The Nutrition Management Guideline for MSUD was first published in 2014. Since that time, there have been reports of new research and experiences that have prompted updates of the guideline. The most significant update, which is expected to be published before the end of the year, is the section on pregnancy in the woman with MSUD. In addition to these new recommendations for nutrition management in the guideline, there will be expansion of the toolkit to give practical examples of nutrition management throughout the stages of pregnancy and links to helpful resources. More details about the development of these new recommendations can be found at: https://southeastgenetics.org/ngp/guidelines.

The recommendations for dietitians and other members of the metabolic team to follow when managing the care of women with MSUD before and during pregnancy and after delivery are:

  1. Provide individualized pregnancy planning information to all women with MSUD of childbearing age and update when pregnancy is confirmed.
  2. Adjust dietary intake to allow blood LEU concentrations to be in the 100-300μM range and VAL and ILE in the upper range of normal (200- 400μM) throughout pregnancy
  3. Provide individualized dietary guidance so that nutrient intake meets the changing needs of each phase of pregnancy and the postpartum period.
  4. Provide nutrient, clinical and biochemical monitoring during all phases of pregnancy and the postpartum period.
  5. For the woman with MSUD who wishes to breast feed, closely monitor her diet and laboratory markers in addition to her infant’s growth and development.

The workgroup of dietitians who have been contributing to this effort include the following: Barbara Marriage, Courtney Allgeier, Beth Ogata, Julie McClure, Surekha Pendyal, Nikki Drilias and Dianne Frazier. A notice will be sent when the updates to the guideline and toolkit are finalized and published.