Pages 26

Sickle cell disease is characterized by the formation of an abnormal globin molecule (substitution of valine for glutamic acid residue at amino acid 6 position of the betaglobin chain). In the UK, sickle cell crisis occurs in 35% of affected pregnant women and is associated with 1-2% maternal mortality and a 4-6 times increase in perinatal mortality. Miscarriage, late fetal loss, type 2 IUGR, proteinuric hypertension, sepsis and thrombo-embolism are known complications. The use of heparin in prophylactic doses should be considered if the risk of thrombo-embolism is unacceptably high. Epidural is preferred because of a lower risk of hypotension. Antenatal diagnosis is currently available via CVS, amniocentesis and cordocentesis and recombinant DNA techniques. The IUCD is associated with a theoretical risk of infection and crisis. The POP is recommended.