EASL Clinical Practice Guidelines

Pregnancy

Successful treatment means that women with Wilson's disease can become pregnant [[148], [149]]. Counseling should indicate that the likelihood of finding a homozygote amongst children is 0.5%; haplotype analysis of the partner is justified. The patient's copper status should be optimized prior to pregnancy. Although there is some concern over the teratogenicity of D-penicillamine, the risks of withdrawing treatment outweigh those of continuing it. A compilation of published case series on 161 pregnancies in 83 women with Wilson's disease (one of them after successful in vitro fertilization) treated with D-penicillamine during pregnancy showed 122 births with 119 normal newborns [150]. A high abortion rate was only observed in a study from India [151].

This is also true for treatment with trientine [152] or zinc [149]. Whether the dose of a chelator should be lowered or not is based on speculations rather than data. The highest risk for fetal teratogenicity is in the first trimester, therefore lowering D-penicillamine during the first trimester was recommended with continued monitoring on the lower dosage for all trimesters [130]. Others recommended reducing the chelators to a minimal dose, i.e. 300–600 mg/day in the last trimester in order to avoid insufficient copper supply to the fetus or insufficient wound healing after Cesarean section or episiotomy [148]. Breast feeding under chelation therapy is not recommended, although there are reports that children breast fed by mothers on D-penicillamine had no problems [153].

Although contraception is an important issue, no detailed studies were performed so far. Estrogens may interfere with biliary copper excretion. In healthy women taking contraceptives, serum copper and urinary copper excretion increased [154], even corneal copper depositions were observed [155]. Many intrauterine devices contain copper. Thus, only spermicide and barrier contraceptives and progesterone-only preparations can be safely prescribed [156].