Thread by @TransfusionWM

Amrana Qureshi on use of hydroxycarbamide in sickle cell disease
b-s-h.org.uk/guidelines/gui…
#BSH2019

HU increases HbF%, reduces adhesion receptors, increases red cell hydration and survival and increases availability of NO #BSH2019

HU should be offered to
All infants aged 9m to 42m (following the BABY HUG study)
All adults and children post severe or recurrent ACS
All adults and children with recurrent crises affecting QOL
#BSH2019

Children on a transfusion programme for abnormal TCD can switch to HU after 1 year if no MRA-defined severe vasculopathy (following Twitch study) BUT need to get to max tolerated dose ASAP #BSH2019

TCDs 170-200cm/s is an indication for HU, to prevent progression to high risk category and subsequent enrollment onto a transfusion programme (SCATE study) #BSH2019

Can also be used to prevent end organ disease although evidence base is weaker #BSH2019

Recommendations are for SS and SB0 but can be considered for patients with other genotypes and severe disease #BSH2019

No risk of leukaemogenesis identified after 20 years of longitudinal data #BSH2019

Some concerns over spermatogenesis although changes appear to be reversible on cessation. Pre treatment sperm storage is suggested where possible #BSH2019

No evidence wrt teratogenicity so HU should be stopped prior to stopping contraception, although those with a severe phenotype may need to be considered for a transfusion programme while off treatment #BSH2019