TIPS Foods NOT ALLOWED in Foods TO LIMIT in • Keep a list of your individualized goals (set by you and your ILE-, MET-, THR-, and ILE-, MET-, THR-, and health care team) on the inside cover of this guide (write in VAL-restricted meals VAL-restricted meals pencil). • Bread with added calcium • Bread • How do you know if products are ILE, MET, THR, and VAL propionate or sodium • Crackers free by reading the label? propionate • Fruit • Butter • Fruit juices — A label may state that a food contains “O” protein. • Cheese and dairy products • Low-protein cereals The zero or “O” is a rounded figure, and the product may • Chicken fat contain up to 0.49 grams of protein per serving. Check • Popcorn with your dietitian or look to see if any of the ingredients • Dried beans and peas • Potato chips may contain ILE, MET, THR, or VAL. • Eggs • Special low-protein foods • For greatest accuracy, weigh foods on a scale that reads in • Fish and other seafood • Vegetables grams (g). If a scale is not available, use approved measuring • Fish oils • Vegetable juices cups and spoons for measuring portion sizes. Table 2 lists • Lard equivalent measurements, and Table 3 provides metric conver- • Meat sion factors. • Nuts • Olive oil • Nut butters • Poultry • Seeds Odd-Chain Fatty Acids. Food fats that contain fatty acids with an uneven number of carbons are called odd—chain fatty acids. These fats produce some propionic acid when they are used in the body. Food fats that contain a small amount of odd-chain fatty acids include butter, chicken fat, cream, some fish oils, lard, and olive oil. Therefore, these foods may be restricted for your child. Avoidance of fasting is very important, as fasting leads to a buildup of odd—chain fatty acids. The higher level of a substance called “propionyl-CoA” acts as a “starter” for the production of odd-chain fatty acids. 6 7

A Food List for Propionic Acidemia (PA) - Page 4 A Food List for Propionic Acidemia (PA) Page 3 Page 5