The institutional challenges of sickle cell

Show Notes

Some 100,000 individuals in America alone are dealing with sickle cell. Daniel speaks with Joel Helle, Vice President of Physician Services for CVS Health, about how to change the often one-size-fits-all approach to this painful disease — to care that is not only more continuous, but personalized, and proactive.

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Joel Helle:
In many of these other therapies that are similar in size and scope, sickle cell is the largest blood disorder, and yet the least unfunded. And it happens to be Black and Brown, makes me concerned that we need to look closer ourselves as to why we're not doing more funding of it.

Speaker 2:
Welcome to Healthy Conversations. Today, I'm in Healthy Conversations with Joel Helle, the Vice President of Physician Services for CVS Health. Just give us a flavor and tell us a bit about your role at CVS Health.

Joel Helle:
A pharmacist by training, I've been with the organization for 26 years. I've really always been in the physician space and out in doctor's offices. Today, I own a bunch of things. One is our relationships with physicians in the specialty world, as well as our relationships with patient support groups in the specialty world. And then most recently over the last eight months or so, health equity at Caremark.

Speaker 2:
What do you mean by specialty pharma?

Joel Helle:
Specialty pharmacy means drugs that are, A, really, really expensive, so probably more than $8,000 a year, $7,000 a year. Often might need special handling like refrigeration. It might need additional education for patients, like injection training. And those drugs are considered specialty drugs that you typically can't get from your regular retail pharmacy.

Speaker 2:
Maybe touch on how has the role of the pharmacist, maybe since you finished your pharmacy degree, evolved?

Joel Helle:
Well, when I got out of pharmacy school, it was fill prescriptions and work in the pharmacy. And I worked at CVS way back when. And now, I think there's a huge change in pharmacy. We have so many pharmacists that become an expert within a particular condition. Those pharmacists do sickle cell all day. They do cancer all day. They do hemophilia all day. And so, it becomes this niche type of learning for pharmacists to really understand what's different about these different disease states that they might not have learned in school. I guess a lot more options for pharmacists in today's world.

Speaker 2:
That means the way pharmacists work, whether it's inpatient, outpatient, in a PBM, et cetera, is shifting. We're here to talk particularly about sickle cell and sickle cell patients and their management. Anything you've seen that's come up about the culture of health and medicine and how that might be impacting the sickle cell patients and the clinicians you deal with who are helping manage them?

Joel Helle:
Sickle cell is this heartbreaking disease that folks are born with, but it manifests itself very often in pain. And it's Black and Brown people that are affected by sickle cell. And as they get older, they often drop off therapy and they move to different areas where they might not have a sickle cell center, because I think there's only about 15 or 20 of them across the country. And when they fall off care and this pain comes, this excruciating pain, they have to go to the ER.
And when they go to the ER, they very often are dealing with physicians that might not know that much about sickle cell. And why that is concerning is, when we talk the Sickle cell Association, they tell us stories about patients who have sickle cell going in a major crisis. And they get walked out by security out of the hospital because they don't believe that they're in pain and think that they're a drug seeker. And additionally, the Sickle Cell Association told us that they tell people, "Dress up. Make sure you dress nice when you go to the ER so that you don't get treated terribly." And so, there's a great opportunity there to understand why that is in the culture of medicine and why we treat people that are Black or Brown maybe differently, and what that means, and how to treat those patients. And make sure that they get equitable care and get treated appropriately.

Speaker 2:
There's about 100,000 or so individuals with sickle cell in the United States alone. I'm trained as a pediatric hematologist oncologist, certainly dealt with many sickle cell patients. And again, as you mentioned, many may see one in the ER once a month or sometimes every evening. There's organ failure from sickling, needs for blood transfusions, et cetera. Are there some key lessons you wish most healthcare providers would know that isn't often top of mind?

Joel Helle:
I think everybody learned in school, sickle cell, the blood cells are like a sickle. Which means, when they go anywhere and they travel around your body, they not only can cause pain, but they can cause strokes. They can cause all kinds of different issues in hearts, lungs, kidneys, et cetera, across the body. But more importantly, I think it's never too late to learn for all of us. And we're actually in the process right now of creating our own educational libraries for our own nurses, and pharmacists, and educators who aren't typically around sickle cell patients. So if it does come to them, that they have a better understanding of how to think about sickle cell.

Speaker 2:
So is that one of the reasons you chose this particular disease to focus on? You've got the disparities, often racial. You've got the challenges of not having a biomarker for pain in many cases. Are there ways that you've seen clinicians, providers of all sorts, up-level and make change?

