Palliative Care in Sickle Cell: Craig Blinderman, Stephanie Kiser, Eberechi Nwogu-Onyemkpa

Eric 00:11

welcome to the GeriPal Podcast. This is Eric Widera.

Alex 00:12

This is Alex Smith.

Eric 00:14

And, Alex, we are going to be talking about the role of palliative care in sickle cell disease. Before we jump into that topic, who are our guests today?

Alex 00:23

We are delighted to welcome three guests today. First, Stephanie Kiser, who’s a palliative care doc at Massachusetts General Hospital and Harvard Medical School. Stephanie, welcome to the GeriPal Podcast.

Stephanie 00:34

Thank you, guys. I’m excited to be here.

Alex 00:36

And we’re delighted to welcome back Craig Blinderman, who is a palliative care doc and chief of supportive care at Memorial Sloan Kettering Cancer Center. Craig, welcome back to the GeriPal Podcast.

Craig 00:46

Thanks so much.

Alex 00:48

And we’re delighted to welcome Eberechi Onyempa, who is a palliative care doc at Washington University in St. Louis. Eberechi, welcome to Jerry Palmer.

Eberechi 00:57

Thank you. A pleasure to be here.

Eric 00:59

So, Eberechi, I think you have a song request for Alex. Before we jump in the topic, what song did you request?

Eberechi 01:06

Yeah, I chose thank you for being a friend, which we all know as the theme song for the Golden Girls. I grew up with three older sisters, and growing up, we will binge on the Golden Girls. And this was just like a staple childhood memory for me, but kind of bring it back to palliative care.

I think thinking about this, the work that we’ve done in palliative care and sickle cell disease really has been just fostered by the relationships and the friendships that I’ve formed along the way, from Craig, who was my former program director for fellowship and also the director of adult palliative care at Columbia, to Steph, who I met. And we’ve done so much work on this space. I think really the relationship that we form in palliative care has really fostered the work that we are going to talk about today. So thank you for being a friend to everyone.

Alex 01:53

I love it. Great choice. Here’s a little bit.

Alex 02:01

(singing)

Eric 02:52

That is awesome. Thank you. That’s a great song. And we were going to be talking about palliative care and sickle cell disease, but I also. I may ask some questions about what’s the role in geriatrics as well, but maybe just let that one simmer for a second. Since we’re talking about the golden girls, I’m going to actually take a big step back and ask you a question. Like, this has been your research focus. What got you interested in sickle cell disease and the role of palliative care in that?

Eberechi 03:25

That’s. That’s a great question. So I was born in Nigeria, and as a young girl growing up in Nigeria, there were two diseases I knew about from a very young age, malaria and sickle cell disease. Like, from a very young age, everyone knew about those two, and we knew that people with sickle cell disease often did not live past childhood. So a lot of my childhood friends who have sickle cells, sickle cell disease, did not survive to adulthood.

So I came to the US Went through my training, and then I came across Craig Lindemann. And during my fellowship training, he had a pretty good panel of individuals with sickle cell disease that he saw his outpatient practice, and I just saw the compassionate care that he provided to this patient. And it sparked my interest in this. In this space. And then fast forward to my first job in Lansing, Michigan. I moved out from Washington Heights to Lansing, Michigan, and I was an attending physician. We had a consult for sickle cell disease, and I was like, oh, yeah, sickle cell.

I want to see the patient. They were like, oh, no. Sickle cell is outside the scope of practice of palliative care. I was like, whatever do you mean by that? And that just kind of sparked my interest in trying to say that people with sickle cell disease do have true palliative care needs. And really, we need to be aware of these needs and step up as a specialty to provide the support and care that they need to improve the quality of life.

Eric 04:43

That’s a short version, and it’s interesting, but malaria and sickle cell disease, which is interesting because sickle cell disease is protective from a malaria standpoint. But outside of that, and we’ll be talking about that, there are things that come up, which we’ll be talking about that, where the palliative care needs come into play. Craig, you also had a panel, it sounds like, of patients with sickle cell disease. I don’t think that’s actually fairly common in the palliative care world. Tell me how you got Interested in this?

Craig 05:16

Yeah, sure. So it goes back to my fellowship days where I was in both a pain medicine and palliative care department. And so we actually had a sickle cell clinic. We had several clinics for our palliative care fellows. We had an HIV AIDS clinic, pain clinic, we had a sickle cell pain clinic, and then we had a cancer pain clinic. So we had multiple sort of sites where we would be, you know, working with patients that had serious illness or what we thought of as serious illness. And so I thought that was.

That was actually pretty unusual. I don’t think that’s a typical palliative care training. And I really, you know, I really fell in love with working with that population. I remember one of my memories is we used to have these music jams actually once a month, where we would invite our sickle cell patients to bring in either instruments or we would provide some instruments for them, and they would just jam, you know, play drums, you know, different rattles, things like that. And I was just, like, just in awe that this was even possible, that we as healthcare providers had the privilege of, you know, being part of this.

