INTRODUCTION Table 1. Preparation of Glutarex® Medical Food FOOD GUIDE FOR GA-1 Step Instructions This guide for Glutaric Aciduria Type 1 (GA-1) and lysine 1 Mix a 24-hour supply of Glutarex all at once (LYS)- and tryptophan (TRP)-restricted meal plans identifies or in individual servings as instructed by your the amount and type of food permitted each day and can make metabolic dietitian. checking and balancing meals much easier. Careful eating helps control blood levels of LYS and TRP, with positive effects on 2 Carefully follow mixing instructions on the health. Propimexlabel. Do not mix longer than LYS AND TRP IN THE BODY instructed. Many foods contain LYS and TRP, two of the 20 amino acids that 3 Refrigerate the formula after mixing. make protein. When protein is digested, LYS and TRP are split off and absorbed for use in the body. 4 Use medical food within 24 hours after mixing. When LYS and TRP are split off from protein during digestion, For an infant, Glutarex-1 mixture stored in people with GA1 cannot handle the LYS and TRP coming 5 bottles in the refrigerator may be warmed from digested protein in a normal way and symptoms result. before feeding. Do not heat mixture above 100°F These include vomiting, metabolic acidosis (acid in the blood), (37.8°C) or add hot water. Heat can cause degenerative or permanent brain injury, and weakness. Limiting vitamin loss. protein intake reduces LYS and TRP intake so there is less chance LYS and TRP will build up in the body. 6 For a toddler or an older child/adult, Glutarex-2 Normal growth and development depend on protein. However, mixture may be consumed plain, chilled, or people with GA-1 who eat enough protein to meet their needs flavored. Recipes for flavoring Glutarex-2 are get too much LYS and TRP. To get enough protein and not get found in the GA-1 family guide and on pages 70-71 too much LYS and TRP, a special medical food that is high in of this guide. ® protein and free of LYS and TRP is necessary. Glutarex -1 and ® Glutarex -2 provide protein (without LYS and TRP), energy, vitamins, and minerals. Table 1 lists the steps for preparing Glutarex. Glutarex-1 and Glutarex-2 are to be used under the supervision of a doctor. 4 5