Synopsis
Prevent stroke and invasive infection, prescribe antigen-matched transfusion for defined indications, detect alloimmunisation and iron loading, and recognise delayed haemolytic and hyperhaemolysis reactions.
- Functional hyposplenism makes encapsulated bacterial infection a lifelong risk; vaccination, antimicrobial prophylaxis and rapid empirical treatment are complementary layers, not substitutes.
- Children enter a transcranial Doppler programme because persistently abnormal cerebral arterial velocities identify a group that benefits from regular transfusion for primary stroke prevention.
- A new neurological deficit activates the hyperacute stroke pathway and urgent sickle transfusion discussion; a normal initial CT does not exclude ischaemic stroke.
Key red flags
Haemoglobin below the pre-transfusion baseline with haemolysis and reticulocyte suppression or rise requires urgent specialist management before more blood.
Investigation priorities
Stratify primary stroke risk in children with sickle-cell anaemia.
Management branches
Sickle-cell disease is diagnosed or a first planned transfusion approaches.
- Obtain extended antigen phenotype or genotype before transfusion where possible and retrieve records from every previous transfusion provider.
- Record all current and historical alloantibodies and issue a patient-held transfusion alert linked to the haemoglobinopathy network.
Routine review, travel planning or a change in vaccine or prophylaxis status occurs.
Key medicines
PhenoxymethylpenicillinFollow the current sickle and asplenia protocol, commonly 250 mg orally twice daily in adults for ongoing prophylaxis, with macrolide or other local alternative for genuine allergy.
DeferasiroxFor transfusional overload, a common film-coated-tablet starting dose is 14 mg/kg orally once daily; adjust in 3.5–7 mg/kg steps to transfusion rate, ferritin trend and MRI tissue iron. Dispersible tablets use different strengths and must not be interchanged dose for dose.