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Haemoglobin Concentration Improves Prediction of Cognitive Outcomes in Patients with Sickle Cell Anemia

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Sickle cell anemia (SCA) is associated with cognitive challenges that often worsen as children age. Anna Hood discusses the first study of pediatric patients with SCA to examine the extent to which indices of Haemoglobin Concentration control may provide additional prognostic information.

Image courtesy of interviewee. July 10, 2024

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This video discusses sickle cell disease (SCD), the most common inherited disorder globally, which is rapidly growing in the UK. SCD affects hemoglobin in red blood cells, leading to their sickle shape, which impairs oxygen transport throughout the body, particularly affecting the brain. This video can result in cognitive challenges, especially in processing speed and executive functions, crucial for daily life and education.

The author highlights a study aimed at examining the relationship between hemoglobin levels and cognitive outcomes over time, utilizing longitudinal data collected from children with SCD in the UK from 2005 to 2014. Results indicated that higher mean hemoglobin levels were significantly associated with better processing speed but not necessarily with working memory or the presence of silent infarctions in the brain.

This video also emphasizes recent advancements in treatments for SCD, including emerging drugs that specifically target hemoglobin improvement, which could lead to better cognitive outcomes. The author calls for more longitudinal studies and the integration of cognitive assessments in clinical trials for SCD treatments to provide a more comprehensive understanding of the disorder's impact on patients' cognitive functions.

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Sickle cell or sickle cell, disease

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or anemia, as it's often called,

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is actually the most common inherited disorder in the world

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and is actually the fastest growing

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inherited disorder here in the uk.

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Um, and what it affects is the hemoglobin

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in our red blood cells.

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And so hemoglobin is what carries oxygen around the body.

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Um, and in, sickle cell, that,

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process sort of breaks down.

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Um, and, that process breaking down means the,

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red blood cells take on the shape

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of a sickle giving it its name,

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and it means that those cells are poor oxygen carriers.

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So they, you know, you've gotta get oxygen to all

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of our organs around the body, including the brain,

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which is our biggest sucker, essentially,

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really needs oxygen.

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Um, and so those sickle cells don't carry oxygen very well.

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And unfortunately they're quite sticky

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and they get caught in the red blood cells,

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and, in our vessels

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and stop the healthy blood cells getting through.

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And so, one of the challenges that can face

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because of having lack of oxygen is

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to the brain, as I mentioned.

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And so not having enough oxygen to the brain,

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as you can imagine, can cause sort

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of this cascade of problems.

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But one of the things I'm particularly interested in is how

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that affects cognition.

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So the thinking abilities, what we need to be able to do at,

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at school to do well, for the chil,

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young children and young people.

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And then obviously into adulthood, being able

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to manage our day-to-day lives.

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And so not having, enough hemoglobin that's going

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to the brain means

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that these cognitive challenges occur sort of across

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many different types of cognition,

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but particularly around, processing speed.

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So being able to take in information, understand what it is,

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and process it quickly.

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And something called executive functions, which are sort

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of these higher level,

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cognition challenges and thinking abilities.

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So being able to do multiple things at once

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or flex, be flexible from moving one to,

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from one thing to the other.

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And so this study was, because, myself

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and my colleagues have been interested in this area in

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thinking about how can we, improve hemoglobin,

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and so what's the process, that's happening?

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What are the underlying, underlying mechanisms so

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that we can improve, different outcomes

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and particularly how we can improve, cognitive outcomes.

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The literature, really, is a little bit mixed.

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So in terms of the knowledge that hemoglobin is the thing

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that, is the problem in sickle cell, is well known,

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well established,

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and has really been sort of the heart of the research

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that we, we do, but how it might affect cognition.

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There's, some earlier studies that have looked at this,

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and that it's been a little bit mixed as to whether

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or not, hemoglobin might have this sort

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of more direct effect on cognition.

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Does there have to be, you know, we, we see changes

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that happen in the brain.

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Something called, oxygen, extraction fraction,

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blood flow.

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So these changes to the brain, but how much

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Is it that, hemoglobin changes these things?

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And then cognition, has been a little bit mixed.

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One of the reasons, we wanted to do this study was

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because what hadn't been done

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before was looking at hemoglobin longitudinally.

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So looking at over time, getting lots

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of different measurements from the children that we studied.

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Um, and so in some of my previous work, what we were able

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to show is that, for, children

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and young people who have sickle, oftentimes they are

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receiving blood transfusions.

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So they're getting blood transfusions

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because, they need to be able to,

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replace those sickle, blood cells,

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with healthy blood cells.

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And so in my previous research, we showed

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that the closer somebody is to that transfusion,

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the closer they've had to new hemoglobin coming in,

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the better they do on cognitive tasks.

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And then when we looked directly at hemoglobin, it seemed

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to show that that was there.

