Prevention

Give hydroxyurea in severe cases ^a 
Initial dose 15 mg/kg per day, increase by 5 mg/kg per day every 12 weeks to maximum of 35 mg/kg per day, unless marrow depression noted (neutropenia <2.0 x 10 ⁹ /1, reticulocytes or platelets <80 x 10 ⁹ /l; Hb <4.5 g/dl). If this occurs, stop therapy until recovery. Resume at 2.5 mg/kg per day lower. 

Consider

• depo-provera (methoxyprogesterone acetate) contraception in women ^a  
• folate ^b  
• regular penicillin prophylaxis

There is no clear benefit from:

• routine transfusion therapy in adults  ^d

Priapism

Give oral stilboestrol 5 mg daily to patients with frequent attacks. ^a

Surgery

• Avoid surgery if possible, especially for patients with severe disease ^b  
  
  • If surgery is required, keep it simple ^b  
  • Transfuse patients before surgery (up to Hb > 10. 0 g/dl) ^b

There is no clear benefit from aggressive transfusion before surgery (to achieve SS 30% or less) ^d