• Iron-fortified infant formula for the dietary management of infants and toddlers with maple syrup urine disease.


Iron-fortified infant formula and medical food powder for the dietary management of infants and toddlers with maple syrup urine disease (MSUD).

Last Updated: Monday, October 5, 2015

FREE Metabolic Starter Case
  • Indication
    • BCAD 1 is an iron-fortified infant formula and medical food powder that is free of the branched chain amino acids, isoleucine, leucine and valine, for the dietary management of infants and toddlers with maple syrup urine disease (MSUD). The product provides all other essential amino acids as well as nonessential amino acids, carbohydrate, fat, essential fatty acids, vitamins and minerals. Use under direct and continuing supervision of a doctor.

  • Product Features
      • DHA and ARA

      • Isoleucine-, leucine-, valine-free

      • Vitamin and mineral levels meet the U.S. Infant Formula Act requirements per 100 Calories

      • Increased levels of B vitamins, compared to routine infant formulas, for cofactor production

      • Vanilla scent

      • Mixes easily and stays in suspension well

      • Choline, an essential nutrient important for brain development and the normal functioning of cells1-2

  • Nutrients*
    • Per 100 grams Powder
      Calories 500
      Protein equivalent, g 16.2
      Fat, g 26
      Linoleic acid, mg 4500
      DHA, mg 88
      ARA, mg 175
      Carbohydrate, g 51
      Per 100 grams Powder
      Vitamins/Other Nutrients
      Vitamin A, IU 1520
      Vitamin D, IU 380
      Vitamin E, IU 10
      Vitamin K, mcg 40
      Thiamin (Vitamin B1), mcg 1000
      Riboflavin (Vitamin B2), mcg 1000
      Vitamin B6, mcg 1000
      Vitamin B12, mcg 2
      Niacin, mcg 10000
      Folic acid (Folacin), mcg 100
      Pantothenic acid, mcg 3800
      Biotin, mcg 38
      Vitamin C (Ascorbic acid), mg 60
      Choline, mg 124
      Inositol, mg 86
      Taurine, mg 30
      Carnitine, mg 50
      Per 100 grams Powder
      Calcium, mg 660
      Phosphorus, mg 440
      Magnesium, mg 66
      Iron, mg 9.6
      Zinc, mg 8.6
      Manganese, mcg 380
      Copper, mcg 860
      lodine, mcg 76
      Selenium, mcg 14.1
      Sodium, mg 240
      Potassium, mg 600
      Chloride, mg 500
      Molybdenum, mcg NA
      Chromium, mcg NA

      * Product nutrient values and ingredients are subject to change. Please see product label for current information. † Protein is incomplete since it does not contain the essential amino acids isoleucine, leucine, and valine. NA = None Added

  • Nutrient Facts
    • Protein Equivalent (% Calories) 13
      Fat (% Calories) 47
      Carbohydrate (% Calories) 40
      Potential Renal Solute Load (mOsm/100 g powder)3 147
      Osmolality (m0sm/kg water) 330
      Osmolarity (m0sm/L) 300

      † Protein is incomplete since it does not contain the essential amino acids isoleucine, leucine, and valine. ‡ Determined at 20 Cal/fl oz dilution.

  • Product Form
    • BCAD 1 is available in powder.

      Item #: 892801
      Description: Powder
      Unit Size: 1 lb can
      Cal./Unit: 2280
      Prod. Yield / Unit (fl oz): Varies
      Case: 6 cans per case
      HCPCS Code: B4162
      Reimbursement Code: 00087-510184

      HCPCS Code

  • Composition
    • Ingredients: Corn syrup solids, vegetable oil (palm olein, coconut oil, soy oil, high oleic sunflower oil), amino acids (L‑glutamine, L‑lysine hydrochloride, potassium aspartate, L‑proline, L‑alanine, L‑arginine, L‑phenylalanine, L‑tyrosine, L‑serine, L‑threonine, glycine, L‑histidine, L‑methionine, L‑tryptophan, L‑cystine), modified corn starch, sugar, calcium phosphate, and less than 1%: Mortierella alpina oil§, Crypthecodinium cohnii oil||, ethyl vanillin, sodium citrate, potassium citrate, magnesium oxide, ferrous sulfate, potassium chloride, zinc sulfate, cupric sulfate, manganese sulfate, sodium iodide, sodium selenite, choline chloride, inositol, ascorbic acid, niacinamide, calcium pantothenate, vitamin B6 hydrochloride, thiamin hydrochloride, riboflavin, vitamin D3, folic acid, biotin, vitamin K1, vitamin E acetate, vitamin A palmitate, vitamin B12, taurine, L‑carnitine.

