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Hello. Welcome to our expert roundtable today. It's entitled Transforming Sickle Cell Disease Care. This is a CME accredited program, and before we begin, it's important to share our disclosures with you. And we're gonna bring those up here on the screen now, just for your information. But for too long, sickle cell disease has been viewed primarily through a lens of acute, painful crises, and while managing these events remains the cornerstone of care, this perspective really overlooks the more insidious insidious reality, and that that is that today we understand that sickle cell disease is a chronic, progressive, and systemic condition, and it's defined by cumulative organ damage. Despite the advances that have allowed over 95% of children to survive into adulthood. The median life expectancy still really lags behind more than 20 years compared to the general population. And so it's a stark reminder that the disease has a relentless toll. So today we're here to address this critical practice gaps that still contribute to this reality, and it can be difficult to recognize and manage long-term organ dysfunction. And, and as well, it's difficult to integrate therapies that could change the disease trajectory. So our discussion today with our wonderful panel here. It is structured around 3 core pillars that directly address these gaps. Now, first, uh, it's imperative for proactive monitoring and management of progressive organ damage. Second, we'll discuss how to navigate the rapidly and thankfully evolving therapeutic landscape that we have for sickle cell disease, and that goes from established agents that we have to the curative intent therapies that are that are coming and have already started to come and be available. And finally, the third aspect that we'll talk about is the need to integrate the patient's voice into clinical decisions and through the systematic use of either patient reported outcomes or mindful discussions about how the patient is doing from day to day. So my name is Dr. Nurma Shah. I'll be chairing today's roundtable session. I'm the director of the sickle cell Transition Program at Duke University, taking our kids and hopefully getting them as successfully as we can to the adult world, and we're doing an amazing job. Uh, and doing that, uh, I think across many programs now around the United States. But joining today, I'm really thankful to have, uh, amazing hematologists, uh, and, uh, panel here. Uh, we have Dr. Enrico Novoli, who is the professor of medicine and division chief of Classical hematology at the University of Pittsburgh. Uh, we have Dr. Bie Eomerian, a professor of medicine and the American Red Cross Endowed Chair in Transfusion medicine at the University of Connecticut School of Medicine, and finally, Dr. Donnell Ivey, who's the Chief Medical Officer for the Sickle Cell Disease Association of America, also the education manager at the Sickle Cell Awareness Group of Ontario, as well as the registered mentorship manager for GANSA, the Global Awareness Network for Sickle Cell and Inherited Diseases. So there, there are, uh, those pillars that we're going to go through, uh, again, I, I have, uh, a wonderful panel that I, I really encourage to chime in, but I do have a couple questions that I thought would be a nice way to start, uh, and kind of going through each of those, uh, pillars we were just talking about. So, I'm gonna start with really, uh, a question about, uh, progressive organ dysfunction, and, and the first topic is really focused on cerebrovascular health. And so for cerebrovascular health, for an adult patient. With sickle cell disease, we have them now aged out of TCD screening. So the question comes up, how do we practically assess the ongoing risk of stroke. You know, it's important, of course, in pediatrics to do this up until age 16, but now as an adult, we're, we're trying to understand, do they have, uh, the risk of stroke? Do they have a silent, uh, stroke? I guess that would, I think it would be an important question, and I thought the panel, and we could start with Dr. Annamarian, you know, how do you assess your adult patients? Is there a routine process you go through? Um, but what are your thoughts in, in, in those type of situations? Thank you, Doctor Shaw. You know, I think this is one of the trickiest parts of being an adult-focused, um, sickle cell disease specialist is that we know that chronic complications, um, including the development of stroke later in life, um, can be devastating and it, and it doesn't happen. Um, infrequently, um, data is out there that supports that 50% or higher, uh, numbers of adults, um, have silent cerebral infarcts, and the American Society of Hematology guidelines from several years ago actually suggests, not with a strong recommendation, but suggests