When your child is older, the differences between the LEU- blood LEU and organic acid levels are well controlled. Various could harm your child’s growth and development, you will restricted diet and the diets of other children will be greater. methods are used to monitor the amino acids and organic acid be asked to take your child to the clinic frequently. If growth Your child with a DLC will require I-Valex all her life to levels in the blood and urine. Your baby’s metabolic doctor or and development are normal and laboratory concentrations provide most of her protein, mineral, and vitamin needs. nutritionist will determine how often your child is tested. remain within treatment range, the frequency of clinic visits Diet Guide and Food Lists. At each clinic visit, you will be Before taking a blood sample, you may be asked to accurately may be decreased with time. given guidance that spells out in detail what your child can eat. record your child’s total food and beverage intake. In this food At clinic visits, your child may be given developmental, The amount and how to prepare the medical food mixture, diary (notebook or form the clinic may provide), record the physical, and neurological tests. Family interaction, which is as well as the types and amounts of food that your child is name of the food, the exact amount in grams or household important to your child’s development, may also be evaluated. allowed, will be outlined. The nutritionist will help you work measures (cups, teaspoons, or tablespoons) that your child ate, Diet changes will be made, if needed, and any questions you out a plan that meets your child’s needs and fits into the family and the LEU content based on the food lists or information may have will be addressed. budget and lifestyle. given to you by the nutritionist. This will help the metabolic In addition to the metabolic specialist, you should have a local Lists of foods make meal planning easier and help you be sure doctor and nutritionist evaluate your child’s laboratory test pediatrician or family doctor to provide required ongoing your child’s nutrient needs are met. You will have time to results. well-child care. This doctor should give immunizations at become familiar with the food lists and their nutrient content, Urine Tests. A urine test may be requested by your metabolic the usual times, or you may obtain them from the health because foods will be added slowly to your child’s diet. doctor to monitor metabolic control. When a child is not department. When your child is older, you may need to use Free Foods getting enough calories, his body fat and protein may be used (see Table 1) to meet her energy needs. Free foods, which are for energy. In certain DLCs (ie, HMG-CoA lyase deficiency), YOUR CHILD’S GROWTH AND high in energy and contain little or no LEU, must not replace breakdown of body fat and protein may release ketones and DEVELOPMENT prescribed foods nor be used in large amounts. If your child organic acids, which can affect his health. Ketones may be By 4 to 6 months of age, your infant’s birth weight will double. eats too many of these free foods, she may become overweight, detected in the urine by the use of Ketostix®. Daily testing of The child with a DLC whose LEU intakes are well controlled or the extra sugar may cause tooth decay. Special low-protein the first urine in the morning will provide information useful and whose diet supplies adequate nutrients should grow as foods, including pasta, rice, crackers, cookies, and breads, can in managing your child’s diet. well as a child without a DLC. be added to the diet. These foods will help satisfy your child’s LEU and Organic Acid Levels. The amount of LEU in the During the second 6 months of life, the growth rate decreases. hunger. blood is an indirect measure of how much LEU is present in may be more pronounced in an open cup. Some parents find body tissues. Of most concern is the brain, because too much Your child may grow 1 inch (~2.5 centimeters [cm]) per month a training cup that has a lid and a spout to be very useful. If you are not able to find the LEU content of a certain food, LEU, organic acid, and ammonia, as well as too little sugar, during the first 6 months and 4 inches (~10 cm) total during Offering the I-Valex at a cold temperature may also increase ask your nutritionist. The nutritionist can calculate the are harmful to brain development. Because blood transports the second 6 months of life. This normal decline in the growth your child’s willingness to drink from a cup. nutrient composition of the food and help you include it in the nutrients to the brain, the concentration of LEU, organic acid, rate usually causes a decrease in appetite. diet plan if it is not too high in LEU. and sugar in the blood will give the doctor and nutritionist an During weaning, your child may not want to take all the idea of how much of these substances might be in the brain. Although the requirement for energy (calories) and protein prescribed I-Valex in liquid form. You may offer some of it in based on body weight decreases, the total daily requirement instant puddings, cereals, fruits, and soups, or it can be mixed Be sure to check with the nutritionist before Blood LEU levels that are high may indicate that your child is for most nutrients increases with age. You will need to adjust into a paste with fruits and fed by spoon. using any food that is not on the food lists provided. eating more foods that are high in LEU than his body needs food choices accordingly to ensure that your child has an for growth. Illness, such as colds and flu, can also cause the adequate nutrient intake. The nutritionist will help you with ADDITIONAL WATER MUST BE OFFERED WHEN body to break down its own protein, releasing LEU into the food selections that are right for your child. ® CHECKING YOUR CHILD’S PROGRESS blood. When your child is not getting enough protein, because I-VALEX IS FED AS A PASTE. of rapid growth or inadequate intake of the medical food, the Weaning from Bottle to Cup. When the time comes to Consult your child’s nutritionist. Laboratory Tests. Because your baby grows rapidly during blood LEU levels may also rise. Initially during rapid growth, switch from the bottle, your child may need extra attention, the first year of life, blood and urine samples will be tested the blood LEU level will decrease and then increase again as as any child would. Weaning takes patience, especially if your frequently to monitor how well her DLC is controlled, and the body breaks down its own protein. child shows no interest in drinking from a cup or a glass. A child of 15 to 18 months of age may drink more medical to indicate if changes in treatment are needed. Your doctor food from a cup if she is given a small pitcher of I-Valex and may ask for a blood and/or urine sample once or twice a week A low LEU level usually indicates that your child is not getting Begin offering I-Valex-1 from a cup when your child is is encouraged to pour it into a small cup without help. Many during your baby’s first 6 months, then once every 2 weeks enough LEU in the diet. between 5 and 8 months of age. Because taste buds vary on parents have found using brightly colored straws, special until 1 year of age. After 1 year of age, checking blood and urine different parts of the tongue, I-Valex may taste different when cups, or sports bottles to be good transitional tools to help levels and diet records may be decreased to once a month if Clinic Visits. Because a DLC is a lifelong condition that taken from a cup instead of a bottle. Also, the smell of I-Valex wean a child from the bottle. 10 11