How strict must fat restriction be in the diet?

The required fat restriction is individually determined for each patient. A mild, moderate or strict fat reduction is recommended depending on the type and form of the enzyme defect. But there are also variations in between.  

Many foods are naturally low infat. These include, apart from a few exceptions, fruit and vegetables, pulses, potatoes, cereals and drinks. As long as the fat content of these foods is not increased through processing (so-called hidden fats), these can be eaten without limitation. A good example of “hidden fats” is potatoes: potatoes themselves have no fat. They can be used to make boiled potatoes, jacket potatoes or dumplings. When prepared and eaten as fried potatoes, chips or crisps, a large amount of fat is added to them, which is absorbed by the potatoes and therefore also eaten and therefore not suitable for a fat restricted diet. Foods very rich in fat such as butter, cream, bacon, very oily or chocolate should not be eaten. In foods containing fat such as meat, fish, sausages, milk, cheese, ready-made products and sweets, the fat content is reduced in the diet by choosing suitable products. Many of these foods can be eaten in low-fat variants. For example, whole milk with 3.5 % fat can be replaced with semi skimmed milk with 1.5 % fat. By restricting the quantity of fat, the diet is also lower in calories. The deficit is met using MCT fat. Upon consultation with your treatment team, MCT fat is used in varying quantities in your daily diet. Furthermore, the intake / supplmentation of essential fatty acids must be considered.  

This therapy builds upon the moderate therapy however foods containing fat are even more restricted. Just like in the moderate therapy, all foods which are naturally low-fat can be consumed without limitation. However, the patient relies on the very low-fat variants of foods containing fat. Whole milk with 3.5 % fat is replaced by skimmed milk with 0.3 % fat. Regarding meat, fillets, tartare or skinless chicken can be eaten. Within a strict fat reduction, the use of MCT fat is a necessary complementary component of the therapy. MCT fat supplies important energy which can be used by the body without any subsequent fatty acid build-up. The precise quantities for your child are determined by the treating metabolic team. The dietitian / nutritionist will check the intake of essential fatty acids and recommend suitable products as required.

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