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Contraception and sickle cell disease.


 Although women with sickle cell disease have been found to have a delay in puberty of 2.3 years, there is no evidence that they are less fertile than women without hemoglobinopathy. Women with sickle cell disease therefore need adequate family planning advice to prevent unwanted pregnancy. The need for good and comprehensive advice is particularly important given the association among these women between pregnancy and increased maternal and fetal mortality and morbidity rates. Moreover, unwanted pregnancy rates among women with sickle cell disease have been reported as being in the range of 38-64%, and one study found that only 33% of a group of women with sickle cell disease used any form of contraception compared to 66% in a control group. Sickle cell disease is listed in the manufacturers' data sheets in the UK as a contraindication to the use of the majority of combined oral contraceptive pills, but there is little good evidence to support this restriction. In fact, women with the sickle cell trait have no particular added risks and can be offered the usual range of contraceptive methods. Evidence that the use of injectable progestagens may reduce the risk of crises, however, suggests that they should be recommended as a first option of contraception for these women. Good evidence exists that the injectables are safe and effective for women with sickle cell disease despite the adverse publicity which they have received. The IUD appears to be safe as are the common barrier methods, while the combined oral contraceptive pill is a convenient, effective, and reliable form of contraception which should continue to be prescribed for these women, albeit with caution.
