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Hi, my name is Doctor Wally Smith. I'm in Florence Neal Cooper Smith, Professor of Sickle Cell Disease at Virginia Commonwealth University. And today we're gonna talk about a very important topic to those living with the genetic hemoglobinopathy, sickle cell disease. The topic is strengthening the sickle cell disease triad. These are my disclosures. Then the learning objectives. We're going to talk about the why of why we need uh Pr Os today. And we'll also discuss uh some of those pr O SA little bit later, but this talk is gonna be focused on the triad itself and why we need uh to support these patients. I'll be covering the sickle cell disease triad. What is that improving the medical home empowering community based organizations and then in enhancing collaboration within uh the sickle cell disease triad. So, what is the sickle cell disease triad? This is a concept that was created from a grant called A Step Up Grant. And the Step Up grant was a collaboration between medical centers to try to improve care for those with sickle cell disease. And I'll explain it in me more detail. Uh in a few moments, sickle cell disease of course, is a genetic hemoglobinopathy in which a single mutation in the seventh codon of the beta globin gene codes for an abnormal RNA, which codes for an abnormal protein which causes an abnormal hemoglobin. And as you know, hemoglobin uh is the oxygen carrying molecule for all red cells, the hemoglobins turn into crystals and when they polymerize into these uh crystals in the de deoxygenated state cells no longer are able to flow. The crescent shaped cells are clogged up and the blood vessels um are unable to transmit them. They burst, they spill their toxic contents and then anemia results, pain results, ischemia results, organ damage results uh ultimately early death and uh many, many uh in organ damages result from this one's tiny mutation. Well, the problem that I wanna talk about today is the problem of culture. Physicians and biomedical scientists have their own language and culture that they use to communicate among each other. And they almost are unable to come outside of their cultural bubble when talking to their patients. Similarly, patients have their own culture beliefs about doctors. And of course, society has its own concept about patients in particular sickle cell patients and then about doctors in the culture. And so these three cultures seldom overlap. There was one man, Trevor Thompson who was able to span the cultural gaps between patients, doctors and the biomedical society. And Trevor was unusual, he had sickle cell disease and he founded the Sickle Cell Disease chapter of Tennessee in Memphis. And he really spend a lot of volunteer hours, 40,000 or more hours in the name of Sickle Cell aware. And it's trying to get patients to understand each other and to understand the medical complex and vice versa. He organized walks, he went to medical meetings and unfortunately died, uh too early, uh, in 2016 of his disease after having made an impact in the entire United States uh in the name of sickle cell disease. So we named a $10 million research project after Trevor called the Step Up project. And this was a project to do what Trevor wanted done. Get the doctors, get the patience and get the community. That's the triad to talk to one another and to manage care together. And so we got this grant to organize several states sickle Cell programs so that the patients, the doctors and the communities in those programs would talk to one another and maybe help save the lives of the young adults. Uh rising out of childhood with this disease, these partners were not accustomed to talking to each other. There's a power imbalance with the community feeling uh disempowered and the doctors feeling quite empowered and the uh patients feeling powerless to deal with either the community or the doctors, no psychological safety ability of patients to say what is on their mind, talk about uh what they would rather do. Uh But simply take medical advice. And so there was a need to improve the triad in order to improve uh communication and improve uh patients lives. The next thing I wanna talk is about the medical home itself. Even though the doctors have the power, they don't have a lot of resources. When it comes to sickle cell disease. There are not enough adult sickle cell providers. There's very poor handoffs from the pediatric to the adult providers. And the patients therefore are very poorly prepared from going to the, from the feathered nest to the dark abyss of adult uh care. They end up medically homeless because there are not enough providers. We estimate 100,000 patients with sickle cell disease in the United States, but only 28% of the patients who even bother to find care. And we estimate that somewhere between 10 and maybe 50,000 patients, only 28% of them get this lifesaving drug called hydro hydroxyurea, which is really uh for the longest time was the only federally approved uh drug to manage this disease. And so the patients are medically homeless and are not taking the medicines that they need to take, especially uh after pediatrics. And then the doctors don't feel prepared to manage leadership and change and certainly to build um like you would a cancer center build a sickle cell center that has financial stability and can make money for the medical center and for them, uh these patients are often uninsured, doctors don't wanna manage them and they don't feel confident uh on what to do uh and, and how to uh pay for it. So you got this iceberg, this big disease that, that is taking a lot of time pain and only partially seen by the medical community because they don't come to the hospital for most of their days. So it's like an iceberg. Only the tip of the iceberg is seen by the providers. There are not enough of them. The quantity and the severity of the disease is underestimated because the patients aren't being seen for most of their days. The doctors don't feel competent to take care of the patients and therefore the patients are left in the abyss, uh managing symptoms alone, no care, coordination and the organ failure kills them. Unfortunately, in their twenties and their thirties, we don't study this. We don't study what happens at home. We don't have good metrics. So that's the why of why we need health-related quality of life measures. We haven't had good ones. The specialist needs to understand a little bit about sickle cell disease. The way you would want a specialist to understand a little bit about cancer if they were a cancer specialist. And we don't have enough of them. The things that they need to know are how to manage this lifesaving drug hydroxyurea, how to give transfusions, how to screen for organ damage, how to manage pain, how to deal with sickle cell emergencies and how to deal with the complicated psychosocial and cognitive issues that plague young adults with sickle cell disease. Patients want doctors who are humble enough to say, I don't know, humble enough to respect that. Maybe the patient does know and that should, they should be treated with the uh dignity and respect that a cancer patient would get or that somebody else who is suffering from a chronic illness would get. They should be held in high regard. They should have, their culture should be respected and they should be complimented. The patients should be compliment.