should burp your baby at least once during and again at the end types and amounts of food that your child is allowed, will be CHECKING YOUR CHILD’S PROGRESS of each feeding. Hold him upright against your shoulder or lay outlined. The nutritionist will help you work out a plan that Laboratory Tests. Because your baby grows rapidly during him face down on your lap and gently pat his back. meets your child’s needs and fits into the family budget and the first year of life, blood is tested frequently for BCAA lifestyle. content. Many doctors will ask for a blood sample once or DO NOT WARM THE BOTTLE IN THE MICROWAVE. Lists of foods make meal planning easier and help you be sure twice a week during your baby’s first 6 months, then once a Uneven warming may cause serious burns. your child’s nutrient needs are met. You will have time to week until 1 year of age. After 1 year of age, checking blood become familiar with the food lists and their nutrient content, levels and diet records may be decreased to every 2 to 3 weeks because foods will be added slowly to your child’s diet. if blood BCAA levels are well controlled. Introduction of Solid Foods. No baby is born with the ability Various methods are used to monitor the amino acid to swallow solid foods. The swallowing reflex develops at 2 to 3 When your child is older, you may need to use Free Foods (see and ketoacid levels in the blood and urine. Urine may be months of age. Before this time, the baby’s “tongue thrust” causes Table 1) to meet his energy needs. Free foods, which are high monitored for the presence of ketoacids. Home monitoring the tongue to protrude, making swallowing food difficult. Wait in energy and contain little or no BCAAs, must not replace using dinitrophenylhydrazine (DNPH) or Ketostix® strips ing to feed solid foods until the baby is 4 to 6 months old is best. prescribed foods nor be used in large amounts. If your child may be used as a way of detecting ketones in the urine. How Feeding time should be a pleasant experience for you and Ketonex-1 is similar to infant formula, and the amounts, as eats too many of these free foods, he may become overweight, often your child is tested will be determined by your baby’s your baby. well as the kinds of cereals, fruits, and vegetables prescribed, or the extra sugar may cause tooth decay. Special low-protein metabolic doctor or nutritionist. are about the same as for any baby. Your baby’s nutritionist or foods, including pasta, rice, crackers, cookies, and breads, • Always test the temperature of heated formula before metabolic doctor will advise you about when your baby should can be added to the diet. These foods will help to satisfy your Before taking a blood sample, you may be asked to accurately feeding by shaking a few drops on your wrist. start eating infant cereal and strained baby foods, and which child’s hunger. record your child’s total food and beverage intake. On a • Heating above 100° F (37.8° C) or adding hot water may foods to introduce. Guidelines for adding various foods to your form the clinic will provide, record the name of the food, the cause loss of vitamins A and C and lead to the Maillard baby’s diet are given in Figure 5. However, your nutritionist or Be sure to check with the nutritionist before exact amount in household measures (cups, teaspoons, or reaction—a reaction in which some amino acids bind with metabolic doctor may suggest different ages. Follow their advice. using any food that is not on the food lists provided. tablespoons) or in grams that your child ate, and the BCAA carbohydrate, making them unavailable to the child. content based on the food lists or information given to you by Feeding Your Infant. The way you feed your baby with At 7 to 8 months of age, your child may begin trying to eat foods the nutritionist. MSUD is the same as for any baby. The Ketonex-1 formula such as crackers, low-protein toast, or pieces of fruit without If you have any questions about the content of certain foods, Blood BCAA Levels. The amount of BCAA in the blood will be supplemented with breast milk or infant formula such help. At about 9 months of age, your child may begin self-feeding the nutritionist can calculate the nutrient composition of the is an indirect measure of how many BCAAs are present in as Similac Advance EarlyShield. The nutritionist may have using a spoon. food and help you include it in the diet plan if it is not too high body tissues. Of most concern is the brain, because too much you mix the two formulas together. The Ketonex-1 mixture If your 9- to 12-month-old child has never eaten without help, in BCAAs. ILE, LEU, VAL, and their ketoacids are harmful to brain stored in bottles in the refrigerator may be warmed before dip her fingers into the food to give her the idea of finger feeding. feeding. Later, you can teach her to pick up a spoon and help guide it to • Shake the formula mixture and pour into a bottle. her mouth. Putting your child on your lap to guide her hand may Age in Months be easier. Start with thick foods, such as mashed potatoes, since • Set a bottle in a pan of cold water on the stove and they will not slip off the spoon easily. 1 2 3 4 5 6 7 8 9 10 11 12 gradually warm it or run hot tap water over the bottle. Do not worry if your child does not eat all of the foods you • Never use a microwave oven to warm formula as this can measure out; just estimate what was not eaten, replace the Ketonex-1 plus Similac Advance with Iron or breast milk result in hot spots that can burn your baby. BCAAs with another food, and write it down. Strained cereals • Always test the temperature of heated formula before When your child is older, the differences between the BCAA- Strained fruits and vegetables feeding by shaking a few drops on your wrist. The formula restricted diet and the diets of other children will be greater. Very-low-protein foods should feel lukewarm. Your child with MSUD will require Ketonex all her life to Cup feeding • If the Ketonex-1 mixture drips freely, the nipple holes are provide most of her protein, mineral, and vitamin needs. Self-feeding finger foods the correct size. Shake the bottle well before feeding. Coarsely chopped foods To feed your baby, sit in a comfortable place, hold him in Diet Guide and Food Lists. You will be given guidance at each the curve of your arm, and keep the nipple filled with the clinic visit that spells out in detail what your child can eat. The Self-feeding spoon Ketonex-1 mixture so that air will not be swallowed. You amount and how to prepare the medical food mixture, and the Raw foods 8 Figure 5. Suggested timetable for beginning solid foods and self-feeding. 9