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So, we really wanted to be able to look at that,

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but look at lots of different measurements.

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'cause maybe it's because they're just

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looking at one measurement.

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We wanted to look at multiple.

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And so looking at multiple really is

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the, the key for this study.

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And then hopefully we can get a better idea of

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how people are doing over time.

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And what I had done in previous work,

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and there'd been some previous work in diabetes

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and, in my work in,

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a disorder called Phenyl Keel Noria, was to show

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that if you look at either blood in Phenyl, keel Noria,

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HB C one in diabetes, look at that over time.

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That is a better measurement.

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So is it, you know, looking at over time

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and is there variability over time?

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And that's what we wanted to do here.

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This is a longitudinal study, obviously be able

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to be able to do this over time.

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Uh, this study, was, there was a UK base

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and a US base, but we are primarily here in this study just

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look at, UK data.

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Um, and so we collected data from 2005 to 2014.

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Um, and we had looked at sort of every hemoglobin available

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that, the, children

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and young people had when they went into clinic.

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So when they go into clinic, they get their

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hemoglobin measured, right?

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We don't want it to going, too low

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because that's really indicative of anemia,

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and then we need to have a transfusion or intervention.

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Um, and then we also followed up again in 2017

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and 2019, we didn't report it here,

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but we, you know, we got multiple measures including MRI,

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but obviously we also got, cognitive data

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to be able to look at.

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Um, and in terms of our demographics, all of the children,

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were, identified as black British,

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about an even split of males and females.

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Uh, almost 50% of each.

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Um, we, almost all of them had,

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a genotype called the HBSS genotype,

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which is the most clinically severe form of sickle cell.

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Um, nearly half of the children had something called a

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silent infarction, which means that, which a lot

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of children with sickle cell, prior

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to our interventions used to have stroke,

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but still many children have something called an infarction.

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So there's still something there on the brain,

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but it hasn't, doesn't have outward neurologic signs,

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but still has an impact on the brain.

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And then about, a fifth of our children were taking,

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a drug called hydroxyurea, which is a drug

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that actually affects something called a fetal hemoglobin,

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so changes hemoglobin in some way.

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Um, and so we were able to look at,

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these different cognitive markers, particularly

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around processing speed

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and executive function, as I mentioned earlier.

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Um, and we also looked at the mean lifetime hemoglobin, so

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that hemoglobin over time.

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Uh, we looked at the median as well,

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just in case there were, you know, large,

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deviations from that mean, just to be able to get,

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a good measure.

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But they were reasonably similar actually, the mean

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and median, we looked at standard deviations

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that's looking at variability.

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So, so they're going up and down quite a lot.

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Or we've got like, you know,

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level staying, pretty similar.

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And then we also looked at, closest to data collection,

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which is what the other studies had normally done,

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just looked to see, is that, is that a,

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similar to a lifetime?

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Is that a good measure? And actually wasn't

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that different, but it was different.

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And so, generally looking at those, you know,

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those variables gave us more insight into be able to seeing

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what might be related to, cognition.

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And what we actually found was that,

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that processing speed measure, a measure of processing speed

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and, but not our measure of,

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working memory was significantly associated with hemoglobin

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and actually was associated with, the, mean,

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we didn't, we saw small,

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but non-significant correlations between,

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variability, in terms.

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And interestingly though, that medication that I mentioned,

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and was not related and neither was having that infarct.

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So it, in terms of the cognitive status,

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so it really was, seemed to be driven by

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how much oxygen does this child have to the brain?

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One of the things that,

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has come in the last few years is there has been really this

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proliferation of new drugs for sickle cell.

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Um, prior to, you know, really about this last five years,

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we really only had that one drug, the hydroxyurea

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that I mentioned, and chronic transfusion.

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So those were really our only tools.

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And so, more recently,

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and I think that which fits in with our findings is,

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there are, drugs now

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that are actually working on specifically,

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changes to hemoglobin.

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And so, there's a couple of drugs, one's called Altor,

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another is called acetor.

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Um, but both of those drugs actions work

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by increasing hemoglobin.

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And so, those types of, drugs are,

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their primary outcomes are to reduce, you know,

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this organ damage and secondary outcomes are to reduce pain.

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But what also seems to be happening, from our,

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studies, which suggest is that if we can re

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if we can make hemoglobin better, which is increasing it,

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we should also see these improvements in cognition.

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Um, you know, there are now curative options

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with sickle cell, but they're, they're very expensive.

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Um, so it was licensed here.

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CRISPR was licensed here in the UK very recently.

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Um, but it didn't pass NICE guidelines, which is

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what needs to be to get to the NHS.

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So currently costs sort of 1.5 million,

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to be able to do that.

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We're hoping to move towards that.

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Um, individuals, from my previous study in this study,

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it suggests the keeping, with chronic transfusions

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for those who are on them, because

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that really helps regulate.