And I, you know, owe that to also to Lauren Shioba, who’d done some really interesting research using opioids to treat sickle cell pain for patients who are having vaso occlusive crisis. And I was like, oh, you can do that for, like, the long term, and indeed you could for, you know, with chronic opioid therapy and actually show that you can decrease some of their hospitalization. So I was kind of early on thinking, like, well, maybe there is a role here for palliative care. And then when I took this position at Columbia and running the program there, we had a large sickle cell population in Washington Heights.

And our hematologists colleagues were actually quite good at pain management, in my estimation. But where they got to their discomfort or their, you know, they started to feel like they couldn’t handle the same, or they would start referring mostly to myself and to maybe one or two other providers. And it started to turn into an actual program. And we saw that we were seeing their suffering as more than just their pain and starting to see it similar to the way we were looking at clients, some of our cancer patients and others with serious illness.

There’s a lot of psychosocial determinants of health that were impacting their quality of life in ways that were very profound. There’s obviously a lot of systemic racism that was being uncovered and being a part of why they weren’t getting adequate pain treatment. And so just bearing witness to all of that, it seemed like we were the right fit, namely our interdisciplinary palliative care team were the right fit to meet some of these needs. So I took that very seriously as something that, that, that we should be part of, that we should be part of the solution and not be like, well, that’s someone else’s problem.

Eric 07:59

Yeah, great story. Stephanie, how about you? How did you get interested in this?

Stephanie 08:03

Yeah, I love these stories because we’ve all come to it in different ways, but I think we share the same passion. And you know, for me, I did med peds training leading to palliative care. So we all came from different sort of disciplines and that was my path. And coming out as a, you know, bright eyed, bushy tailed, attending, taking my first job, I think I had this passion for the patients I had seen on both sides who have chronic disease their entire lives and have incredible morbidity and early mortality, but yet don’t get a lot of palliative care.

And sickle cell was certainly one of those. You know, there are others. But around that time, also at Mass General, we had a hematologist come and join Mass General Charles Azar, who is a leader in this field and very forward thinking. And he came to our palliative care group and he said, I need palliative care in this clinic. I’m starting a sickle cell clinic. I want it to be comprehensive, and there’s no way we can adequately care for these patients without palliative care. And so it sort of all came into alignment at one time. And, you know, I’ve learned a tremendous amount over the last years doing that with, with Charles. And I think when you start to understand the patient’s experience, it’s pretty obvious to most of us that there’s incredible need.

Eric 09:20

Yeah, well, we’ll talk about the need. But maybe for our listener’s sake, which is probably just my sake, can we take another step back and just remind me what, when we say sickle cell disease, like, what are we referring to? Kind of really big, big picture.

Eberechi 09:37

Yeah. So sickle cell disease 1 is a genetic disorder. So people are born with this disease. So what causes sickle cell disease is an abnormality in your hemoglobin gene which causes the normal spear shaped red blood cells to become sickled in shape. So like, almost like a crescent moon shape. And if you remember from your pathophysiology that the shape of the hemoglobin is this shape allows it to kind of go through the blood vessels, like seamlessly. But when you have these sickle cells, that happens in sickle cell disease.

These sickle cells get kind of blocked as they go through the blood vessel. And whenever they get blocked in the blood vessel, they cause ischemia. So wherever they are in the blood, whether you know, which whatever system they’re in, they cause ischemic and organ damage. So just repeated blockade of blood flow or impedance of blood flow leads to just chronic assaults and insults to organs in the body. And this manifests as the most common is this acute pain, right, this vaso occlusive pain crisis where they’re in incredible pain that often requires for them to come into the hospital to get further pain management.

But it also causes essentially just like havoc everywhere in the body. So from head to toe, like I said, any organ is fair game for sickle cell disease. So they have retinopathy from ischemia to the retina. They have foot ulcers from stasis as well. That’s pretty hard to heal. They get avascular necrosis of their joints. They have heart failures, kidney disease, that leads to esrd. So essentially every organ is affected by sickle cell disease. So it has a very high morbidity and also a very high mortality. So if you kind of think about it maybe 40 years ago, the life expectancy was less than 20 years. So these kids, then, they’re diagnosed at birth, well, close to birth, after birth, and they don’t live past 20 years old because we didn’t know how to really care for these patients. But over the years, with advances in screening and the things that we do and the medications that we have, people with sickle cell now are living beyond the 30th, the third, the fourth, the fifth, even some the sixth decade. So they’re getting to that geriatric age.

Stephanie 11:52

Right.

Eberechi 11:53

Like you mentioned, Eric. Um, but it’s, it’s not without any complications if they’re living longer. But now with this compounded chronic complications on top of it, plus the other acute issue that they’re dealing with from the sickle cell, the acute vaso occlusive crises and everything else that goes with it. So it is a very insidious disease that’s fraught with suffering. And again, the care that they’ve, they often receive, that they go from pediatrics to adult can be a bit bumpy. And we can talk a little more about that transition as well. But that is, sickle cell affects head to toe. So life limiting and also early morbidity.

Craig 12:30

And I think it’s important to state that it affects certain people and not others. It’s largely found in the black and brown communities of people of African descent and Latinos. So it’s generally speaking a disease that coexists with also minority populations and also the structures that exist for minority populations in this country, which is often lack of access to good healthcare, other social determinants of health. So these compounded factors that I think we have to take into consideration that do make it a particularly difficult disease whereby it could be treated. But sometimes there are these other factors that make it a lot more difficult to treat than if it were affecting just a white population, for example.

