Prognosis

• The risk of dying is increased if patients have ^b  
  • low-level fetal haemoglobin
  • acute chest syndrome
  • renal failure
  • seizures
  • white cell count > 15 x 10 ⁹ /l

Watch out for:

• acute chest syndrome
  
  • Patients are at increased risk with ^a  
    • younger age
    • low HbF
    • anaemia
    • raised steady state white cell count
  • It can progress to chronic lung disease, which is often fatal. ^b

   

• strokes - usually haemorrhagic in adulthood  ^b
  
  • Further strokes and death are common. ^b
  • Patients with Hb-SS are at increased risk. ^b

   

• priapism  ^c