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So hopefully now we've got sort of this, sort of toolkit

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for the, for our individuals living with sickle cell

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to think, okay, what, what might you need?

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Is it, is it one of these drugs, these medications

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that increase hemoglobin?

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Is it chronic transfusion or is it a curative option?

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But always thinking about this hemoglobin

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because it does seem to be having multiple effects,

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not only improving organ damage,

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but also improving what's happening in the brain,

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and particularly what's happening in cognitive functions.

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I think what we, would like

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to see is other longitudinal

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investigations of hemoglobin, right?

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So this is really one of the first to be able to do that.

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As I said, we're only looking at these static time points.

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And then with these investigations of these new drugs,

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look at having a cognition

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as an endpoint in a clinical trial.

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So currently, you know, oftentimes an endpoint is,

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you know, some form of this, clinical severity,

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which is very important.

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But patients

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and families tell us how important, you know, their kids

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and school is to them as well.

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And so we have written another paper that's shown that,

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provided cognitive tests that can be done, as part

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of clinical trials, provided some of the tools to be able

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to think how do you, how do you use them,

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how do you incorporate them into clinical trials?

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You know, obviously you'll still have your main

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primary outcomes.

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We're asking that they be incorporated

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as secondary outcomes in clinical trials as well.

"Understanding Sickle Cell Disease: Impact on Hemoglobin, Oxygen Delivery, and Cognitive Functioning"

00:00:00 - Sickle cell or sickle cell, disease or anemia, as it's often called, is actually the most common inherited disorder in the world and is actually the fastest growing inherited disorder here in the uk. and what it affects is the hemoglobin in our red blood cells. And so hemoglobin is what carries oxygen around the body. and in, sickle cell, that, process sort of breaks down. and, that process breaking down means the, red blood cells take on the shape of a sickle giving it its name, and it means that those cells are poor oxygen carriers. So they, you know, you've gotta get oxygen to all of our organs around the body, including the brain,
00:00:45 - really needs oxygen. and so those sickle cells don't carry oxygen very well. And unfortunately they're quite sticky and they get caught in the red blood cells, and, in our vessels and stop the healthy blood cells getting through. And so, one of the challenges that can face because of having lack of oxygen is to the brain, as I mentioned. And so not having enough oxygen to the brain, as you can imagine, can cause sort of this cascade of problems. But one of the things I'm particularly interested in is how that affects cognition. So the thinking abilities, what we need to be able to do at, at school to do well, for the chil, young children and young people.
00:01:26 - to manage our day-to-day lives. And so not having, enough hemoglobin that's going to the brain means that these cognitive challenges occur sort of across many different types of cognition, but particularly around, processing speed. So being able to take in information, understand what it is, and process it quickly. And something called executive functions, which are sort of these higher level, cognition challenges and thinking abilities. So being able to do multiple things at once or flex, be flexible from moving one to, from one thing to the other. And so this study was, because, myself and my colleagues have been interested in this area in
00:02:09 - and so what's the process, that's happening? What are the underlying, underlying mechanisms so that we can improve, different outcomes and particularly how we can improve, cognitive outcomes. The literature, really, is a little bit mixed. So in terms of the knowledge that hemoglobin is the thing that, is the problem in sickle cell, is well known, well established, and has really been sort of the heart of the research that we, we do, but how it might affect cognition. There's, some earlier studies that have looked at this, and that it's been a little bit mixed as to whether or not, hemoglobin might have this sort
00:02:49 - Does there have to be, you know, we, we see changes that happen in the brain. Something called, oxygen, extraction fraction, blood flow. So these changes to the brain, but how much Is it that, hemoglobin changes these things? And then cognition, has been a little bit mixed. One of the reasons, we wanted to do this study was because what hadn't been done before was looking at hemoglobin longitudinally. So looking at over time, getting lots of different measurements from the children that we studied. and so in some of my previous work, what we were able to show is that, for, children and young people who have sickle, oftentimes they are
00:03:30 - So they're getting blood transfusions because, they need to be able to, replace those sickle, blood cells, with healthy blood cells. And so in my previous research, we showed that the closer somebody is to that transfusion, the closer they've had to new hemoglobin coming in, the better they do on cognitive tasks. And then when we looked directly at hemoglobin, it seemed to show that that was there. So, we really wanted to be able to look at that, but look at lots of different measurements. 'cause maybe it's because they're just looking at one measurement. We wanted to look at multiple. And so looking at multiple really is the, the key for this study. And then hopefully we can get a better idea of

"Longitudinal Study on Hemoglobin Variability and Cognitive Function in Sickle Cell Disease: Insights and Findings"

