Management
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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
Topic 5.1.1  Link to Topic 5.1.1
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Evidence

Some women with IMD, including PROP, have symptoms of metabolic decompensation that correspond to their menstrual cycles (F.3399). The use of hormonal birth control has been effective and shown to be safe in helping to regulate menstrual cycles and for planning pregnancies among women with PROP (F.3399). It has been suggested that a woman with PROP begin discussion of pregnancy options in adolescence (F.3399). 

Consensus based on clinical practice

Delphi 2:

There was nearly consensus (79% of all respondents agreed) for the use of hormonal birth control in women with PROP who had signs and symptoms of metabolic decompensation corresponding to their menstrual cycle. There was a higher level of agreement among the MD than RD respondents.

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
Topic 5.2.1  Link to Topic 5.2.1
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Evidence

Pregnancy is complicated for the woman with a metabolic disorder and considerations of her own and her fetus’s nutrient needs must be balanced (L.283).  Being in good metabolic control prior to pregnancy helps to establish what intake provides an appropriate clinical, biochemical and nutritional profile.  Successful pregnancy outcomes have been observed in women with PROP  who were in good metabolic control prior to conception and worked closely with both the metabolic and obstetrical teams (F.3399, F.2969, F.3491).  In addition, the possibility of maternal neurocognitive delays may make understanding the treatment modalities and compliance difficult (L.283, F.2969). 

Consensus based on clinical practice

Delphi 2:

There was consensus regarding coordinated care for the woman with PROP during her pregcancy.

100% of the MD and RD respondents agreed that women considering pregnancy should be evaluated by a high risk obstetrics team for possible PROP related complications. 

100% of the MD and RD respondents of the also agreed that women with PROP, who are pregnant, should have a coordinated plan of care for the pregnancy and post- partum period that involves monitoring by both the metabolic team and a high risk obstetrics team.

Topic 5.2.2  Link to Topic 5.2.2
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Evidence

There have been few detailed descriptions of pregnancies in woman with PROP, and the published cases have all been in women described as having somewhat milder forms of the disorder (F.60, F.3466, F.2969). Although this may represent a reporting bias (F.3399), having PROP does not appear to affect fertility in women with the disorder.  In addition there has not been reported any teratogenic effect of the propionic acid on the mental and physical development on the fetus as the infants were all healthy at birth and normal on later developmental testing.(L.283, F.60, F.3491, F.2969).  

Consensus based on clinical practice

Delphi 1:

Only 1 dietitian reported experience treating a PROP patient during pregnancy.   

Delphi 2: 

Only 4 physicians (29%) had treated between 1 and 5 patients during a pregnancy.  None of the dietitians responding had experience treating a pregnant patient with PROP.   

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
Topic 5.3.1  Link to Topic 5.3.1
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Evidence

As in other individuals with PROP, the diet for woman in early pregnancy is a combination of complete (or, intact) protein and medical food with limited propiogenic amino acids (see Q#1 and TABLE #3, Recommended Intakes of PRO and Energy for Well Individuals with PROP). It is suggested that during the first trimester, a goal of 70 grams per day of total protein should be established.  This increases to 85 grams/day during the second trimester and 100 grams/day in the third trimester.  The complete protein versus medical food protein ratio of these recommendations will need to be assessed during this time by measuring plasma amino acid levels and making adjustments accordingly (G.51).

Consensus based on clinical practice

Statements regarding protein intake during pregnancy in women with PROP were not included in the Delphi surveys

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Evidence

The nutrient needs change considerably during pregnancy and ongoing monitoring and guidance regarding appropriate nutrient intake are necessary for women with PROP (F.60, F.3466, F.2969, F.3491).

Consensus based on clinical practice

Delphi 2:

100% of the MD and RD respondents of the also agreed that women with PROP, who are pregnant, should have a coordinated plan of care for the pregnancy and post-partum period that involves monitoring by both the metabolic team and a high risk obstetrics team.

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Evidence

Energy needs increase during pregnancy; a suggested range of 1700-2700 calories per day should be attained based on the assessment of the woman’s clinical status and weight gain (G.51).