Eric 13:18

So I, I got a question then. So, Richie, you’re. You seem to be. Everything you said seemed to be aligned with like our definitions of palliative care, you know, potentially life limiting, high morbidity, high need populations dealing with diseases that until recently haven’t really been curable. We can talk about some of that a little later on. Why do you think palliative care hasn’t been very involved in sickle cell disease? Or has it been? Am I just not seeing it?

Stephanie 13:53

No, I mean, I don’t think you’re wrong because when I told my story about how I got involved caring for these patients and I was like I said, a brand new attending. And so the first thing you do is you look to your left and your right and you’re like, who can help me learn how to do this? Well, and it was hard. You know, Ebrechi and I were. Found each other sort of trying to figure out who else was doing this work. And she introduced me to Craig. But if you look around the country, it’s not like there are a lot of palliative care physicians caring for sickle cell patients.

And I think that, you know, getting to that question, it’s for a number of reasons. I think because of the geography differences, patients live in different places. And so some people train in places where there’s not a lot of sickle cell disease, for example, also in palliative care fellowship, we all know that a lot of our training is still very focused on managing cancer related pain. And so the, the idea of thinking about how to manage sickle cell pain is totally different. And I think we come out of our training feeling a little bit uneasy about that. We can get into this a little bit more too. But the pain landscape for these patients is complicated because they have these acute episodes, but over time they develop a chronic pain component as well as, and we all know that when we start talking about chronic pain and palliative care, we can get a little bit iffy about how to go about that and who should be managing pain.

And so I think I often say, and I’ve said this to fellows who have rotated with me in the sickle cell clinic, the pain in sickle cell patients is some of the most complex pain I’ve ever seen. And unraveling that and figuring out how to best address it requires a very complex approach that’s not just palliative care, but lots of other subspecialties sort of thinking together.

Eric 15:35

And so, and Stephanie, I’m guessing when you’re saying complex, you’re not talking about the receptor pathophysiology, but this idea of like, total pain. Right, Total pain, like a social, like structural, like all of these things.

Stephanie 15:50

Absolutely. And so again, you’re going back to your initial question was why not palliative care? Who can address that better than us? I mean, this idea of total pain, most other clinicians don’t get that sort of training and that perspective. So I think that’s one reason. We could probably name 10 others, and Ruchi, Craig may weigh in, but I think the pain piece is often daunting for folks.

Craig 16:14

Yeah, I think that’s right. And I think also if you think about historically, how palliative care programs have developed and what was their mission initially. So in the early days, it was really to work on helping patients who were nearing the end of life. And then the field worked really hard to push palliative care earlier into the disease process. So we’re not just an end of life care program. And then when you think about the sickle cell population or patients who are persons with sickle cell disease, you know, they’re, they’re not at the end of life until they are.

Right. But that predominantly they’re in the middle of a crisis, in the middle of a healthcare emergency. And so it wasn’t so obvious that at least in those early days, that palliative care should be the right service to offer pain management and support. So you had this kind of, you know, the landscape was kind of was very heterogeneous in terms of who would get called in to help manage the pain. Was it an anesthesia pain service? Was it the hematologist who just had this particular interest and maybe develop some additional skill sets around pain management? And so they started to just handle it themselves.

And like I said, I think I may have been in a relatively unique position in that I had a lot of training around It. But I don’t think that was at that time. I don’t think that was. That was normative at all. But now we are starting to see more programs at least start to feel that they have a little bit more bandwidth to take this on. But it does require a more mature program from a palliative care perspective to be able to do that. An early program, you have to pick and choose what you’re going to focus on.

You only have so many staff members. You’re, you know, what is your mission? I mean, are you there to help out with the cancer center and make sure that those patients get palliative care? They help out in the ICUs, make sure patients who are dying get adequate palliative care or whatever, you know, but that’s so. So some of it turns out to be cultural and institutional and where you’re practicing as well as. As a skill set that. That. That Stephanie was. Was pointing to.

Alex 18:15

Okay, I think you just answered one of my questions, which is, I’m sure that there are listeners now who are saying, wow, now I have to do sickle cell, too. How are we gonna do that? You know, like, I don’t have the training. I. Like when I did fellowship, we declined the sickle cell consults. We said, we don’t have the resources, the staff, we can’t follow them in an outpatient setting. So I think you’ve answered some of those concerns. Anything else any of you would say to those listeners who have that question?

Eberechi 18:46

Yeah, I think also, I think when we think of sickle cell disease, we kind of get kind of like, caught up on just the pain aspect of it. But like we said, is the disease with, like, high morbidity and, like, high mortality, and they have all these chronic complications that we, as palliative care physicians manage. People with heart failure. Right. And CKD are going on esrd. Right. So there are other domains of palliative care that need to be addressed aside from just pain management and sickle cell disease. There’s other symptom management as well, right.

