Management
Guidelines
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PROP Nutrition Management Guidelines
First Edition
March 2017, v.1.2
Updated: September 2017
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Nutrition Recommendations
Question
5. For the woman with PROP, what nutrition therapies are associated with positive outcomes during menstruation, pregnancy, delivery and the post-partum period?
Conclusion Statement
Derived from evidence and consensus based clinical practice

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.

Recommendation 5.1

Consider, in consultation with the medical team, the use of hormonal birth control in a woman who has signs and symptoms of metabolic decompensation that coincide with her menstrual cycle.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.2

Advise women who are considering pregnancy to meet with both the metabolic and obstetric teams to establish good metabolic control prior to conception, and to understand the frequency and type of monitoring necessary to optimize outcome.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.3

Provide dietary guidance throughout gestation to help the woman with PROP meet the nutrient intake goals for pregnancy and avoid catabolism. See TABLE #3, Recommended Intakes of PRO and Energy for Well Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.4

Monitor maternal weight gain, fetal growth, and biochemical, nutrition and clinical markers throughout pregnancy as described in TABLE #7, Monitoring the Nutritional Management of Well/Stable Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.5

Have a plan in place for monitoring and preventing catabolism during labor and delivery (including IV access for provision of fluids, nutrients and medications should it be prolonged and/or complicated), and during the post-partum period.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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