Consensus based on clinical practice

There was consensus in both surveys regarding the avoidance of catabolism during pregnancy

Delphi 1:

100% of all respondents agreed that adequate energy needs to be provided both during pregnancy and up to 6 weeks postpartum to prevent catabolism. 

Delphi 2:

91% of all respondents agreed that poor nutritional intake secondary to nausea and vomiting during pregnancy should be treated aggressively to prevent catabolism.

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Evidence

Significant increases in the dosage of oral carnitine, to maintain concentrations of plasma free carnitine in the 20-30µM range, have been reported (G.51, F.60, F.2969). The recommended intake of vitamins and minerals has been based on the DRI for pregnant women. Supplementation may be necessary if these nutrients are not provided in adequate amounts in the medical food and shown to be inadequate by laboratory testing. Also, see Q.1 and Q.4 for further information about vitamin/mineral recommendations and monitoring, respectively. 

Consensus based on clinical practice

Statements were not included in the Delphi surveys about recommendations for vitamin/mineral supplementation during pregnancy

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
Topic 5.4.1  Link to Topic 5.4.1
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Evidence

The metabolic team needs to provide ongoing guidance to advance the diet as indicated by monitoring (F.2969). Women may also need guidance to meet their nutrient intake requirements and avoid catabolism-induced metabolic decompensation if they have nausea or hyperemesis gravidarum (L.283, F.3491, F.2969).  Complete protein may need to be increased by > 50% over the course of the pregnancy (F.60) necessitating frequent monitoring of the amino acid profile, protein status, maternal weight gain and fetal growth (G.51) in addition to routine monitoring.(See Q.4).

Consensus based on clinical practice

Delphi I:

There was consensus (82% of the MD/RD respondents agreed) that a lower protein intake is required after childbirth than during pregnancy unless the patient is breastfeeding. 

Comments: this intake would depend upon the type of delivery as well as whether or not the patient is breastfeeding.

There was consensus (91% of respondents agreed) that there should be continued monitoring of metabolic labs after childbirth until at least 6 weeks postpartum. 

Comments: it was noted that labs should be monitored beyond that when clinically indicated..

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
Topic 5.5.1  Link to Topic 5.5.1
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Evidence

To prevent catabolism during the period of labor and delivery, several authors have reported success in giving IV 10% glucose at 2cc/kg/hr (L.283, F.2969, F.3399); while one individual was given D10, 0.45% NaCl at 1.5 times maintenance along with infusion of intralipid 20% at 2g/kg/day (F.3466). Energy intake at these levels prevented metabolic decompensation in these individuals. IV carnitine was also given in one case (F.3466). IV access should be maintained until sufficient postpartum enteral feeds are established.

Consensus based on clinical practice

Delphi 2:

There was consensus (100% of all respondents agreed) that the plan for labor and delivery should address the prevention of catabolism including the use of IV access for nutrition, medication and fluids. 

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Evidence

There is a large decrease in the protein needs of the woman with PROP after delivery. In addition, there is the protein load from catabolism following the involution of the uterus. Several authors have suggested that this puts the woman with PROP at risk for serious post-partum decompensation (F.2969, L.283, F.3491 F.3399). However, in none of the reported pregnancy outcomes for women with PROP did they experience such problems in the post-partum period (F.60, F.3466, F.2969). As these were all women with milder forms of PROP, providers should be cognizant of the theoretical possibility of post-partum decompensation in woman with PROP.

Consensus based on clinical practice

Statements were not included in the Delphi surveys about recommendations for protein intake after delivery.

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Evidence

No reports of women with PROP breast feeding their infants have been published. Should a woman with PROP want to breast feed, experience gained from other IMD, such as other organic acidemias and MSUD, may help to guide the recommendations (https://southeastgenetics.org/ngp/guidelines).

Consensus based on clinical practice

Delphi 2:

79-80% of the respondents agreed that a mother with PROP, who delivers a baby without PROP, can breastfeed her infant with the understanding that it requires management and monitoring of the mother's protein intake.

However, only 32% of the respondents agreed that it is appropriate for a mother with PROP to breastfeed a baby with PROP.

Comments: the RD respondents were is less agreement than the physicians.  There was concern about the protein load in the baby, but there was some support in the use of pumped breastmilk rather than allowing breastfeeding. 

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