There they have all these other symptoms, whether it’s. Whether it’s pruritus, constipation, nausea. There’s a whole slew of other symptoms that we could also manage for people with sickle cell disease. But I think the part that we miss is just the. The. The. The chronicity of the illness that they go through, not that they live in longer. Right. There’s need for these conversations about goals of care and treatment options. Right. With the advent of gene therapy, like, people Are thinking, should I go through a gene therapy? There’s the bone marrow transplant. Right.

And patients go through bone marrow transplantation, and that’s a very hard. Right. Treatment to go through. And they need a lot of support when they go through that treatment path. So there’s a lot beyond just pain for sickle cell disease. And that’s my message. There’s more than just pain and sickle cell disease. There’s a whole slew of other palliative care domains that we unfortunately kind of sometimes forget that is there that we can also address besides the pain.

Eric 20:13

Yeah. And this concept of, I mean, going back to the roots of palliative care, like Dame Cicely Saunders, total pain. Like it’s not.

Stephanie 20:21

And I guess I would also add in, it’s like any other patient population. You know, if you look at your institution, you’re not going to be able to see every patient that has palliative care need in your institution, no matter the disease group. And I’d also say, you know, and we’re doing a lot of work, the three of us, along with colleagues around the country, to understand this, but what is the role of palliative care in sickle cell disease? It’s certainly not to see every patient with sickle cell disease.

You know, every patient with sickle cell disease is not going to have palliative care needs. And so I think it’s bringing all of that together as well and looking at the patient in front of you and figuring out how you can best serve them. As Abrechi said, you know, a lot of our core palliative care skills translate to much of the morbidity that this pop. You haven’t seen a lot of sickle cell disease throughout your career.

Eberechi 21:09

Yeah.

Eric 21:10

And big decisions come up. Like.

Stephanie 21:12

That’s right.

Eric 21:12

Develop nephropathy. Thinking about the dialysis decisions, man, that transplant decision is hard. Right. Because who knows what the next three years has in store as far as genetic editing crispr, do you want to put yourself upfront to those risks, which is not trivial. Right. I think like 2 or 5% chance of mortality with transplant for sickle cell, like, it’s there. Yeah. Versus continuing treatments that have actually worked really well, like hydroxyurea for a lot of people. You know, big game changer. Forty years ago, if memory serves me correct, how we think about managing sickle cell. I wonder, as we think about this. And Reg, I just want to highlight that you just published a New England journal piece on this. Right.

Eberechi 22:00

Yes. Very excited.

Eric 22:02

Which I loved, because you also Listed challenges and action items for supporting integration of palliative care into sickle cell disease. Which is like, it’s a great table that lists all of this, including this idea of like, a lot of people still equate palliative care with hospice care. I wonder how much of this too, is that. A lot of sickle cell disease may be managed by hematologists and you know, for other diseases like liquid cancers, like, we don’t get as many palliative care referrals for those. Is, is that, is that a big part of this? Is that people.

Eberechi 22:35

I think it is. I think so. Hematologists are, I think they’re very protective of their, of their sickle cell patients. I think for many years and decades they’ve been like the go to, you know, the go to for their sickle cell patients. Like they wear all the hats, like pulmonologists, cardiologists, they do it all right? And I think they’re starting to realize that, you know, as these patients are getting older and more complex, they can’t serve as the one, the one stop shop for these patients. So they have to learn how to collaborate with other disciplines to really provide that comprehensive care, which is why they have now comprehensive sickle cell centers like the one at MGH and the ones at different parts of the country.

So I think, yes, historically they have been quite protective of their patients, but they’re starting to realize that, yes, we do need help from other people. I did a recent study where I actually, I surveyed hematologists and sickle cell specialists and actually the majority of them said that they think that palliative care would benefit their patients from sickle cell disease. And if you look at what they sort of manage, they manage like they manage all the symptoms themselves, right? It’s like high symptom burden disease and they manage all them. So yeah, but a palliative care physician might be a good person to kind of help to co manage these symptoms along with me. So it was very interesting that they’re realizing it and they’re willing to collaborate with palliative care teams if we’re also willing to kind of lend a helping hand as well.

Alex 23:55

Emraji, can I ask Annette or anybody else, do you know to what extent like. So this is kind of what happened in cancer first, like palliative care, okay. You can be involved with the cancer, the pain stuff and the other symptoms, but let’s not. You can’t get involved in goals of care until like they built up relationship and we built up a trusting Relationship and then. Okay, yes. Oh, actually, you’re really good at communication, actually. Yeah. I’d love it if you got involved with goals of care and that sort of stuff, like, where are we now? And a lot of these decisions about, like, you know, gene therapy and bone marrow transplant, is that something that they feel ownership of? They don’t want us to get it? Like, that’s off limits. Just deal with the symptoms or. And it’s probably. I’m sure it’s regional, too. Stephanie, I see you nodding your head.

Stephanie 24:41

Yeah, I mean, I think it’s a little bit of a mixed bag, I can say in my local environment, they’re begging for our involvement because of the complexity of these decisions. I think, you know, one thing to know about gene therapy specifically, I think it’s been, you know, sort of marketed as a pretty simple process to folks who don’t understand a lot about it. And when you dive in deep and understand what you have to go through for the gene therapy, there’s pretty significant morbidity and mortality. And as you guys were sort of hinting at and naming before, you know, I have conversations with my adult patients in my clinic frequently where they’ve already collected a lot of morbidity from the disease.

