development. Because blood transports nutrients to the brain, YOUR CHILD’S GROWTH AND Toddlers. Toddlers, children from 1 to 3 years of age, have a the concentration of BCAAs in the blood will give the doctor DEVELOPMENT slow growth rate compared with that of infants. Toddlers may and nutritionist an idea of the amount of ILE, LEU, VAL, and gain 4 to 5 pounds (1.8–2.3 kilograms [kg]) a year, compared their ketoacids that might be in the brain. By 4 to 6 months of age, your baby’s birth weight will double. with the infants’ gain of 12 to 22 pounds (5.5–10 kg) per year. The child with MSUD who is kept on a controlled intake of Growth during this period involves changes in body form. Blood BCAA levels that are high may indicate that your child is BCAAs and whose diet supplies adequate nutrients should Legs lengthen and body fat decreases. Energy needs are eating more foods that are high in BCAAs than her body needs grow as well as a child without MSUD. decreased because of the slower growth rate. However, for growth. Illness, such as colds and flu, can also cause the During the second 6 months of life, the growth rate decreases. mineral and vitamin needs increase. body to break down its own protein, releasing BCAAs into the Your child may grow 1 inch (~ 2.5 centimeters [cm]) per month blood. When your child is not getting enough protein, because during the first 6 months and 4 inches (~ 10 cm) total during Toddlers seek independence and are very curious about of rapid growth or inadequate intake of the medical food, the the second 6 months of life. This normal decline in the growth their environment. Because toddlers want to do things for blood BCAA level may also rise. Initially during rapid growth, rate usually causes a decrease in appetite. themselves, encourage your child to feed himself. the blood BCAA level will decrease and then increase as the Preschoolers. Preschoolers also have a slow weight gain of body breaks down its own protein. Although the requirement for energy (calories) and protein 4 to 5 pounds (1.8–2.3 kg) per year. On the other hand, their A low BCAA level usually indicates that your child is not based on body weight decreases, the total daily requirement total energy needs are greater than those of toddlers. Because getting enough BCAAs in the diet. Sometimes to keep the for most nutrients increases with age. You will have to adjust your preschooler’s nutrient and energy needs are greater, the level of LEU low enough, ILE and VAL drop. The doctor may food choices accordingly to ensure that your child has an nutritionist may tell you to increase foods with a high nutrient prescribe ILE and VAL supplements to keep the BCAAs in adequate nutrient intake. The nutritionist will help you with content. These foods are packed with vitamins and minerals balance. This will help your child grow properly. food selections that are right for your child. and are energy dense. Clinic Visits. Because MSUD is a lifelong condition that could Weaning from Bottle to Cup. When the time comes to switch Let your preschooler make some decisions. For example, harm growth and development, you will be asked to bring from the bottle, your child may need extra attention, as any permit him to choose which cereal, fruit, or vegetable to eat. your child to the clinic frequently. If growth and development child would. Weaning takes patience, especially if your child Be aware that most preschoolers want to do things at their Some materials that you may find helpful are listed on page 17. are normal and blood BCAA concentrations remain within shows no interest in drinking from a cup or a glass. own speed. Be prepared to have your child spend so much Toddlers and Preschoolers. Permit your preschooler the treatment range, the frequency of clinic visits may be Begin offering Ketonex-1 from a cup when your child is time talking that little is eaten. This is normal behavior. to make food choices such as what fruit to eat for a fruit decreased with time. between 5 and 8 months of age. Some parents find a training Social Interaction at Mealtime. Mealtime is an important serving. Plan meals that have variety in color, texture, flavor, At clinic visits, your child may be given developmental, cup that has a lid and a spout to be very useful. part of every child’s social development and, whenever and preparation methods. A child who is involved in food physical, and neurological tests. Family interaction, which is possible, the family should eat together. Younger children can selection and preparation will be more interested in trying important to her development, may also be evaluated. The During weaning, your child may not want to take all of the learn how to feed themselves by watching older brothers and a new food. Involve your child in planning menus so he can clinic can address any questions or concerns you may have. prescribed Ketonex in liquid form. You may have to use more sisters. become familiar with foods allowed and excluded. Let him Diet changes will be made, if needed, and any questions you of it in instant puddings, cereals, fruits, and soups. Some of Make meals for your child with MSUD as similar as possible help with grocery shopping, setting the table, and preparing may have will be answered. the Ketonex can be mixed into a paste with fruits and fed by to the family’s meals. Menus for him can be planned from the food. spoon. those for the rest of the family. For example, whenever In addition to the metabolic specialist, you should have a local At about 3 to 4 years of age, children want to serve pediatrician or family doctor to provide required ongoing possible, use the same fruits and vegetables for everyone. The themselves. Teach your child the proper food portions. Your well-child care. Immunizations should be given at the usual ADDITIONAL WATER MUST BE OFFERED WHEN family’s help and support are very important to maintaining dietitian may provide you with a technique or way to count ® the child’s diet. protein that you can teach your child. times by this doctor, or you may obtain them from the health KETONEX IS FED AS A PASTE. department. Consult your child’s nutritionist. TEACHING YOUR CHILD DIET School Age. When your child reaches school age, she will MANAGEMENT become more independent in many aspects of her life and A child of 15 to 18 months may drink more medical food from a eating is one of them. As she begins to develop logic and math cup if he is given a small pitcher of Ketonex and is encouraged Explaining the diet to your child can begin by calling allowed skills, it is important she use these skills to understand the to pour it into a small cup without help. Many parents have foods “special” or “just for you.” From the time she is very diet. Encourage your child to help prepare the medical food found using brightly colored straws, special cups, or sports young, teach her to ask about unfamiliar foods before eating and calculate the amount of BCAAs in foods. As your child bottles to be good transitional tools to help wean a child from them. As your child grows older and is able to understand the gets older, she should understand what levels of BCAA are the bottle. concept of a missing or nonworking enzyme, explain MSUD. considered normal and the consequences of high BCAA levels. 10 11