Nutrition

PROP Nutrition Guidelines   – The purpose of this guideline is to foster optimum nutrition management of individuals with Propionic Acidemia (also known as PROP, PA or PPA) and reduce the uncertainty and variability in the management of PA.

Metabolic Diet App –  You can track your diet through this website application on a computer or mobile device.

Low protein Diet 

One of the most common treatments in Propionic Acidemia is the restriction of natural protein or  whole intact protein in foods and formula. The specifics of the diet are followed by a dietitian or medical provider and are monitored with growth, development and labs.

Formula supplementation

Frequently, not enough protein is taken in through diet because of protein restriction for growth and development, so formulas are used to increase the amount of protein in the diet. This is done through formulas that do not have or have very little valine, isoleucine, threonine, and methionine.  The amount in the diet changes with growth, development, labs, and other factors.

There are a number of formulas that are available to drink or go through a feeding tube. In the United States, the formulas can often be obtained from a durable equipment supply company, state programs, and sometimes pharmacies.

Some of the formulas available are:

Abbott Nutrition

Prophree

Protein-free to permit protein restriction or the addition of extra energy, minerals, and vitamins. L-carnitine (25 mg/100 g) and taurine (50 mg/100 g) to help supply amounts normally found in human milk and foods of animal origin. Approximately 49% of energy as fat to help achieve acceptable formula osmolality. 7.8% of energy of linoleic acid. Nutrient profile specifically designed for infants and toddlers. May be used by children and adults. Lactose-free. Gluten-free. Halal.

To meet the nutrient needs of the infant or child, infant formula, breast milk or additional food choices must be given to supply protein requirements. These may be added to the Pro-Phree mixture or prepared separately as instructed. Depending on the specific needs of each patient, preparation will vary as prescribed by physician.

Propimex-1

Methionine- and valine-free to allow greater intake of intact protein; low in isoleucine and threonine. L-carnitine (900mg/100g) to help supply amount normally found in human milk and foods of animal origin and to help excrete toxic metabolites. Taurine (40mg/100g) to supply amount normally found in human milk and foods of animal origin.  Approximately 40% of energy as fat to help achieve acceptable formula osmolality.  6.6% of energy as linoleic acid.  Nutrient profile specifically designed for infants and toddlers.  When fed according to the Abbott Nutrition Support Protocols, provides adequate amounts of all nutrients.  Lactose-free.  Guten-free.  Halal.

Propimex-2

Methionine- and valine-free to allow greater intake of intact protein; low in isoleucine and threonine. L-carnitine (1800mg/100g) to help supply amount normally found in foods of animal origin and to help excrete toxic metabolites. Taurine (50mg/100g) to supply amount normally found in foods of animal origin.  Approximately 30% of energy as fat to help achieve acceptable formula osmolality.  Nutrient profile specifically designed for children and adults.  When fed according to the Abbott Nutrition Support Protocols, provides adequate amounts of all nutrients.  Lactose-free.  Guten-free.  Halal.

 

Cambrooke Therapeutics

Promactin AA Plus Berry

Promactin AA Plus is a methionine, threonine, and valine–free, isoleucine low, nutritionally complete, ready-to-drink, amino acid based medical food for the dietary management of Methylmalonic Acidemia (B12 non-responsive) or Propionic Acidemia.

Promactin AA Plus has a creamy, non-acid taste profile in an all-natural Berry flavor. Suitable for ages 1 through adult.

Mead Johnson

OA 1 USA

OA 1 is a medical food powder that is free of the essential amino acids isoleucine, methionine, threonine, and valine for children with propionic or methylmalonic acidemia (organic acidemia). The product provides all other essential amino acids as well as nonessential amino acids, carbohydrate, fat, essential fatty acids, vitamins and minerals. Use under direct and continuing supervision of a doctor.

OA 2 USA

OA 2 is a medical food powder that is free of the essential amino acids isoleucine, methionine, threonine, and valine for children and adults with propionic or methylmalonic acidemia (organic acidemia). The product provides all other essential amino acids as well as nonessential amino acids, carbohydrate, fat, essential fatty acids, vitamins and minerals. Use under direct and continuing supervision of a doctor.

PFD Toddler USA

PFD Toddler is a protein- and amino acid-free product designed with carbohydrates, vitamins and minerals as well as the essential fatty acids for young children with various amino acid metabolic disorders. This product is for persons requiring a protein-free diet. PFD Toddler can be used as a dietary supplement supplying calories, vitamins and minerals, or specific amounts of amino acids or protein can be added to make a complete beverage. Use under direct and continuing supervision of a doctor.

PFD 2 USA

PFD 2 is a protein- and amino acid-free product designed to help meet the calorie, vitamin, mineral and essential fatty acid needs of children and adults with amino acid metabolic disorders. PFD 2 can be mixed with condition-specific amino acids or protein to make a nutritionally complete beverage, or it can be used as a calorie, vitamin and mineral supplement when amino acid and protein requirements are met but needs for calories, vitamins and minerals are not. Use under direct and continuing supervision of a doctor.

 

Nutricia

MMA/PA Anamix Early Years

A methionine-, threonine-, valine-free, and isoleucine-low powdered infant formula with iron, for the dietary management of Vitamin B12 non-responsive Methylmalonic Acidemia or Propionic Acidemia. Contains docosahexaenoic acid (DHA) and arachidonic acid (ARA) and a unique blend of prebiotic fiber.

MMA/PA Anamix Next

A methionine-, threonine-, valine-free, low isoleucine mildly flavored powdered drink mix containing a balanced mix of the other essential and non-essential amino acids, carbohydrates (including a fiber mix), fat (including docosahexaenoic acid (DHA)), vitamins, minerals, and trace elements. To be used for individuals ages 1+.

Vitaflo USA

MMA/PA gel

MMA/PA gel is an unflavored, powdered methionine, threonine and valine free and low isoleucine protein substitute, containing essential and non-essential amino acids, carbohydrate, vitamins, minerals and trace elements.

MMA/PA cooler15

MMA/PA cooler15 is a ready-to-drink, methionine, threonine, valine free and low isoleucine protein substitute, containing essential and non-essential amino acids, carbohydrate, vitamins, minerals, trace elements and the Omega-3 long chain polyunsaturated fatty acids (LCPs); Docosahexaenoic acid (DHA) and Eicosapentaenoic acid (EPA). Available in a red flavor option.

MMA/PA express15

MMA/PA express15 is an unflavored, powdered methionine, threonine, valine free and low isoleucine protein substitute, containing essential and non-essential amino acids, carbohydrate, vitamins, minerals and trace elements.

Amino Acid Supplementation

Some may have their diet supplemented with one or more of the offending amino acids: valine, isoleucine, methionine, and threonine.

 

Disclaimer:

The Propionic Acidemia Foundation website is designed for educational purposes only and is not intended to serve as medical advice. The information provided on this site should not be used for diagnosing or treating a health problem or disease.  It is not a substitute for professional care.  If you suspect that you or your children may have Propionic Acidemia, you should consult your health care provider.

The Propionic Acidemia Foundation does not recommend nor endorse any particular products, companies, or manufacturers.