait? And if they do, like that could be a potential thing that can exacerbate these bacterial infections, right, like pneumonia or even influenza. So that’s, am I correcting that assumption or am I getting it wrong? Yes. Yes and no.

It is, you know, they are more prone to all kinds of infections, more so bacterial than necessarily flu. But I don’t, I don’t know that asking every patient, hey, do you have sickle cell or sickle cell trait is something that I would You know, add to my history taking, you know, most patients who have it will know it and tell you. So I don’t This is part of the newborn screening as well.

So every state has a panel of tests that they do on newborns and sickle cell is one of those that they automatically do. And so if they have sickle trait or sickle disease, they’ve lived with it their entire lives. Believe me, they’re going to tell you.

Okay, that makes sense. Okay, we’re coming up on nine o’clock. Has anyone got any last questions before we close the room? It’s just been a great discussion.

Good topic, Dr. Hepperlein. Thank you. Thank you, Dr. Phillips for giving it to me.

We’ve heard from everybody, Dr. Phillips, I believe. Let me run down the list one more time. Yes, everyone tonight has participated.

Excellent. Well, if nobody else has got any questions, I think we can close the room and Dr. Hepperlein, you are so welcome to teach for us anytime. We really enjoy it.

You’re a fresh face. It’s kind of fun because you have a different perspective. Awesome.

Well, I appreciate it. I enjoy coming and talking with you guys. So, well, anytime you want to do another one, just let me know, you know.

All right, everybody. Good night. Remember, we’ve got Dr. Phillips will be back on Monday, even though it’s a federal holiday.

We’ll still have a roundtable, but that office will be closed officially, but we’ll still have a roundtable. Jane, I’m probably going to send you the reference material. And can we just go ahead and send that out to all the paramedic students so that they can read up on it ahead of time? And then that way, you know, the slides themselves are kind of boring, but that way we have more time for discussion and they can formulate their questions ahead of time.

Well, I really have no way to send them all out to everyone. And I don’t know who’s going to be in the room. It’s going to be there.

It would be a mass email thing going out that could get us blacklisted. So I’d rather not do that. I can.

I don’t know if it’ll allow me to do attachments and student forums. I’ll have to look and see. I don’t know if it does.

Yeah, or if there’s a student forum. We have a student forum, but I don’t know if it’ll let me put in attachments. Send it to me and I’ll see if I can.

Well, or, you know, you can put my email and I can forward it to them. Yeah, I guess I could do that and say, if you’re planning on being in Roundtable on Monday, email Dr. Phillips for the documents ahead of time to study. Well, yeah, and put what the topic’s going to be.

Yep, will do. All right. You’re going to be doing pain management, correct? Yes.

All right. Thank you. Jane, can you send me the link to that Roundtable too so I can listen in? Yeah, and Dr. Phillips, if you’ll help me remember in case I have a brain damage moment, but I think I can remember that.

I’ll forward it to you as well, Katie.