Joel Helle:
For years, there was no new drugs to treat the underlying cause of sickle cell. And I think in late 2019 is when we first saw new drugs to come treat it. And that's in the specialty world. And so all of a sudden, we have these new clinicians that never knew sickle cell before to get out there and learn what's different now than it was 40 years ago. And it includes monoclonal antibodies that can be infused in the hospital or infused at home, especially now with COVID.
Frankly, when I first got into this related to sickle cell and we started to talk to hospitals and institutions across the country, and they were so excited to talk to a pharmacy partner. They were like, "We've never had a pharmacy partner, and we don't have the data for some of the basic things around hydroxyurea and some of the drugs that are used to treat sickle cell to understand if patients are on it." Are they adherent to it? Do they take it every day? So all that data, very often, isn't even available to physicians who do know all about sickle cell. We have this huge opportunity, and we're on this pathway and journey to gene therapy, which could be a cure if it makes it to the market. And I think we've seen some good clinical trials.
But already in the community when we talk to the Sickle Cell Association, there's hesitancy about what might be a cure. And it's really related to a couple things. One is trust within the Black and Brown communities. And then second is, 60 Minutes had done a 20-minute thing on sickle cell and they talked about the HIV virus and how they altered it. And they put it back in the body, and lo and behold, there was a cure. We're going to already have to make sure people understand that we're not giving people HIV, and we're not putting HIV back in your body, and what gene therapy will mean. The learning curve in sickle cell is changing by the moment, and I'm super excited to be part of it because I really feel like we can make a big change and a big difference here within sickle cell.

Speaker 2:
You used the word beginning of the end in a sense with some of these emerging, very exciting, what look to be cures using genetic modification of hematopoietic stem cells and fixing the core, root problem so your hemoglobin is no longer sickled, causes sickling, is looking extremely promising. But as you brought up, there is a lot of, not just disparities in accepting next-generation therapies, but misinformation, the challenges like we have with the COVID pandemic and vaccine hesitancy. Even when you might have a cure or something hugely preventative in terms of vaccine, getting folks to understand it. And certainly with some of the baggage from whether it's Tuskegee, particularly when they're new, and might be tied let's say to HIV. It's a interesting challenge. Are you finding that the role of the pharmacist, the role of the PBM, the role of the CVS can help mitigate and educate these patients?

Joel Helle:
One is, we need to. And we have this huge opportunity with the breadth of assets we have at CVS to make a difference here. We've got nearly 10,000 stores out there across the country. And how do we get closer to the community to understand how they want to be impacted? We learned from COVID, like you mentioned, that Black people want to learn from Black people and Brown people want to learn from Brown people about their options. I talked to National Black Church Association last week, and another opportunity for us to think differently about how we educate both patients as well as those communities around the new treatments and around what's available. To learn how to influence it, instead of one-size-fits-all has very often [inaudible 00:08:56] programs that we have. Let's go to local communities and understand what the specialists in those areas are saying about sickle cell and how we might think about programs differently.

Speaker 2:
Traditionally, sickle cell research has been generally underfunded. Any perspectives on the funding situation and how we might catalyze everything across the continuum from prevention, to understanding, to therapy, to cure?

Joel Helle:
In many of these other therapies that are similar in size and scope, sickle cell is the largest blood disorder and yet the least unfunded. And it happens to be Black and Brown makes me concerned that we need to look closer our ourselves as to why we're not doing more funding of it. I feel like because of gene therapy and whatever we're on the cusp. We could really make some effort and make some change here, but we have to get education and information out to physicians, pharmacists, nurses, et cetera, everyone out there around all this new, exciting technology that I certainly never learned in school.
And things like trait testing is extremely important. And we've been working with the local Connecticut organization... They've seen significant increases in babies born with sickle cell because of non-trait testing. And I think we need to find a way as an organization, CVS Health, to help there. How can we do that? We have what we call workforce initiatives, where we're out in communities. There's one in Hartford where we're helping people to get jobs. At the same time we're there, we can provide people information about trait testing.
In fact, I was talking to our colleague resource group this morning, and there was a woman on there who said, "I have the trait. And in my family when we were kids, everybody talked about it, and everybody would get it when they got married." And for some reason that's dissipated within our community, and we need to go learn more why. But those are the things... If it's dissipated, how can we help as an enterprise to get more trait testing to happen out there? And we need to learn first from the community themselves why it's not happening, and then put in tools and ways in education and information to make it happen.

Speaker 2:
Anything that's top of mind in your own work when it comes to sickle cell disease right now?

Joel Helle:
The opioid crisis has created angst for this community because they get treated differently now to be on opioids. And how do we help pharmacists in stores? Not just CVSs, but all pharmacists in stores understand what it means to be on opioids for a sickle cell patient.

Speaker 2:
Have you seen any creative ways that can help manage a pain crisis that don't go right to the opiate?

Joel Helle:
We have pain scales that you can talk about in an app to help physicians and other clinicians who are helping you manage your pain understand what it means as you're going through the crisis, to get to a place where we can hopefully manage the pain to get off the opioid, and then after the crisis over, go back to normal. And it's very simple and easy to use. And then it tracks it over time, which then would go to the doctor. And then the doctor would have the information around pain over time, how it's happening, and get a good feel for how their patients are doing.
We have new platforms that connect to doctors through their EHR to make it easier and smoother to get started on medication, to continuing on medication. If you need an infusion at home, for example, of a monoclonal antibody, we can make that path and that process easier through, A, connecting to the doctor, and then B, connecting to the patient through either text, or app, et cetera, to bring healthcare into the 21st Century here and not be fax and back and forth phone calls when nobody picks up their phone. So those trends can really change what we're doing in sickle cell, I believe.

