Ketonex®-2 Amino Acid-Modified Medical Food is a Table 1. General Guide to Foods on BCAA-Restricted Diets medical food used in the dietary management of children Table 2. Metric to English Conversions and adults with MSUD. This product contains no BCAAs, so Metric English Foods That Are Not Allowed Foods That Are Limited Foods That May Be Eaten Freely BCAAs must be met by using food sources. Ketonex-2 is also well supplied with fat, carbohydrate, minerals, and vitamins. Solids Dairy products (cheese, milk, ice Breast milk, infant formulas, bread, Gumdrop candy, hard candy, Your nutritionist will tell you which of the Ketonex products 1 g (0.001 kg) = 0.035 oz cream, yogurt), soy milk and soy crackers, fruit, fruit juices, low- jelly, Kool-Aid®, lemonade, is right for your child. ® 28 g = 1 oz products, beans and peas, eggs, fish protein cereals, popcorn, potato lollipops, Popsicles , pure fat, pure Ketonex-1 and Ketonex-2 taste different from milk. Most 454 g = 1 lb and seafood, meat, nuts, nut chips, special low-protein foods, sugar, soda children and adults get accustomed to the flavors of the foods butters, poultry, seeds, tofu vegetables, vegetable juices that they eat. They may seem distasteful to you, but it is very 1000 g (1 kg) = 2.2 lb important not to show this to your child, either by word or action. Liquids NUTRITION SUPPORT OF MSUD eating these foods in the amounts needed to provide just 5 mL = 1 tsp A diet that reduces BCAA intake is used to prevent the enough BCAA does not provide enough protein to meet the One mother disliked the odor of the medical food so much 15 mL = 1 Tbsp developmental delay and other problems associated with child’s needs for growth. To get enough protein for growth that she made a face every time she gave it to her son. 60 mL = 1/4 cup untreated MSUD. This diet, which is different for each and not get too much BCAA, a special medical food that is Because of this, he refused the medical food for several days 240 mL = 1 cup person with MSUD, can lower the blood BCAA to a range high in protein and free of BCAA is necessary. until she and her family realized what was wrong. She said 1000 mL (1 L) = 4 1/4 cup (1.06 qt) that permits normal mental development and growth. later, “We changed our attitude to thinking this wonderful The special MSUD diet for your child is very important. To be sure your child is getting enough energy, protein, and diet will make it possible for our child to have a happy life.” To provide enough energy and adequate BCAAs, protein, adequate BCAAs for growth and development, the nutritionist carefully calculates the amount of each nutrient needed. Too Most children taking medical foods for MSUD like them system to the English system is given in Table 2. However, minerals, and vitamins, a diet restricting BCAAs is designed little BCAAs, protein, or energy can result in growth failure. IF they are started early and IF their family has a positive the best way to be sure your child is getting the proper just for your child to maintain proper BCAA blood levels and Frequent diet adjustments are necessary, especially during attitude. amount of BCAA is to weigh food on a scale that reads in growth. the first 6 months of life when babies grow rapidly. The grams. nutritionist or metabolic doctor will make these diet changes Flavorings such as Kool-Aid® Unsweetened Soft Drink Mixes, Many foods contain protein. Those foods also contain BCAA. based on your baby’s health, growth, BCAA intakes, and blood Wyler’s® Unsweetened Soft Drink Mixes, Mio Liquid Water Formula Preparation. Mix a 24-hour supply of formula at Therefore, the amounts of foods that contain protein must be levels of BCAAs. Enhancers, and concentrated fruit juices can be added to one time, or as instructed by your nutritionist. limited in your child’s diet. Table 1 is a general guide to foods Ketonex. Remember to count the added BCAAs if they are that are not allowed, foods that are limited, and foods that Ketonex®-1 Amino Acid-Modified Infant Formula With present in any of the flavorings. Tips for preparing formula: may be eaten freely if obesity is not a problem. Iron is an infant formula used to provide protein for infants • Always follow the instructions on the label and mix and toddlers. Ketonex-1 does not contain any BCAAs. Similac® Ketonex may be made into a paste and combined with some the formula according to the recipe provided by your Additional Therapies: Your metabolic doctor may Advance® Infant Formula with Iron, breast milk, or other allowed fruits, such as applesauce or other fruit purees, or nutritionist or metabolic doctor. recommend certain vitamins, minerals, and medications for intact protein must also be fed to provide the specific amount combined with instant pudding mixes. Check the label and be your child with MSUD. Common ones include: of BCAAs a baby needs for growth and development. Breast careful which pudding mixes you buy, as some contain more • Wash your hands and all supplies carefully before • Thiamine may help improve enzyme activity. milk is lower in BCAAs than infant formula or cow’s milk protein than others. preparing formula. • L-ILE may be supplemented in pure amino acid form if and can be used to supply the required BCAAs. The decision • Mix in the approved natural protein if recommended by deficiency is noted. to breastfeed should be discussed with your nutritionist and INTERNATIONAL SYSTEM OF your nutritionist or metabolic doctor. • L-VAL may be supplemented in pure amino acid form if metabolic doctor. The nutritionist or metabolic doctor will MEASUREMENT (METRIC SYSTEM) • Do not mix longer than indicated on the Ketonex label. deficiency is noted. tell you the exact amount you will need in addition to your The metric system is the International System of Requirements for ILE, LEU, VAL, Protein, and Energy. A child’s infant formula. Ketonex-1 is well supplied with fat, Measurement. It is used for all medical and scientific • Overmixing causes the fat emulsion to break. Separation child with MSUD who eats enough protein to grow properly carbohydrate, minerals, and vitamins. Supplemental minerals measures. of the formula mixture then occurs. Overmixing may also gets too many BCAAs. Foods high in protein are cheese, milk, and vitamins are not usually needed when the diet is followed add air that destroys vitamins A and C. soy milk, eggs, meat, poultry, fish, nuts, tofu, beans and peas, as directed. In the metric system, solids are weighed in grams (g) or • Refrigerate the prepared formula after mixing. Discard seeds, and nut butters. Foods low in protein include some kilograms (kg) and liquids are measured in milliliters (mL) any unused formua 24 hours after mixing because of grains, fruits, fats, vegetables, and sweets. On the other hand, or liters (L). A list of common conversions from the metric nutrient loss. 6 7