Clinical Case Database / Category: Patient Management
Sickle ß thalassemia and secondary prevention of stroke
Publication details
Philippa Anna Stilwell, Michele Afif
Foundation Years Journal, volume 8, issue 6, p.50 (123Doc Education, London, June 2014)
Abstract
A girl with Sickle ß Thalassemia was started on a long term transfusion programme after suffering from a stroke at the age of 7. This involves regular blood transfusions, with the aim of keeping the percentage of sickle haemoglobin (HbS%) as low as possible, ideally below 30, to reduce the risk of further strokes. Despite a combination of top up transfusions and exchange transfusions, which have allowed some control of the patient's HbS%, ischaemic changes and vascular abnormalities in her brain have deteriorated. She now awaits a stem cell transplantation. Previous international trials have demonstrated that children who have had an infarctive stroke have high risk of having a further stroke if they remain untreated. This risk can be signifiantly reduced if children are established on the transfusion programme. The transfusion programme can only be effective if the HbS% is adequately controlled, which can be difficult, as is seen in our patient. Stem cell transplantation (matched sibling donor/haploidentical) is potentially curative and offers the option for definitive secondary prevention of strokes, but is a toxic procedure and currently reserved for patients with a severe clinical course.
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Authors
Philippa Anna Stilwell (Corresponding author)
FY 1 Doctor, Jack's Place, Paediatrics,
Northwick Park Hospital, Watford Road,
Harrow, Middlesex, HA1 3UJ
annastilwell@gmail.com
Michele Afif
Consultant Paediatrician & Paediatric Lead for Haemoglobinopathy,
Jack's Place, Paediatrics,
Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ
micheleafif@nhs.net
References
3. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet 2010;376:2018-31.
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The Foundation Years Clinical Cases Database is a selection of 600 peer-reviewed clinical cases in the field of patient safety and clinical practice, specifically focused on the clinical information needs of junior doctors, based around the Foundation Year Curriculum programme (MMC). The cases have been chosen to align with the Foundation Year Curriculum.
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