And once you undergo gene therapy, it doesn’t reverse that. So I can’t tell you the number of patients I’ve seen who saw gene therapy as a cure for their sickle cell disease. But their sickle cell disease has already wreaked havoc on their body in so many ways, and they get to the other side of this really risky, hard process, and it doesn’t feel the way that they expected. So I think the hematologists who are starting to do that and have those conversations with patients realize how complicated those decisions and discussions are, and they really want our help. The other thing, I’ll just, You know, I think our audience will be interested in this, because I was astounded to know this when I started this. There actually aren’t a ton of adult hematologists out there that care for sickle cell disease patients.

So when you’re on the pediatric side, I think it often looks a little bit different. But there is an incredible workforce shortage of hematologists in our sickle cell disease center. We have one, and patients drive from five, six hours away because it’s the closest person they can access. And so also what you get with that is they’ve had people caring for their sickle cell disease who actually don’t understand it. You know, they go to their local Er, they go to their family practitioner and those folks are trying their best, but I think this is such a complicated illness. And so you bring all of that together and you think about decision making and sometimes there’s mistrust because not everybody’s understood what they’re going through. The decisions are getting even more complicated. And I think all of that is right for palliative care involvement. And my experience has been that the hematologists are really begging for it.

Eric 26:51

And can I ask, what does your clinic look like? What’s your role in the clinic, Stephanie?

Stephanie 26:56

Yeah, so this is a really good question and we’ve had the same discussion with lots of our colleagues, because I am an N of 1 and Eva Ruchi is an N of 1 and Craig is an N of 1. You know, all sort of doing this. Our practice looks a bit different.

Eric 27:11

Maybe each of you could get like lightning round. What is, what is the palliative care role in your clinic?

Stephanie 27:16

Exactly. So I’m in a comprehensive adult clinic, so every sickle cell patient that comes in sees myself, social work, a PCP and a hematologist on the same day. I don’t see all the patients that come in, but we try to identify the high risk needs. And that’s an outpatient space at Baretti. Her practice looks a little different.

Eberechi 27:33

Yeah, so I’ve always been an inpatient palliative care provider. I’m trying to branch out to the outpatient world. Because of sickle cells. Actually, I think a lot of the work has to be done on the outpatient side. So in the inpatient setting, I think a lot of what I do is those patients who come in with acute vaso occlusive pain crisis, they’re in pain. I can’t have, like, meaningful conversation about, like, goals of care with them. That’s not fair. Um, so what I do in those, in those instances that I build trust and I think I serve more of an. As an ally for these patients who often are voiceless or feel voiceless, like they have no say in their medical care. Um, they’re like, you know, I’m in pain and the, the hospitalist is decreasing my PCA dosage.

Why is that? So a lot of education to say, you know, they have your best interest at heart. How can we involve you in this conversation? And the goal is to get you through this pain crisis and go home safely. So a lot of advocacy for patient and allyship and also building trust, because every once in a while you have those patients who actually come into the hospital and A regular vaso occlusive pain crisis turns into an ICU stay, and then you’re having goals of care conversation, and then you’ve already had that trust built with the patient and family. You can actually be able to be productive in having those conversations with them when that does happen. So again, I think a lot of the work has to be done in the outpatient setting, but in the inpatient setting, there’s still a role for it to be. For us to be involved.

Craig 28:50

Yeah. I spoke a little bit earlier about my experience as a fellow and at Columbia is a little different. We have both outpatient and inpatient models for integrating palliative care. The outpatient model was really mostly working with two specific hematologists who were managing all of our sickle cell patients. And there was more of a referral model. Sometimes some co management when they were getting to points where they felt uncomfortable with their. With the pain management specifically, occasionally with goals of care.

And then on the inpatient side, we actually had a proposal which was approved by the hospital to hire a dedicated nurse practitioner who worked as a kind of liaison between the sickle cell program, palliative care, and the hospital, hospital medicine. And so we would ground together with that person when our patients, when the sickle cell patients were admitted and kind of co manage their pain management in the setting of a vaso occlusive crisis, which was usually the phenomenon that we would mostly be addressing, occasionally patients going to the ICU and so on here at Memorial. Less so on the adult side, it’s much more the pediatric side that’s seeing a lot of the bone marrow transplant and management there. We’re not seeing so much of that on the adult side.

Eric 30:03

Okay, let’s. Let’s say you’ve convinced our audience that palliative care should be involved in sickle cell disease. And we will have a link to an hpm. What was it? Flight?

Craig 30:14

Flight. You know, flight.

Alex 30:16

This is like when you go out.

Craig 30:17

For drinks, you know, you have like…

Eric 30:19

A flight of like you get a little wines or whiskey.

Craig 30:23

It’s like a little. A little flight of whiskeys or beer.

Eric 30:26

This is like a flight of educational sessions. Yeah, yeah.

Craig 30:30

So it’s rather than being a big long, hour long lecture, they’re like 15 minute chunks of content to give an overview of palliative care in sickle cell disease.

