Menstruation, fertility, pregnancy, labor/delivery, postpartum recovery and breastfeeding pose challenges in the management of woman with PROP. Women with PROP have been able to successfully use hormonal birth control to plan pregnancy and/or to regulate their menstrual cycles.The management of relatively few pregnancies in women with PROP has been published, and these described women with milder forms of the disorder; however, it appears that infertility is not an issue.  In the few pregnancy cases documented, the infants born to women with PROP have had normal growth and development, and elevated maternal propionate appears to have no teratogenic effect on the development of the fetus. Pregnancy does present some challenging issues as the maternal and fetal nutrient requirements increase over the course of gestation. There is a need to balance the increased nutrient intake to support the pregnancy while limiting propiogenic amino acids. Close monitoring of nutritional, biochemical and clinical status (including weight gain of the mother and fetal growth) is necessary throughout the pregnancy. The risk for catabolism-induced metabolic decompensation is increased if the woman has nausea with poor intake or hyperemesis gravidarum during her pregnancy. Catabolism may also be a risk if there is a prolonged labor and/or complicated delivery; this necessitates IV access to meet energy, fluid, and, possibly, carnitine needs. While there are no documented cases of women with PROP breastfeeding their infants, for the woman who wants to breastfeed, nutrient demands will be theoretically similar to those during her third trimester of pregnancy; careful monitoring of both the mother and infant is important.

Definitions of the protein terminology used throughout this guideline are listed in Appendix B.