00:04:12 - And what I had done in previous work, and there'd been some previous work in diabetes and, in my work in, a disorder called Phenyl Keel Noria, was to show that if you look at either blood in Phenyl, keel Noria, HB C one in diabetes, look at that over time. That is a better measurement. So is it, you know, looking at over time and is there variability over time? And that's what we wanted to do here. This is a longitudinal study, obviously be able to be able to do this over time. this study, was, there was a UK base and a US base, but we are primarily here in this study just look at, UK data.
00:04:54 - and we had looked at sort of every hemoglobin available that, the, children and young people had when they went into clinic. So when they go into clinic, they get their hemoglobin measured, right? We don't want it to going, too low because that's really indicative of anemia, and then we need to have a transfusion or intervention. and then we also followed up again in 2017 and 2019, we didn't report it here, but we, you know, we got multiple measures including MRI, but obviously we also got, cognitive data to be able to look at. and in terms of our demographics, all of the children,
00:05:33 - about an even split of males and females. almost 50% of each. we, almost all of them had, a genotype called the HBSS genotype, which is the most clinically severe form of sickle cell. nearly half of the children had something called a silent infarction, which means that, which a lot of children with sickle cell, prior to our interventions used to have stroke, but still many children have something called an infarction. So there's still something there on the brain, but it hasn't, doesn't have outward neurologic signs, but still has an impact on the brain. And then about, a fifth of our children were taking,
00:06:15 - that actually affects something called a fetal hemoglobin, so changes hemoglobin in some way. and so we were able to look at, these different cognitive markers, particularly around processing speed and executive function, as I mentioned earlier. and we also looked at the mean lifetime hemoglobin, so that hemoglobin over time. we looked at the median as well, just in case there were, you know, large, deviations from that mean, just to be able to get, a good measure. But they were reasonably similar actually, the mean and median, we looked at standard deviations that's looking at variability. So, so they're going up and down quite a lot. Or we've got like, you know, level staying, pretty similar.
00:06:57 - which is what the other studies had normally done, just looked to see, is that, is that a, similar to a lifetime? Is that a good measure? And actually wasn't that different, but it was different. And so, generally looking at those, you know, those variables gave us more insight into be able to seeing what might be related to, cognition. And what we actually found was that, that processing speed measure, a measure of processing speed and, but not our measure of, working memory was significantly associated with hemoglobin
00:07:39 - we didn't, we saw small, but non-significant correlations between, variability, in terms. And interestingly though, that medication that I mentioned, and was not related and neither was having that infarct. So it, in terms of the cognitive status, so it really was, seemed to be driven by how much oxygen does this child have to the brain? One of the things that, has come in the last few years is there has been really this proliferation of new drugs for sickle cell. prior to, you know, really about this last five years, we really only had that one drug, the hydroxyurea

Advancements in Sickle Cell Treatment: The Role of Hemoglobin-Targeting Drugs and Cognitive Outcomes**

00:08:18 - So those were really our only tools. And so, more recently, and I think that which fits in with our findings is, there are, drugs now that are actually working on specifically, changes to hemoglobin. And so, there's a couple of drugs, one's called Altor, another is called acetor. but both of those drugs actions work by increasing hemoglobin. And so, those types of, drugs are, their primary outcomes are to reduce, you know, this organ damage and secondary outcomes are to reduce pain. But what also seems to be happening, from our,
00:09:00 - if we can make hemoglobin better, which is increasing it, we should also see these improvements in cognition. you know, there are now curative options with sickle cell, but they're, they're very expensive. so it was licensed here. CRISPR was licensed here in the UK very recently. but it didn't pass NICE guidelines, which is what needs to be to get to the NHS. So currently costs sort of 1.5 million, to be able to do that. We're hoping to move towards that. individuals, from my previous study in this study, it suggests the keeping, with chronic transfusions for those who are on them, because
00:09:39 - So hopefully now we've got sort of this, sort of toolkit for the, for our individuals living with sickle cell to think, okay, what, what might you need? Is it, is it one of these drugs, these medications that increase hemoglobin? Is it chronic transfusion or is it a curative option? But always thinking about this hemoglobin because it does seem to be having multiple effects, not only improving organ damage, but also improving what's happening in the brain, and particularly what's happening in cognitive functions. I think what we, would like to see is other longitudinal investigations of hemoglobin, right? So this is really one of the first to be able to do that. As I said, we're only looking at these static time points. And then with these investigations of these new drugs,
00:10:57 - as an endpoint in a clinical trial. So currently, you know, oftentimes an endpoint is, you know, some form of this, clinical severity, which is very important. But patients and families tell us how important, you know, their kids and school is to them as well. And so we have written another paper that's shown that, provided cognitive tests that can be done, as part of clinical trials, provided some of the tools to be able to think how do you, how do you use them, how do you incorporate them into clinical trials? You know, obviously you'll still have your main primary outcomes. We're asking that they be incorporated as secondary outcomes in clinical trials as well.

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