Eric 30:41

So we will have a link to that. But can I ask some very kind of specific. Maybe we can do a little bit more lightning round kind of, kind of key tips and tricks that you’ve learned over the time about how to manage both physical and non physical symptoms of sickle cell disease. So let’s start off with the big one that’s probably on everybody’s mind, managing pain. I’m going to first turn to the acute side. Aberchi, you focus on the inpatient side, right?

Eberechi 31:08

Correct.

Eric 31:08

How do you think about pain management for like acute vaso occlusive crisis, acute pain crisis and sickle cell disease?

Eberechi 31:15

I would say so. I am new to my current institution, but where I was prior, at Baylor College of Medicine, they institute a mandatory PCA for everyone coming in with an acute vaso occlusive pain crisis. And this was sort of like a thing that they did just to sort of try to standardize how we manage pain for this population. And I think that is ideal. Right. Not every patient is going to fit that particular, you know, plan. Right. But I think when someone comes in for acute vaso pain crisis and you already know sort of like what their baseline chronic pain regimen is and you can actually know what their, what they use when they come into the hospital for acute pain crisis, just put them on a pca.

Stephanie 31:53

Right.

Eberechi 31:53

And let them be in charge of controlling their pain versus having to wait for the nurse to come in who’s caring for like 10 other patients. Right. And then they’re pain and the pain regimens. I think the use of PCAs, I think is important and has helped significantly in managing vaso occlusive pain crises. And I think that patients sometimes continue.

Eric 32:13

Their chronic pain meds and then just add the PCA as the patient prns the long acting.

Eberechi 32:18

You continue at the, at the PCA to as a short rescue.

Alex 32:22

And any preferred opioids in the pca?

Eberechi 32:26

I think it depends on what the patient puts in their regimen. I think a lot of people like to use Dilaudid. I don’t know why that’s what we often used, but I don’t have any particular reason why you should. You could use if you use Morpheuses.

Stephanie 32:39

Yeah, I don’t think so. I mean, one thing I’ll point out that you’re hinting at average is that pain plans help. So for a lot of these patients, having a conversation ahead of a pain crisis and then documenting it well in the chart so that the ED provider or whoever’s meeting has an idea about what works in those cases is the right way to go and there’s evidence that that actually leads to better pain control. So in our system we have that. For example, it’s copied into the epic chart in a. No.

Alex 33:04

I remember as a resident we would give patients with sickle cell and acute pain crisis Toradol. Is that a thing still? Yeah, I’m seeing some head nods.

Craig 33:17

Can do that for a few days. Yeah.

Eric 33:19

So ketorolac, NSAID. NSAIDs are generally bad for people with like, nephropathy and people with sickle cell disease are at increased risk for that. So having prepping for this podcast, I kind of saw a little bit of divergent opinions on like ketorolac. Some people said, like, hey, rah, rah, rah, let’s use NSAIDs. They’re great for short term use. Three days, you’ll be fine. Other people say, like the nephrologists say, wait, you’re doing what?

Stephanie 33:53

I think you’re right in the controversy. I mean, my experience is that if they have some renal dysfunction, probably avoid it. Same as you would for other patients. Right. If it seems riskier from the get go, maybe that’s our threshold. But I think even in adult patients, as long as they don’t have an AKI or some renal dysfunction, I think I’ve seen it be really effective.

Craig 34:13

Yeah. I think that aside from the question of whether or not to use NSAIDs, obviously opioids are the mainstay of therapy for these acute vaso occlusive crises and for long term management. The real challenge here, and I think it’s worth really highlighting, and what makes treating this population perhaps different than someone who’s dealing with acute cancer related pain, is you’re dealing with a level of opioid tolerance that is often very scary for a lot of adult providers. Right. If these kids, if you develop sickle cell disease early in childhood and you’re having a really tough time with vaso occlusive crises, I. E. You have a more serious, you know, variation of the disease, you’re likely gonna develop incredible tolerance over time.

And we know that the, you know, the neuroplasticity that you’re capable of developing with respect to opioids is much higher in pediatrics in childhood than it is with adults. So these kids, by the time they get to adult clinicians, can sometimes be on really whopping doses of opioids that make the average, you know, hospitalist and others, even palliative care clinicians, a little bit like what they’re on, like a thousand milligrams a day of oral morphine equivalents or 60 milligrams, you know, three or four times a day of methadone and this is where they, they landed because they’ve had many years of, of being treated. And so then you run this, then you have a problem, right? You have a, you have a pharmacologic and a physiologic problem with respect to how high can you go before you induce neurotoxicity.

And what do you do in those situations when you have somebody, you know, pretty high doses of methadone, they come in with a crisis. What are you going to do? You’re going to give them, you know, 10 milligram IV hydromorphone boluses. I mean, you know, we have to sort of think about, you know, what makes the most sense. So one strategy, and then we had some patients that, you know, would get to extremely high doses and there was just nothing that was working. And we ended up rotating them to buprenorphine. Now, another problem. You have someone coming into the hospital on 32 milligrams of buprenorphine. It’s a sickle cell crisis. What are you going to do?

Eric 36:11

Yeah, all their muopane receptors are taken.

