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ASCAT 2024 | Important considerations when managing a pediatric patient with sickle cell disease
David Rees, FRCP, FRCPath, FRCPCH, King’s College London, London, UK, provides insight into important considerations when managing a pediatric patient with sickle cell disease (SCD), focusing on the two key complications that arise: infections and neurological complications like stroke. Prof. Rees emphasizes the importance of early, aggressive infection treatment, particularly for children who are under-vaccinated or have not received penicillin prophylaxis. Prof. Rees also advises against the broad use of the term “crisis” in medical contexts, advocating for more specific diagnoses to avoid missing severe complications. 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
It’s a very broad question. I’m a pediatric haematologist so I think the, I mean I think, I don’t know where to begin answering that, but I mean the problem in children is two things really. The first one is infections and I think people need to be very aware that children with sickle are prone to infections and if they’re ill then treating infections aggressively at an early stage before you know what’s going on is quite important and that’s particularly true in young children who for whatever reason haven’t been fully vaccinated and aren’t taking penicillin prophylaxis...
It’s a very broad question. I’m a pediatric haematologist so I think the, I mean I think, I don’t know where to begin answering that, but I mean the problem in children is two things really. The first one is infections and I think people need to be very aware that children with sickle are prone to infections and if they’re ill then treating infections aggressively at an early stage before you know what’s going on is quite important and that’s particularly true in young children who for whatever reason haven’t been fully vaccinated and aren’t taking penicillin prophylaxis. And the children we see in London say who die, which is unusual in sickle cell, nearly always it’s from infection and it’s nearly always when there’s been some failure of care in the sense they haven’t received all the vaccinations or they haven’t been given antibiotics in the appropriate way.
The other thing with children that we worry about, although it’s less of a problem now, is children presenting with neurological complications and stroke. And there’s a tendency to use the word crisis in sickle cell, which is kind of meaningful in some ways, but it’s unhelpful in the medical context in the sense that children and possibly adults sometimes get admitted and someone says they’ve got a crisis and that is taken to encompass something meaningful and the fact that it’s kind of assumed they’ve got pain and they’re treated with opiates and so the fact that they’re paralysed down one side and they’ve had a stroke kind of gets missed because there’s an idea that they’ve got a global thing called a crisis really. So I think one thing I would suggest in terms of looking after patients is to avoid using the word crisis in the medical context and to be precise about why someone’s in hospital. If they’re in pain, you should say they’re in pain and if they’ve got chest problems they’ve got chest problems, if they had a stroke they’ve had a stroke. And linking those all together as crisis is unhelpful I think and sometimes leads to important complications being missed.
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