INTRODUCTION Table 1. Preparation of I-Valex® Medical Food FOOD GUIDE FOR DLC Step Instructions This guide for Disorders of Leucine Catabolism (DLC) leucine 1 Mix a 24-hour supply of I-Valex medical formula (LEU)-restricted meal plans identifies the amount and type of all at once or in individual servings as instructed food permitted each day and can make checking and balancing by your metabolic dietitian. meals much easier. Careful eating helps control blood levels of LEU, with positive effects on health. 2 Carefully follow mixing instructions on the LEU IN THE BODY I-Valexlabel. Do not mix longer than instructed. Many foods contain LEU, one of the 20 amino acids that make 3 Refrigerate the formula after mixing. protein. When protein is digested, LEU is split off and absorbed for use in the body. 4 Use medical food within 24 hours after mixing. When LEU is unable to be split off from protein during diges- tion, the body cannot handle all the LEU coming from digested For an infant, I-Valex-1 mixture stored in bottles protein and symptoms result.. These include poor feeding, 5 in the refrigerator may be warmed before vomiting, drowsiness (lethargy), and high ammonia levels. feeding. Do not heat mixture above 100°F Depending on the type of DLC, other symptoms may also be (37.8°C) or add hot water. Heat can cause vitamin present. Limiting protein intake reduces LEU intake, so there is loss. less chance LEU will build up in the body. Normal growth and development depend on protein. However, 6 For a toddler or older child/adult, I-Valex-2 people with DLC who eat enough protein to meet their needs mixture may be consumed plain, chilled, or get too much LEU. To get enough protein and not get too much flavored. Recipes for flavoring I-Valex-2 are found LEU, a special medical food that is high in protein, free of LEU in the DLC family guide and on pages 70-71 of ® ® is necessary. I-Valex -1 and I-Valex -2 provide protein (without this guide. LEU), energy, vitamins, and minerals. Table 1 lists the steps for preparing I-Valex. I-Valex-1 and I-Valex-2 are to be used under the supervision of a doctor. 4 5