Craig 36:14

The binding affinity is so strong you’re not going to be able to break through and get there. So we’d have to decrease their buprenorphine or stop it altogether, start a pca. So it became this very complicated dance of trying to mitigate pretty profound opioid induced hyperalgesia, profound opioid tolerance, profound. And just the optics of this in the setting of an opioid crisis in the country.

Eric 36:40

So I want to push it makes it really challenging. I realize there’s tons of challenges. Now we’re kind of convincing our audience not to get into this space. Let’s drill down to teach me things I could do. Like when I think about PCA. Okay, high tolerance. Most people are used to doing 0.2, 0.4 as your, your bolus demand dose on a PCA. The idea of giving somebody 10 milligrams as your demand dose would scare the bejesus out of everybody. How do you think about the demand dose for a pca? For somebody who’s been on, like, I’m guessing Most people, you’re starting 0204 if they’re not on a lot, but if they’re on a lot of opioids, do you think about PCAs differently?

Craig 37:21

Yes. Yeah.

Eric 37:22

Yeah, definitely. What would your demand dose be? Do you have any tips or tricks around that?

Craig 37:27

I’ll, I’ll give, I’ll give you a sort of a, a sort of a, a, you know, for the, for the audience sort of how I would think about this. I’ll take. Let’s take a hard example and we’ll start there. And if we can kind of work through a hard example, then I think it’ll make. It’ll. Then the easier examples would be more straightforward.

Eric 37:42

Gotta make it quick, though. Cause we’re running outta time. I got more questions.

Craig 37:44

So you have someone that comes in, you know, he’s on 60 milligrams of methadone three times a day. That’s a pretty good dose for the average 180mg a day of methadone. You know, you’re looking at, if you were to do a quick and dirty oral morphine equivalent, something like 600 milligrams of oral morphine equivalent.

Eric 38:01

Yeah.

Craig 38:03

What were more. Right. So you might say this is someone who I might. If they’re not nauseous, not vomiting, they’re able to keep their methadone. Keep that going. That’ll be. That’ll kind of serve as their basal rate, convert what they’re on into an oral morphine equivalent, convert that to your IV of choice, whether it be hydromorphone morphine, you might even. And so you’re familiar with using lpca, that might be another option. But in any case, you’re going to take that.

That total dose. That’ll be essentially what you would give them as if it was your basal rate. So that. That’s 600 milligrams, which would be what I had about 20, 20 milligrams of IV or so an hour or so. I’m not thinking totally straight with my math here, but something. It would be. It would be a good dose. And then you. Then you give a demand dose based on 50 to 100% of that. So it could be. It could be pretty significant.

Eric 38:51

Yeah.

Alex 38:51

Yeah.

Eric 38:51

I think this is the thing always confusing me of PCI. I see some people say 50% of continuous rate, some people say 1/6 of your. Your hourly dose. Like it’s. PCA has always confused me because I think it’s really born out of surgical literature. So trying to apply a PCA into chronic pain on top of acute pain becomes really tricky for me. Stephanie, you’re going to say.

Stephanie 39:13

I was going to say this could be a whole podcast.

Craig 39:15

The whole podcast.

Eric 39:16

Okay.

Stephanie 39:17

We all talk about this every day.

Eric 39:19

Yeah, we’ll skip it. Because the fix is ketamine, right?

Stephanie 39:22

That’s right.

Eric 39:23

Do you use ketamine?

Stephanie 39:25

We do some. So I will say the evidence base is in pediatrics, but the limited Evidence base in all of this is mostly in pediatrics. There’s not a lot of adult data. It works for the right patient. I think about it the same way I would for ketamine in any other patient. You have to think about the patients that won’t do well on ketamine and the risk factors. But it’s worked incredibly well in my practice.

Eric 39:47

Anybody else use it?

Craig 39:49

Yes. Yeah. In those situations when you’re just dealing with such profound opioids, eyebrowgesia or tolerance, that’s, you know, that, that’ll be another strategy.

Alex 39:58

And what do you give Paul Parkish?

Craig 40:01

Anywhere? Five to ten milligrams an hour, something like that.

Alex 40:05

For a period of days.

Craig 40:06

Yeah. As a continuous infusion. And then see if their opioid use goes down. If they’re in a, if they’re in the heat of a vaso occlusive crisis, you’re looking at doing it for, you know, a short period of time or hopefully just a few days. And then start tapering off both the PCA and the ketamine.

Alex 40:21

Oh, okay.

Eberechi 40:23

Use it for chronic pain management.

Eric 40:25

Yeah. I was just gonna ask about chronic pain. How do you think about chronic pain?

Craig 40:28

Yeah, that’s a tough one also. Yeah, buprenorphine especially.

Stephanie 40:33

I was gonna say buprenorphine. Yeah, I think that’s been the most effective across the board.

Craig 40:38

Yeah.

Eric 40:39

Really? Why, like, don’t you run into these issues around these pain crises, high dose buprenorphine, like.

Craig 40:46

Well, I think, I think you have to. There’s also the pre stem cell transplant population and the post stem cell transplant. So I think it’s important to make that. When you’re talking about someone who’s gone through transplant, they’re no longer gonna have any vaso occlusive crisis, but they might still have ongoing pain. Whether it be from avn, chronic bone pain, just the sort of the natural history of multiple episodes of vaso occlusive crisis.

