Low Protein Diet | Vitafriends PKU

What is PKU?

PKU is a genetic condition. In individuals with PKU, an amino acid; phenylalanine (phe), can’t be broken down in the body so it builds up to high levels in the blood. These high levels are toxic to the brain.

To prevent the levels of phe becoming too high, the diet needs to be very low in phe but enough to provide the amount required to support normal growth and development.

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What is protein?

The foods we eat contain different types of nutrients including, carbohydrate, fat, and protein.

For individuals with PKU, protein is the crucial nutrient as this contains amino acids, one of which is phe. Therefore for people with PKU the protein intake needs to be counted in measured portions and intake needs to be monitored closely.

Protein is needed daily by the body and is particularly important for infants and children that also require protein for growth.

Proteins are made up of small units or building blocks called amino acids. There are 20 different amino acids that make up the proteins of the human body, some of these must be provided by the diet as the body cannot make them. For this reason, they are called “essential” amino acids.

One of these essential amino acids is called phenylalanine (phe) and is found in nearly all types of protein.

Foods which are high in protein include meat, poultry, fish and seafood, eggs, nuts, legumes and pulses. Starchy carbohydrate-based foods, such as, bread, cereal, pasta, rice, potato, corn, biscuits, and cake contain small to moderate amounts of protein. Most fruit and vegetables are very low in protein. "Your dietitian will guide you on how much protein your chid can eat, which foods are to be encouraged freely, which foods are eaten in measured portions, and which foods are to be avoided.

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Diet for PKU

Maintaining phe levels within the normal range will be achieved by following a low protein diet. The person with PKU will also require a protein substitute and a small amount of natural protein.

The 4 main components of the low protein diet for PKU, are outlined below.

Natural Protein (grams of protein containing phe)

Those with PKU need a small amount of natural protein to provide the right amount of phe which will help support their normal growth and development.

To provide this amount of protein, a counting system for protein is used where 1g of protein provides 50mg of phe.

The number of grams of protein allowed in the diet each day will be dependent upon the person’s individual phe tolerance, size, and growth. This will be advised by the dietitian.

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Protein Substitute

Protein intake is extremely restricted in the diet for PKU, and so to meet daily protein requirements a protein substitute is essential.

The protein substitute provides all of the amino acids (except phe) and generally, carbohydrate, fat and other micronutrients, such as vitamins and minerals.

The type and amount of protein substitute will be advised by the dietitian.

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Protein-free Foods

Certain foods contain little or no phe. These can be eaten "freely" and do not need to be protein counted. Speak to your dietitian about which foods these are.

These foods are classed as protein-free and help to provide energy and variety in the diet. Protein-free foods include certain fruits and vegetables, oils, fats, and sugars.

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Specially Made Low Protein Foods

There are specially made foods that are low in protein (and therefore low in phe), these include:

Low Protein Bread and baking mixes.
Low Protein Pasta.
Low Protein Rice.
Low Protein Milk Alternatives.
Low Protein biscuits and crackers.
And more!

These foods are useful to provide calories and variety in the diet and are available for purchase from various companies.

Speak to your dietitian and refer to the PKU Handbook (ASIEM) for further information on the diet for PKU.

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