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BSH 2022 | Current and emerging strategies for the management of SCD complications

Sanne Lugthart, MD, PhD, University Hospitals Bristol NHS Foundation Trust, Bristol, UK, talks on the management of sickle cell disease (SCD) complications. Currently, stem cell transplantation (SCT) is the only curative strategy for SCD. Dr Lugthart explains that today, SCD complications are mostly managed with symptom-relieving treatments rather than disease-modifying treatments. Recently, a drug called crizanlizumab was shown to reduce the amounts of vaso-occlusive crises in patients with SCD. However, patients still live with the uncertainty of not knowing when they will have their next crisis. It is therefore important to identify tools that can help predict when a patient will get a pain crisis. This interview took place at the 62nd Annual Scientific Meeting of the British Society for Haematology (BSH) 2022, in Manchester, UK.

Transcript (edited for clarity)

At the moment, there’s no curative treatment available for sickle cell disease, other than a stem cell transplant, and managing acute complications in patients with sickle cell disease is more based on symptom relief such as pain relief, when they have it. When they feel tired or they have an anemia, we give them a red blood cell transfusion. When they have an infection, we give them antibiotics...

At the moment, there’s no curative treatment available for sickle cell disease, other than a stem cell transplant, and managing acute complications in patients with sickle cell disease is more based on symptom relief such as pain relief, when they have it. When they feel tired or they have an anemia, we give them a red blood cell transfusion. When they have an infection, we give them antibiotics. To prevent the actual veno-occlusive crisis or the painful crisis that’s the hallmark of sickle cell disease, there’s certain new treatments at the moment available in England and one of them is crizanlizumab.

And what that does, it prevents the amount of VOCs per year for a patient, but the uncertainty of when that painful crisis comes is not taken away. So the patients still live with the disease on a day to day basis, not knowing if they’re going to get a crisis, yes or no. So if we have tools that predict when a patient can get a painful crisis, I think that would be a way forward, and hopefully, with more treatment or disease modifying treatments, we’ll be able to tackle reducing the amount of painful crisis people have.

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Disclosures

Novartis, GBT