General Updates | Should MRI brain assessment be routinely performed in all patients with sickle cell disease?

This was an interesting session. We had a discussion, it was more like a conversation rather than a debate, that we had in the ASCAT conference this year. And personally, I think we might need to differentiate between MRI scans in children with sickle cell anemia in this case, so SS and S beta-zero thalassemia, rather than any other type of sickle cell disease. And I, in my mind and based on what is clinical practice and evidence, I think it’s definitely helpful and it should really be done in all children with sickle cell. In an asymptomatic child with sickle cell anemia who has regular transcranial Doppler monitoring, I think there should at least be one MRI scan done at a time when they are able to stay still and not need a GA or a general anesthetic for an MRI scan. And I think there can be a lot done based on what you glean from that information. And to me, the purpose of screening anybody for any condition is really predicated on the fact that you can do something about it. So there’s not much point in screening for something that you can’t do anything about. So, for example, if you had a child who comes up with significant, let’s say, silent cerebral infarctions, then you can be more careful about what kind of interventions you offer for that particular child. 

I would, in my own mind, feel that maybe doing one MRI scan in sort of late primary school age and then doing another MRI scan at kind of late secondary school age before they transition to adults is probably the best way of doing it. So the problems that you see in the late primary school age might be quite different for the problems intracerebrally that you see in the late secondary school age, where we are particularly interested in seeing if there are any aneurysms or if there is any ongoing problems with cerebral vasculopathy, particularly at a time when you’ve stopped screening for transcranial Doppler because they’ve gone past 16. So in children, I think it’s a lot more straightforward. It’s a lot more cut and dry. You can investigate them and then initiate some kind of treatment, which could even include discussions about stem cell therapies if you find all of a sudden that there is significant vasculopathy which you haven’t been able to pick up on a TCD scan. 

With adults, it’s a little bit different in my view. I think in principle, one might feel that every adult should have an MRI scan at some stage of their lives, particularly, I guess, in the younger age when there is ongoing risk of intracranial hemorrhage due to aneurysmal disease, for example. But I think it’s difficult to have an open-ended plan of screening, say, annual MRI scan or a five-yearly MRI scan, because partly you’re probably, you’re kind of likely to become stuck as to what you do once you find what you find. Because what we know about children and the risk of strokes and any kind of cerebrovasculopathy is very different from what, you know, what you see in adults. And particularly when the older the adult becomes, the more there is cardioembolic strokes, et cetera, et cetera. So they are kind of a slightly different way of approaching. 

So, I know that there was a discussion about sort of, you know, functional MRIs, you know, to look at cognition, et cetera. And again, it’s great that these things are available and people are having the funding to use it, to access it. But actually it will be quite a lot of input and potential area of parental and patient worry, particularly patient worry when they’re adults, and I’m not entirely brought up to the idea that this should be universally to everybody. But in theory, yes, for a young adult, just getting into the adult care, it’s good to have a baseline to know what your patient has already had in terms of cerebrovascular disease, particularly if they’ve not had anything in their early childhood. 

And finally, just before I stop, now curative options are offered to many, many adults. And having that baseline is also very helpful because then you know that if they’ve got an undiscovered stroke, let’s say, in the past, It’s helpful to delineate that in case, you know, what you do with the patient in terms of referring them onto gene therapy or bone marrow transplant, I think it will have a great bearing on which pathway the patient might take.

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