Nutrition Management After Onset of Menses

1. For pubertal, adolescent, and adult women with a UCD, assess for signs and symptoms of hyperammonemia that coincide with their menstrual cycle. 

2. For pubertal, adolescent, and adult women with a UCD who have signs and symptoms of hyperammonemia that coincide with their menstrual cycle, consider temporarily increasing energy intake (e.g., increase by approximately 10%) and/or temporarily decreasing protein intake (e.g., decrease by approximately 10%) to prevent metabolic instability. 

3. Consider, in consultation with metabolic and primary care providers, the use of hormonal birth control in a woman who has signs and symptoms of hyperammonemia that coincides with her menstrual cycle. 

Preconception Counseling for Risk Assessment

1. Provide annual counseling by the metabolic care team to all women of childbearing age to discuss risks of maternal decompensation during pregnancy, labor, and postpartum. 

2. Perform comprehensive nutritional evaluation and counseling in women who are actively planning pregnancy to support preconception health. 

3. Refer women and men of childbearing age to a certified genetic counselor to support understanding of inheritance risk and reproductive options. 

Nutrition Management During Pregnancy

1. Provide an individualized nutrition prescription for pregnant women to meet pregnancy-specific nutrient goals and prevent catabolism. Titrate protein intake to approach the DRI for the stage of pregnancy based on individual tolerance and guided by ongoing nutrition assessments and laboratory monitoring. See * TABLE #1, Total Protein and Energy Recommendations for Individuals with Urea Cycle Disorders When Well*.

2. Monitor maternal weight gain and biochemical, nutritional, and clinical markers throughout pregnancy as described in * TABLE #2, Monitoring the Nutritional Management of an Individual with UCD when Well*.

3. Aggressively manage pregnancy-related nausea and vomiting to prevent catabolism and metabolic crisis, using antiemetics and nutritional interventions as needed.

Nutrition Management During Labor and Delivery

1. Establish a written management plan with metabolic providers for labor and delivery that includes protocols for IV dextrose, amino acid supplementation (e.g., L-arginine hydrochloride), nitrogen scavengers, and biochemical monitoring. 

2. Monitor plasma ammonia every 4-6 hours and glucose every 1-4 hours during labor and delivery, adjusting interventions as necessary.

3. Continue IV support until the individual resumes adequate oral intake to prevent catabolism and hyperammonemia. 

Nutrition Management During Postpartum Period and Lactation

1. Closely monitor clinical status and ammonia concentrations during the first six weeks postpartum to enable early detection and treatment of metabolic decompensation, with more frequent monitoring recommended during the initial two weeks following delivery. See TABLE #2, Monitoring the Nutritional Management of an Individual with UCD when Well.

2. Provide 100-120% of the DRI for energy in the postpartum period to prevent catabolism. See TABLE #1, Total Protein and Energy Recommendations for Individuals with Urea Cycle Disorders When Well.

3. Resume pre-pregnancy protein intake after delivery unless the individual is breastfeeding. See TABLE #1, Total Protein and Energy Recommendations for Individuals with Urea Cycle Disorders When Well.

4. Support women with mild or severe UCD who wish to breastfeed, including closely monitoring nutrient intake and biochemical markers to ensure adequate protein, energy, and fluid intake to support lactation and metabolic stability.