TY - JOUR AU - Ibrahim, Nadia AU - Mahmood, Sabah AU - O'Driscoll, Sandra AU - Chakravorty, Subarna PY - 2020/09/03 Y2 - 2024/03/28 TI - Effectiveness of a top up transfusion programme in preventing cerebrovascular damage in a birth cohort of sickle cell disease: Single-centre 10-year Retrospective Analysis JF - The Physician JA - phy VL - 6 IS - 2 SE - DO - 10.38192/1.6.2.24 UR - https://www.physicianjnl.net/index.php/phy/article/view/63 SP - 1-8 AB - <p>Regular transfusions are effective in managing strokes in paediatric sickle cell patients. However, there are associated risks, including alloimmunisation and iron overload. This study evaluated the efficacy of top-up transfusions in primary and secondary stroke prevention in a single tertiary paediatric centre in Central London.</p><p>Forty-seven children with sickle cell disease who received transfusions in the last decade were included. No patient on a primary stroke prevention transfusion programme had a cerebrovascular event during the study period but 9.5% on secondary stroke prevention programme did. Twenty-one per cent of patients in this cohort converted to exchange transfusions following transfer to adult services, of which 11% had subsequent strokes. Targeted pre-transfusion haemoglobin S % was not always met; 43% of HbS% readings in a 12- month period were above the set target of 30% and 37% were above the set target of 50%. About a third of patients had evidence of severe hepatic iron overload, but no significant cardiac iron. 25% of patients became alloimmunised, but not severe enough to warrant discontinuation of the transfusion programme.</p><p>Although transfusions are effective for primary stroke prevention, iron overload remains a significant burden.</p> ER -