INTRODUCTION Table 1. Preparation of Propimex® Medical Food FOOD GUIDE FOR MMA Step Instructions This guide for Methylmalonic Acidemia (MMA) meal plans 1 Mix a 24-hour supply of Propimex medical identifies the amount and type of food permitted each day and formula all at once or in individual servings as can make checking and balancing meals much easier. Careful instructed by your metabolic dietitian. eating helps control blood levels of isoleucine (ILE), methionine (MET), threonine (THR), valine (VAL), and some fats, with 2 Carefully follow mixing instructions on the positive effects on health. Propimexlabel. Do not mix longer than ILE, MET, THR, VAL, AND ODD-CHAIN FATS IN instructed. THE BODY 3 Refrigerate the formula after mixing. Many foods contain ILE, MET, THR, and VAL, four of the 20 amino acids that make protein. When protein is digested, amino 4 Use medical food within 24 hours after mixing. acids are split off and absorbed for use in the body. When amino acids are unable to be split off from protein during 5 For an infant, Propimex-1 mixture stored in digestion and some odd-chain fats are unable to be used as bottles in the refrigerator may be warmed before energy, MMA symptoms result, such as refusal to eat, poor feeding. Do not heat mixture above 100°F sucking ability, vomiting, dehydration, lethargy (excessive (37.7°C) or add hot water. Heat can cause vitamin tiredness), seizures, and metabolic acidosis (excess acid in the loss. blood). Limiting protein intake reduces ILE, MET, THR, and For a toddler or older child/adult, Propimex-2 VAL, so there is less chance their levels will build up in the body. 6 mixture may be consumed plain, chilled, or Normal growth and development depend on protein. However, flavored. Recipes for flavoring Propimex-2 are people with MMA who eat enough protein to meet their needs found in the MMA family guide and on pp 70-71 get too much ILE, MET, THR, and VAL. To get enough protein of this guide. and not get too much ILE, MET, THR, and VAL, a special medical food that is high in protein, free of MET and VAL, and ® ® low in ILE and THR is necessary. Propimex -1 and Propimex -2 provide protein (without MET and VAL, and with very little ILE and THR), energy, vitamins, and minerals. Table 1 lists the steps for preparing Propimex. Propimex-1 and Propimex-2 are to be used under the supervision of a doctor. 4 5