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The MSUD diet

Although the term ‘MSUD diet’ is used, the management of MSUD is not a one-size-fits-all approach. The MSUD diet will vary from individual to individual and changes with age. The MSUD diet will also need adjustment for various life changes/events such as growth spurts, illnesses and pregnancy. As a general rule, the more severe somebody’s MSUD is, the more restricted their protein intake will need to be.

What you need to know

Although the term ‘MSUD diet’ is used, the management of MSUD is not a one-size-fits-all approach. The MSUD diet will vary from individual to individual and changes with age. The MSUD diet will also need adjustment for various life changes/events such as growth spurts, illnesses and pregnancy. As a general rule, the more severe somebody’s MSUD is, the more restricted their protein intake will need to be.

The MSUD diet should always be carried out under the supervision of a specialist metabolic team. Supervision is required to ensure that:

  1. Leucine levels are properly controlled
  2. Valine and isoleucine are taken in the right amount
  3. Individuals with MSUD receives all the nutrients they need to grow, develop and stay healthy.

The main principles

Ideally, the diagnosis of MSUD will be made in early infancy. At this stage of life, a baby’s diet is made up entirely of milk. Babies with MSUD may be fed breast milk and breastfeeding is strongly encouraged however, the child nearly always needs to consume a special MSUD formula alongside breastmilk. Formula-fed babies will need to substitute most of their regular formula with this special MSUD formula too. The MSUD formula contains all the amino acids except for leucine, valine and isoleucine as well as vitamins, minerals and trace minerals to ensure nutritional requirements are met.

When a baby is old enough to eat solid foods, careful selection of which solid foods they should be given is required. In most cases, high protein foods need to be avoided because they are too high in leucine. These include:

  • Meat (beef, pork, lamb, goat etc)
  • Poultry (chicken and turkey)
  • Fish
  • Eggs
  • Dairy (cheese and milk)
  • Nuts and seeds
  • Pulses (beans, peas and lentils)

Protein restriction in MSUD also covers foods that many people would not think of as high protein foods. Staple foods, such as bread, rice, pasta, noodles, breakfast cereals etc. often must be excluded from the diet too or eaten in very limited quantities.

To make sure that individuals with MSUD still receive an adequate amount of protein, people with MSUD need to consume a protein substitute. Protein substitutes contain all the amino acids that the body needs, except for leucine, isoleucine and valine. Within the MSUD community, protein substitutes are sometimes referred to by other names such as ‘MSUD formula’, ‘MSUD supplement’ or ‘protein drink’.

It is also important for individuals with MSUD to consume adequate energy. Some low protein foods are naturally occurring, such as most fruits and vegetables. Specially manufactured low protein foods (e.g. low protein bread, low protein pasta etc.) are also available and can help add energy and bulk to your diet.

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