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ASCAT 2024 | Sickle cell retinopathy: prevalence and managing patients with this complication
Christiana Dinah, BSc, MBBS, MRes, London North West Healthcare NHS Trust, Harrow, UK, provides insight into her talk on sickle cell retinopathy in patients with sickle cell disease (SCD). Dr Dinah discusses the prevalence of sickle cell retinopathy and advises on managing patients who present with this complication. This interview took place at the 19th Annual Scientific Conference of the Academy for Sickle Cell and Thalassaemia (ASCAT 2024) in London, UK.
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Transcript
So today at ASCAT I delivered a talk on sickle cell retinopathy and the pathway to an efficacious therapy, and in summary I aimed to inform the audience on what we know about sickle cell retinopathy, the many unknowns of sickle cell retinopathy and also the next steps in terms of moving us forward towards better interventions and treatments for sickle cell retinopathy.
One of the things that my talk flags is that the incidence and prevalence of sickle cell retinopathy increases with age...
So today at ASCAT I delivered a talk on sickle cell retinopathy and the pathway to an efficacious therapy, and in summary I aimed to inform the audience on what we know about sickle cell retinopathy, the many unknowns of sickle cell retinopathy and also the next steps in terms of moving us forward towards better interventions and treatments for sickle cell retinopathy.
One of the things that my talk flags is that the incidence and prevalence of sickle cell retinopathy increases with age. So increasing age is a risk factor and we know that thankfully due to improved care, life expectancy with sickle cell disease has improved over the last few decades. So yes, you will see that there are more patients potentially with sickle cell retinopathy and maybe more significantly with proliferative sickle cell retinopathy which is the more severe stages.
I also flagged that the prevalence is higher in those with HbSC, three to four fold higher than those with SS and S-thal and other sickle cell genotypes. So for clinicians managing these patients, if they’ve got visual symptoms, then it is important that they are managed with and by an ophthalmologist because you won’t really be able to see into the eye to determine where the symptoms are coming from.
But maybe most importantly, what my talk flagged was that certainly in the UK, we haven’t got screening guidelines. There is NICE recommendations that suggest that everyone with sickle cell disease should see an ophthalmologist at least once in two to three years, but that’s not universal and we’re not seeing that happening everywhere.
Our study, the Sickle Eye Project, is designed to help us have robust data on the epidemiology now in this current climate with hydroxycarbamide and better general care for sickle cell disease, what the prevalence of sickle cell retinopathy is, and maybe help us move forward to predicting who is at risk of having the more severe types of sickle cell retinopathy so that they can have more personalized screening regimens. It’s a start. There are many unknowns, as I flagged in my talk, but certainly it is a very important part because we know from our data that sickle cell retinopathy has a significant deleterious effect on quality of life and vision-related quality of life in general. So therefore, it is an important topic or condition for hematologists to be aware of.
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