      § A source of arachidonic acid (ARA) || A source of docosahexaenoic acid (DHA)

  • Preparation of Feedings
    • The child's health depends on carefully following the instructions. Proper hygiene, preparation, dilution, use and storage are important when preparing product. Powdered products are not sterile and should not be fed to premature infants or those persons who might have immune problems unless directed and supervised by a medical professional.

      Discuss with parents whether they need to use cooled, boiled water for mixing and whether they need to boil clean utensils, bottles, and nipples in water before use.

      Metabolic clinician will advise parents about the correct amounts of water and powder. Pour the required amount of water into the bottle. Add the correct amount of powder, cap the bottle and shake vigorously.

      If instructed to use the scoop in the can, each scoop (unpacked and leveled) delivers approximately 4.5 g of power. Store DRY scoop in can.

      Failure to follow these instructions could result in severe harm. Once prepared, product can spoil quickly. Either feed immediately or cover and store in refrigerator at 35°–40°F (2°–4°C) for no longer than 24 hours. Do not use prepared product if it is unrefrigerated for more than a total of 2 hours. Do not freeze prepared product. After feeding begins, use within 1 hour or discard.

      Powder Storage (cans)

      Store cans at room temperature. After opening can, keep tightly covered, store in dry area, and use contents within 1 month. Do not freeze powder, and avoid excessive heat. Use by date on bottom of can.


      To be used only under the supervision of a doctor.

      This product is nutritionally incomplete. Care must be taken to provide enough isoleucine, leucine, and valine to support growth, using other foods with these amino acids as required. Medical professional must carefully and constantly supervise the use of BCAD 1 with other foods and liquids and adjust the diet based on frequent blood tests.

      Nutritional powders are not sterile.

      WARNING: Do not use a microwave oven to warm product. Serious burns may result.

      Not for parenteral (I.V.) use.

  • Product Characteristics
    • Fat

      Fat provides 47% of total calories in BCAD 1. The fat content is similar to the fat content of human milk and consists of a four-oil vegetable blend of palm olein oil (44%), soy oil (19.5%), coconut oil (19.5%), and high oleic sunflower oil (14.5%) along with ARA and DHA from single cell oils (2.5%). This fat blend provides a fatty acid profile similar to the fatty acid profile of breast milk.

      ¶ Comparison based on fatty acid profile of typical mature U.S. breast milk.


      Protein equivalents provide 13% of total calories in BCAD 1. The product provides the essential amino acids (except isoleucine, leucine, and valine) as well as nonessential amino acids (see Amino Acids table); 38% of the amino acids are essential amino acids. Since the product contains no isoleucine, leucine and valine, care must be taken to provide enough isoleucine, leucine and valine from breast milk, infant formula or other foods to support growth.

      Amino Acids

      Essential Amino Acids Per 100 g Powder
      Histidine, mg 550
      Isoleucine, mg 0
      Leucine, mg 0
      Lysine, mg 1770
      Methionine, mg 470
      Cystine, mg 340
      Phenylalanine, mg 920
      Tyrosine, mg 920
      Threonine, mg 760
      Tryptophan, mg 410
      Valine, mg 0
      Nonessential Amino Acids
      Arginine, mg 1180
      Alanine, mg 1280
      Aspartic acid, mg 1560
      Glutamic acid, mg 3100#
      Glycine, mg 710
      Proline, mg 1560
      Serine, mg 780

      # Added as glutamine.


      Carbohydrate provides 40% of total calories in BCAD 1. The carbohydrate blend is corn syrup solids, modified corn starch, and sugar. The sucrose in the product helps neutralize the taste of the amino acids. BCAD 1 is suitable for someone with lactose intolerance.

      Vitamins and Minerals

      Vitamin and mineral levels in BCAD 1 meet U.S. Food and Drug Administration requirements per 100 Calories for vitamins and minerals as mandated by the U.S. Infant Formula Act and the associated Code of Federal Regulations 21 C.F.R. part 107.1004. Amounts of B vitamins have been increased above levels in routine infant formulas for cofactor production. An increased level of choline, an essential nutrient important for brain development and the normal functioning of cells1-2, is provided. Calcium, iron, zinc, manganese, and copper levels are higher in order to help meet the needs for these minerals when low-protein solid foods are introduced.

  • References
    • 1. Food and Nutrition Board, Institute of Medicine, Choline. In: Dietary References Intakes for Thiamin, Riboflavin, Niacin, Vitamin B6, Folate, Vitamin B12, Pantothenic Acid, Biotin, and Choline. Washington, DC: National Academy Press; 1998:390-422.

      2. Zeisel SH, da Costa KA. Nutr Rev. 2009;67(11):615‑623.

      3. Foman SJ, Ziegler EE. Renal solute load and potential renal solute load in infancy. J Pediatr. 1999;134:11-14.

      4. Nutrient Requirements for Infant Formulas. Code of Federal Regulations 21, C.F.R. 107.100.