that every adult have at least one screening MRI to look for the presence of a silent cerebral infarct, and I, I think that that's. I, I think it's great that we have the guideline. I don't know how many of us are implementing that, that soft suggestion from the American Society of Hematology, and I'll tell you why from my perspective. I think if we find a silent cerebral infarct in adults, I think we don't know what to do and we don't have the evidence to support that managing them in the long term, the way that we manage children with uh chronic transfusional therapy is really going to, to work in terms of secondary stroke prevention, but I'd like to hear what uh Enrico does there in Pittsburgh. Yeah, thank you, um, uh, Doctor Anda Maria. It, it's not an easy answer. We, uh, personally, I try to individualize the screening. To the patients, as you know, sickle cell disease is such a, an umbrella term for many different subtypes of sickle cell disease and uh different, there's so much of phenotypic variability. I inherit all the time, I inherit patients from the pediatric program with a history of stroke or a history of high TCD velocity. I inherit patients who are on chronic exchange transfusion. And so those patients obviously I manage very carefully. I oftentimes continue extreme transfusions throughout their adult life and I do frequent MRI monitoring also for the development of moya moya and uh because the risk of hemorrhagic stroke increases um in um young uh young adult life uh and so. So those patients definitely I screened even if I, I don't have them on exchange transfusion, I monitor them very closely. I may do MRI's more frequently, um, than in other populations. For those without a history of stroke who are, uh, who have SS, I, I do think it's, it's a great idea to, to obtain MRI's. Many of them report, um. Some, you know, history of headaches or a history of um of um variable neurological symptoms that they may have a mild cognitive dysfunction that you can detect at some point. And so I have a very low threshold to get uh an MRI for instance, before starting uh medications such as uh Imitrex, uh um for, for, uh, for headaches or other migraine preventive uh strategies. And then for those with SC disease, uh, um, what I mostly focus on is on management of cardiovascular risk factor and traditional risk factor for conventional risk factors for stroke in any population and, you know, uh, over there for that population, I, I may. Not, uh, necessarily do screening MRIs, but I do keep in mind, uh, that, uh, BP management, weight management, uh, cholesterol management are, are important as in other populations. So it's a, it's a complex answer. I, I think it's, it's, but, but I agree that, that we, we probably as a, as a group, we don't do enough MRI's, uh, in, uh, in adults with sickle cell disease. We don't screen enough. And, and, and if I just may add, and, and I would love to hear a brief. And Enrico's answer to this. So, um, I know Enrico brought up the neurocognitive, um, effect and the ability. And so I, I, I don't know what they think about potentially adding in their neurocognitive assessments to, to see if individuals with sickle cell and past histories of stroke are having some neurocognitive decline and, and, and there are strategies that exist for um things like ADHD for individuals with Alzheimer's, you know, hopefully, it doesn't get that. But, but can we employ some of those strategies to help individuals that are aging, that are getting older. And I know myself, you know, I have a history of silent infarcts, I can see those on MRI. I also have a history of um an aneurysm that has been repaired, um, but I'm also starting to notice that, you know, my executive functioning is starting to slip a little bit. And so, so, um, you know, what do we do also in adding neurocognitive assessments to, to the evaluation? Yeah, absolutely important, you know, one of my, uh my first sub one was actually on, uh, neuroradiological correlates of cognitive dysfunction, sickle cell disease, because I realized, you know, a lot of patients, um, um, have a very subtle cognitive dysfunction. You won't detect it unless you test for it, and it emerges only. When you give very complex instructions on how to take certain medications or how to to reach multiple subspecialists and you notice that there's a difficulty in in grasping the distance, so the, the details, so it's really an executive dysfunction and um as you as you pointed out and it's subtle and you won't see it unless, unless you do neurocognitive testing and what to do about it I think you know even if you don't have um. Strategies to prevent the deterioration, you can at least if you know you can target more interventions, case management, social work interventions to help uh those individuals um with their, with their disease management so.