Management
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VLCAD Nutrition Management Guidelines
First Edition
February 2019, v.1.0
Current version: v.1.1
Updated: February 2019
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Nutrition Recommendations
Question
6. For the woman with VLCAD, what nutrition interventions are associated with positive outcomes during menstruation, pregnancy, delivery and the post-partum period?
Conclusion Statement
Derived from evidence and consensus based clinical practice

There is no information in the formal literature specific to menstruation in an adolescent with very-long chain acyl-coA dehydrogenase deficiency (VLCAD); however, the onset of rhabdomyolysis often occurs in the pre-menarche years (age 9-11 years).

The metabolic changes and energy demands of pregnancy, delivery, and the post-partum period present challenges for the woman with VLCAD. Careful planning between the woman and metabolic and obstetric teams can help minimize decompensations and promote successful outcomes for mother and baby.

Nutrition management during pregnancy requires adherence to pre-pregnancy diet (minimize periods of fasting, maintain long-chain fat (LCF) restriction, medium chain triglycerides (MCT) supplementation, and use of L-carnitine, if prescribed) and providing additional energy in the second and third trimesters of pregnancy. Pregnancy-related nausea and vomiting in the first trimester must be treated aggressively and can help the woman meet nutrition recommendations. Myalgia and rhabdomyolysis are commonly reported complications that can be resolved with 10% intravenous (IV) dextrose support and bed rest.

Routine monitoring of creatine kinase (CK) and acylcarnitine profiles should be completed throughout the pregnancy and post-partum period.

To minimize catabolism during delivery, the woman with VLCAD should receive adequate energy including 10% IV dextrose, monitoring of biochemical indicators of rhabdomyolysis, and medication management.

Women frequently experience rhabdomyolysis in the post-partum period due to inadequate energy intake and catabolism associated with tissue breakdown during the puerperium period. Breastfeeding is not contraindicated; however, it can contribute to catabolism if energy needs are not met. Close monitoring of the woman's nutritional intake and laboratory markers should continue through eight weeks post-partum and as long as the woman is breastfeeding.

Recommendation 6.1

Monitor young women for rhabdomyolysis events during the premenarchal period and provide supportive care.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 6.2

Develop a plan with the metabolic and obstetric team for pregnancy planning, precautions, and monitoring. The plan should include delivery method, pain management, and emergency protocols.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 6.3

Provide nutrition guidance and encourage diet adherence throughout the pregnancy to support a healthy pregnancy and prevent complications such as rhabdomyolysis. Nutrition recommendations should include a high carbohydrate, low LCF diet with MCT supplementation, replenishment of carnitine deficits and avoidance of fasting. Follow other normal pregnancy recommendations for energy and protein needs, vitamin/mineral supplementation and weight gain (RECOMMENDATION TABLE #8, Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well).

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 6.4

Monitor for and aggressively treat pregnancy-related illness and rhabdomyolysis. (See Question 4 for monitoring recommendations.)

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 6.5

Develop a plan with the metabolic and obstetric team to minimize catabolism during labor and delivery. This includes preventing fasting, providing continuous IV support (10% dextrose with appropriate electrolytes at 1 to 1.5 times maintenance), managing medications and monitoring laboratory values.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 6.6

Provide additional calorie support during the post-partum period, especially if the mother is breastfeeding (RECOMMENDATION TABLE #8, Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well). Provide close monitoring for signs of decompensation, rhabdomyolysis, and cardiac complications for up to 8 weeks postpartum and as long as the woman is breastfeeding.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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