INTRODUCTION Table 1. Preparation of Propimex® Medical Food FOOD GUIDE FOR PA Step Instructions This guide for Propionic Acidemia (PA)-restricted 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 e asier. Careful instructed by your metabolic dietitian. eating will help control blood levels of isoleucine (lLE), methio- nine (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 instructed. IN THE BODY 3 Refrigerate the formula after mixing. Many foods contain lLE, MET, THR, and VAL, 4 of the 20 amino acids that compose protein. When protein is digested, amino Use medical food within 24 hours after mixing. acids are split off and absorbed for use in the body. 4 When amino acids are unable to be split 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, PA symptoms result, such as refusal to eat, poor sucking feeding. Do not heat mixture above 100°F ability, vomiting, dehydration, lethargy (excessive tiredness), (37.7°C) or add hot water. Heat can cause vitamin seizures, and acidosis (excess acid in the blood). Limiting protein loss. intake reduces lLE, MET, THR, and VAL, so there is less chance lLE, MET, THR, and VAL will build up in the body. 6 For a toddler or older child/adult, Propimex-2 mixture may be consumed plain, chilled, or Normal growth and development depend on protein. However, flavored. Recipes for flavoring Propimex-2 are people with PA who eat enough protein to meet their needs get found in the PA family guide and on pp 70-71 of too much lLE, MET, THR, and VAL. To get enough protein and this guide. not get too much lLE, MET, THR, and VAL, a special medical food that is high in protein, free of MET and VAL, and low in ® ® lLE and THR is necessary. Propimex -1 and Propimex -2 provide protein (without MET and VAL, and with very little lLE and THR), energy, vitamins, minerals, and carnitine. 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