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How can a disorder in the oxidation of long-chain fatty acids be managed?

There are three ways of “managing” dysfunctional fatty acid oxidation:

 

  1. Avoiding long periods of fasting 
    When long periods of fasting are avoided, the body continues to use carbohydrate reserves as the main energy source and energy production is not required to take place from the breakdown of long-chain fatty acids. Therefore, if you eat a balanced diet at regular intervals, there will always be enough carbohydrates reserves from which energy can be gained. Regular meals are are not normally a problem, as long as the child with a fatty acid oxidation disorder is healthy. The risk occurs when the child has an illness e.g. a fever inducing infection, as the bodyrequires more energy than healthy condition, especially important as some children may not want to eat or drink much or at all.
  2. Low-fat diet
    A low-fat diet is required to avoid the accumulation of long-chain fatty acids in the body.
  3. Use of medium-chain fatty acids
    Long-chain fatty acids can be replaced by medium-chain fatty acids (MCT), which are used for energy production without any subsequent fatty acid build-up. 

 

What does daily therapy look like?

The long-term therapy of disorders in the oxidation of long-chain fatty acids is the diet therapy.

This has the following objectives:

  • Normal mental and physical development
  • Age-appropriate weight gain
  • Age-appropriate growth
  • Avoidance of symptoms, e.g. muscle pain
  • Avoidance of long-term effects such as heart muscle damage
  • Avoidance of catabolic (deteriorating) metabolic situations which could lead to metabolic crises with hypoglycaemias and muscle cell degradation

To reach these objectives, the diet therapy must meet the following requirements:

  • Sufficient intake of protein, essential fatty acids, vitamins and minerals
  • Meet energy requirements 
  • Avoid long periods of fasting
  • Reduction of long chain fatty acid intake
  • Age-appropriate, i.e. melas acceptable to a child
  • Tasty dishes
  • Suitable for everyday life
     

How is the dietary therapy carried out?

The diet is based on the recommendations for healthy, age-appropriate nutrition from the German Research Institute of Child Nutrition (Forschungsinstitut für Kinderernährung, FKE). However, these recommendations must be adapted to the specified requirements of the individual therapy (see chapter “What does daily therapy look like”).

The adaptation comprises the following aspects:

  • Regular meals
  • Sufficient energy intake
  • Low-fat range of foods to reduce long-chain fatty acids in the diet
  • Use of MCT fat to cover the energy demand
  • Dose of essential fatty acids to counterbalance their deficiency in a low-fat diet
  • Adjustment of the child’s diet to the family’s diet

The therapy is individually adjusted according to the type and severity of the enzyme deficiency.
Therapies can roughly be divided into a “moderate” and a “strict” diet. But there are also variations in between based on the individual. Your metabolic team therefore gives you precise recommendations for the therapy of your child. In some children, regular meals are sufficient, whereas other patients need a very strict low-fat diet.

Note: The content of this website is for information purposes only and in no way replaces individual medical and dietary advice. 
 

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