You can still have ongoing pain. And so in those cases, I think that’s long term use for chronic pain in that situation. I think buprenorphine is very safe, it’s helpful. It doesn’t have the same kind of complications and side effects as we all know, for long term use. So that could be very. As well. Remember, there’s also some comorbid, you know, opioid misuse too, that I think we have to name as well, that we do see. It’s not that we don’t see that as well. And so you have, you know, teenagers, people in their 20s and so on are at higher risk of opioid misuse.

And you start to see some element of this as well. And so, you know, things like methadone and buprenorphine would be something I would go to sooner, you know, if I’m, if I’m worried that there is some risk factors for opioid misuse and especially if I’m going to be doing something for the long term and not just a short, discreet, you know, prescription like for after surgery or whatever, but like this is going to be long term therapy. So that’s a, that’s another reason.

Eric 42:04

Richie, any other tips from your standpoint or other symptoms as far as tips that our audience could hear about?

Eberechi 42:13

Oh, goodness, yeah. Or an acute pain crisis?

Eric 42:17

No, let’s say more. You’ve gotten through the acute pain crisis. Now you’re either transitioning to start thinking about outpatient care or you’re in there for Craig and seven, you’re an outpatient clinic. Other kind of key tips that you’d like to share with the audience?

Eberechi 42:34

I think the one thing that we haven’t talked about is the mistrust in this patient population towards the health system. Often these people have gone through childhood and they get through childhood and go to adult world and they’ve had a lot of encounters with the hair care system and often there are good encounters and there’s a lot of bad encounters. So often they have had bad experiences where there has been some biases against them, there have been some mistreatment against them. So I will say some patients with sickle cell disease do mistrust, you know, anyone who’s wearing a white coat.

I think you have to build trust with this patient population. Especially if we are, you know, delving into beyond symptom management. We’re going to delve into like goals of care and like treatment decision discussions. Right. So building trust is imperative with this patient population and building trust with their family members as well is imperative. So often, like I said, in the inpatient setting, for me, I spend a good chunk of time just building that rapport and that trust with the patient. And I always say, who is your support person? Who can I talk to to see sort of how they are also experiencing what you’re going through.

So I think that’s something that we can’t avoid. You know, that anti racist framework that we need to approach these patients with is very critical because they have seen a lot of just mistreatment from the healthcare system and it takes a lot to kind of rebuild that trust again. So, and again, you walk in as a palliative care doctor, they think hospice. Right. End of life. Do they think I’m dying? Like, why are they sending, you know, palliative care doctors? Come and see me. So that’s something that I face in the inpatient setting as well. So I think the trust part is. Is we can’t avoid that part as well.

Eric 44:14

Yeah, I can imagine that trauma informed care becomes a really important part because correct all of these traumatic, even just the pain crisis, not getting adequately treated in the past traumatic.

Craig 44:26

Yeah, yeah.

Alex 44:28

Could we end with a different sort of final question?

Eberechi 44:31

Eric would have.

Eric 44:32

Go ahead, Alex. All right.

Alex 44:34

We started about talking about how, like, we’re all doctors. Could we. Could we each take a turn and give a shout out to another member of the interdisciplinary team who’s really important in caring for people with sickle cell disease. Anybody can go first.

Craig 44:49

I’ll say our spiritual care. You know, I found that working with our. Our chaplains, our spiritual care providers have had really meaningful impact on the quality of care, the connections that they felt with our palliative care service. And that’s often been a real link for. For them to feel trust and to feel valued from their own spiritual traditions, if they have one and even if they don’t, to just to be able to have that. That space to be able to talk about some existential issues that they’re facing.

Alex 45:18

That’s great.

Stephanie 45:19

I’ll name social work. I think you can all imagine all the things that social work can help with, but especially even just accessing the healthcare system and a lot of these systemic issues that everji was talking about, it’s vital to these patients sort of getting the care they need.

Eberechi 45:34

And I will say, while I was at Baylor, the nurse practitioner that I work with was an incredible asset to this patient population. She was a bedside nurse and had worked with a lot of patients with sickle cell disease, so had that compassion and empathy and really understood what they go through when they’re in the hospital. So I was able to bring that with the palliative care hat to take care of them. So shout out to the nurse practitioners. Norma Puente.

Eric 45:58

I’ll put in a plug for a palliative care pharmacist because, man, there is a lot of drugs that people like. Even hydroxyurea, like a mainstay. Most providers never prescribe it, like, thinking about this, how it interacts with other medications. So shout out to our palliative care.

Alex 46:16

Pharmacists out there, yes, we’re all friends.

Eric 46:19

We yeah. Thank you for being a friend.

Alex 46:28

(singing) Thank you for being a friend. Thank you for being a friend. Thank you for being a friend. Thank you for being a friend.

Eric 46:44

Eberechi, Stephanie Craig. Thank you for being a friend to the Cherry Powell Podcast.

Craig 46:48

Thank you.

Stephanie 46:49

Thank you for having us.

Eric 46:51

And thank you to all of our listeners for being a friend and for your continued support.

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Disclosures:
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