Speaker 2:
Yeah, 100%.

Joel Helle:
And what's funny, similar to hemophilia, sickle cell, not everybody's the same. There's a wide range of people who get severe, severe, debilitating pain. I was talking to a mother the other day who, her daughter never had any pain, so she didn't feel like she had to be on hydroxyurea at all until she had a stroke. And then realized, "Oh my God, there's other things that are happening in my body." So there's a whole host of other things that we could do to support those patients.

Speaker 2:
Are you seeing any ways that, that can integrate with the pharmacist, with a specialty clinic, with the hematologist that really can help shift sickle cell care from one-size-fits-all, and intermittent, and episodic, and reactive to one that's much more continuous, and personalized, and proactive? That can really start to bring care anytime, anywhere.

Joel Helle:
We're building now, within the sickle cell world I'll call it, additional tools within the app to help support these patients. So one is around hydroxyurea and adherence and reminders, that type of stuff, to take it every day, et cetera, reminders to get a stroke test once a year. Giving your doctor a good picture of what's happening related to opioid use and even going to pharmacies and ERs, et cetera. I'm going to say proving that you actually have sickle cell. Because sometimes that's the case, it's how do I tell this doctor I really do have sickle cell? And these apps and other places can show a doctor over time that you really are a sickle cell patient and I think can really change the treatment of those patients when they do go through crises, both in pharmacies, and in hospitals, and the ERs, et cetera, with people who might not be familiar as much with sickle cell.

Speaker 2:
One of the things often physicians or others in the ER resent is when the patient comes in, says, "I need exactly eight milligrams of this drug right now to manage my pain." But often they do know best, and particularly if the electronic medical records and systems can help cross-connect-

Joel Helle:
Yes.

Speaker 2:
And validate that, that can play a role.

Joel Helle:
For getting started on a specialty drug and if you did have to go there, I think we're connected into about 50% of... Prescribing doctors have access to about 80% of EHRs now. So over time, the ability to pull that information, understand what's happening, provide it to the payer, who's often trying to determine if they want to cover it... We can do a lot of that quicker, faster, easier when someone's in the middle of a crisis.
Three years ago, certainly five years ago, we were connected to zero doctors. And now, we're connected to more than 50% on this journey, to probably 80% on this bigger journey to everyone. Because the government, et cetera, is helping us now to force everyone to get connected. CVS is ahead of the curve here in connecting to doctors and having the information available to be able to provide to payers to understand what's going on with a sickle cell patient, and get them their medication quicker, sooner, faster.

Speaker 2:
Anything new coming? Any previews that you want to share? Even if it's early in the process.

Joel Helle:
I mentioned monoclonal antibodies being infused at home. What I found interesting is that even the doctors that we talk to who do sickle cell every day at Duke and St. Jude didn't realize that patients could be infused at home with the monoclonal antibody. And what he said to us is, "Wow, Joel, that's unbelievable." Because some of these patients live 50 miles away, 70 miles away from coming to the hospital to have the infusion, and they're like, "I'm not doing it." Even though it could completely pull them out of a crisis and make them better...
And so, that information, and education, and tools for people to understand that's available is really up to me and my team within the enterprise to make sure we are making physicians aware. And then our national and local partnerships with these patient support groups is, we really need to spend more time with them to understand, even locally, what's different about Atlanta, versus Boston, versus Chicago, et cetera, in those areas to influence and educate both physicians and patients perhaps differently. Like I mentioned before, one-size does not fit all.

Speaker 2:
Speaking of infusions, there's some new technologies evolving, essentially a little robotic pill, that just swallow... Goes into your gut in the right location, injects the biologic right into the gut wall. It's in clinical trials now, a company called Rani Technologies.

Joel Helle:
The only reason I know this is because it's my uncle. He's working with this pharma company right now. You swallow the pill, and actually you hold a magnet out on your stomach here. And it keeps it there, and it's a slow-release thing that stays within your gut because it's only need to be released in the gut for that particular drug. And then after I think nine hours, it's gone. You take the magnet off, and it comes out the other side. There's amazing, new technologies coming out every day, and that one's as simple as a magnet.

Speaker 2:
And you can imagine just an outpatient visit for long-term cures.

Joel Helle:
Hemophilia, HIV, sickle cell, there's so many different things that, probably, I think within my lifetime we will see cures too. And so, I hope that, that's true.

Speaker 2:
Thanks, Joel, so much for being with us on Healthy Conversations. Thanks for your great work on behalf of sickle cell patients and the community. And let's hopefully build a brighter future for sickle cell and other diseases across the continuum.

Joel Helle:
Yeah, and thank you for having me today. It's nice to be talking to someone who knows so much about it. So thank you so much for having me.