Case 73 – update 2 

Given the girls high Right MCA doppler at screening the patient started regular transfusion for prevention of CVA. This was initiated due to Adams et al 1998 NEJM paper that showed a 92% risk reduction in patients with high dopplers and SCD. She has had a good response to exchanges and so far has not developed any allo antibodies or had any haemolytic reactions. She has an MRI brain that shows no evidence of cerebral vasculopathy. 

After two years of regular exchange transfusion the patient and her mum ask you about the possibility of changing treatment. Her mum feels that the visits for exchange are interfering with school. 

What are the treatment options for this girl two years into successful exchange treatment? 

What evidence may support a change in treatment?

How should a change in treatment be initiated if warranted? 

Reference:

Adams et al. Prevention of a first stroke by transfusions in children with sickle cell anaemia and abnormal results on trans cranial Doppler ultrasonography. NEJM; 339, 5-11.

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