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Hello, I'm Doctor Wally Smith, Florence Neal Cooper Smith, Professor of Sickle Cell Disease at Virginia Commonwealth University. And going to talk to you today about uh patient reported outcomes. How to integrate them and measures of health related quality of life into personalized sickle cell care. Uh I have these disclosures and these are my learning objectives. I'd like to be able to utilize patient reported outcome measures or pros and quality of life assessments to personalize sickle cell disease management. How do we do that? We implement the validated pro tools to assess disease burden, pain, severity and freq and treatment effectiveness. We use these insights to guide shared decision making with the patients and optimize treatment selection for individualized patient care. And then we incorporate these assessments into routine practice as we go improving uh quality and improving risk stratification and access to transformative therapies. You may have figured out that pain and quality of life are hopelessly intertwined. We have data showing unlimited impacts of sickle cell disease, pain on patients, health related quality of life on their sleep, on every as aspect of their life, including their psychological and their social health. Here's a diagram that shows Children, uh, and adults and their quality of life scores or a number of diseases that you might be familiar with on the bottom. You see adults and you can see people with dialysis on the right hand side and then you see healthy adults on the left hand side and look at cystic fibrosis. You might think that would be a severe disease, but it is, um, interestingly associated with a better quality of life than either sickle cell disease or cancer. And look at asthma and then look on the top side for Children who have various illnesses and sickle cell disease is not much better in the quality of life for Children. Let's compare diseases. So this is the Pisces study. This is our study of pain and sickle cell that we did over 15 years ago. And we rated the quality of life and that's in the purple. And you see that the scores there are equivalent pretty much to those of dialysis. If you compare dialysis and the like turquoise, they're pretty much the same on at every uh level of uh inquiry, vitality, general health, bodily pain, um physical function and look at how good cystic fibrosis is. Once again, surprisingly, um compared to some of these other diseases in uh teens, uh sleep is a real issue. We have very, very much uh bilateral influences of sleep on pain and of pain on sleep quality. And we have stress related to sleep. And so the the the relationship between sleep and pain, especially in Children is very, very tight. And one of the things we try to teach Children to do is to learn how to get better sleep. But that's not it. We have all these other uh measurements. If you wanna measure fatigue, we have a pa patient reported outcome for that. Actually, there are 23 there. If you wanna measure pain, we have the brief pain inventory, sleep, we have the Pittsburgh sleep quality index, anxiety, we have the state trait anxiety, inventory, depression. Uh You can measure the uh depression, uh a 20 item scale, perceived stress. And then the medical outcome study, which is the oldest uh health related quality of life nationally used uh measure containing 36 items and eight domains. Here's how the scores uh come out with pain and health-related quality of life in patients with sickle cell disease. First, you have the brief fatigue inventory and as you can see, uh people tend to, to uh score down in the worst, worst uh categories. If you look at the uh um the 2nd and 3rd fatigue scores, you see a similar thing and then uh on the righthand side, the straight state trait anxiety, you can see that a full 40% of patients have uh pretty severe trait anxiety. Uh If you look at depression, you can see uh uh some effects of depression. 0 to 60 is the scale. And these patients are scoring at uh 16 and then the sf 36 that's the one most people are familiar with and look at those the physical function uh is definitely affected. But then so is uh physical pain and you can almost directly relate physical pain and sickle cell disease to what the pain uh score is on their SF 36 but not only that their social function, their mental health, their general health all affected by this disease. Then you've got these two major new uh pain and health related quality of life scales. One is called the promise patient reported outcomes measurement information scale on the left. And one is called the ask me the adult sickle cell quality of life measurement scale on the right. And you can see very similar domains. So these have more largely taken the place of the SF 36 and these are the most commonly used measures of health related quality of life in all diseases and in sickle cell disease specifically. And they ask me is sickle cell specific. So the promise it is it is asking about uh their social activity is asking about their activities of daily living, about physical activities, about cognitive function and about emotions. And then the uh scale and means show uh significant um di diminishing uh scores similar to what you see with the SF 36. You can map the scores of the SF 36 onto the promise of scores. And then the uh ask me is a sickle cell specific measure and it evaluates and monitors the same sort of uh the same sort of uh domains, physical mental, social wellbeing. Uh It's accurate for people with sickle cell disease developed among people with sickle cell disease. Comprehensive for the issues related to sickle cell disease. I'll show you some things that they ask me measures in sickle cell disease that other health related quality of life measures do not attempt. Let me just show you what I mean. Um first, it was in intended to characterize the effect of therapies uh and to compare the effectiveness of therapies. But importantly, in italics, evaluate the effectiveness of the healthcare delivery system. The healthcare delivery system in sickle cell disease is woefully inadequate, especially in adults. This instrument captures that. So here is a comorbidity checklist. How sick are you uh coming from as me and you can see here that the severity total was 2.1 you can see on the right hand side, the 34% reported in a low severity group, but 26.8% reported in a high severity group and in the various uh organ functions, all reported by patients uh including the need for transfusion and the need for daily opioids. All uh reported by as me, here's the global health scale for promise, uh similar kind of severity uh scale and excellent noticed only 4.3% of sickle cell patients say they're in excellent health. And then uh the visits uh that relate to quality, look at how many times patients have gone to the hospital, to the ambulatory care um uh or to the emergency department. Uh in the last year, this was important uh work and we were able to show um these changes uh over uh a a long period of time and able to um to document that uh patients with sickle cell disease were um are compromised in their healthcare quality. Look at the usual uh MD. Now that's my clinic. 89% say yes, I have a usual MD. But you can look at other scores and other clinics and not see that uh 89% have a doctor that is dedicated to their sickle cell disease. And then they asked me um scales, look at the emotional impact and look at all the other scales and on the top, look at how they relate to other behavioral or social uh measures. We developed our own Smith Boever sickle cell stress scale. Uh We developed a health readiness for transition scale called the tip R FT. Uh And there are several coping scales that we uh tested this against. And so we were able to show lots of validity uh in the as me scales, things hung together. Uh basically 11 score was low, another score was low, similar correlations between the promise school.