The ‘building blocks’ of protein are called amino acids. In MSUD, due to the lack of an enzyme, the body cannot properly deal with three amino acids, leucine, isoleucine and valine – collectively known as the branched chain amino acids (BCAAs). People with MSUD therefore need to severely restrict the amount of protein they eat.
A key element in the management of MSUD is the use of protein substitutes. Protein substitutes for MSUD provide all the amino acids found in protein but no leucine, isoleucine or valine. In combination with the small allowance of regular dietary protein, protein substitutes ensure that an individual with MSUD has an adequate intake of protein.
MSUD gel™
MSUD gel™ A food for special medical purposes.
MSUD gel™ is an unflavoured, powdered leucine, isoleucine and valine free protein substitute containing essential and non essential amino acids, carbohydrate, vitamins, minerals and trace elements. MSUD gel is easily made up to a smooth semi‐solid consistency. Suitable from 6 months of age, this is a simple choice for weaning onto a second stage protein substitute
MSUD gel Datacard
MSUD amino5™
MSUD amino5™ A powdered amino acid supplement for use in the dietary management of Maple Syrup Urine Disease (MSUD). MSUD amino5 can be used as the protein component of a bespoke modular feed or in acute emergency management, if required. MSUD amino5 is suitable from 3 years of age.
MSUD amino5 is a food for special medical purposes and must be used under medical supervision.
MSUD amino5 Datacard
MSUD explore5™
MSUD explore5™ A powdered protein substitute for the dietary management of Maple Syrup Urine Disease (MSUD).
MSUD explore5 is a second stage protein substitute easily made up to a smooth spoonable consistency. Suitable from 6 months of age, this is a simple choice when introducing solids.
MSUD explore5 is a food for special medical purposes and must be used under medical supervision
MSUD explore5 Datacard
MSUD cooler®
MSUD cooler® A food for special medical purposes.
MSUD cooler is a ready-to-drink, leucine, isoleucine and valine free protein substitute, containing essential and non-essential amino acids, carbohydrate, vitamins, minerals, trace elements and the omega-3 long chain polyunsaturated fatty acids (LCPs); docosa-hexaenoic acid (DHA) and eicosapentaenoic (EPA). With sugar and sweeteners. Available in a choice of flavours.
MSUD cooler is suitable from 3 years of age.
MSUD cooler Datacard
MSUD express™
MSUD express™ A food for special medical purposes.
MSUD express is a range of unflavoured, powdered leucine, isoleucine and valine free protein substitute, containing essential and non essential amino acids, carbohydrate, sugar, vitamins, minerals and trace elements.
MSUD express can be easily made to an individual’s preferred volume and is suitable from 3 years of age.
MSUD express Datacard
MSUD express™ plus
MSUD express™ plus MSUD express™ plus is an unflavoured powdered leucine, isoleucine and valine free*, protein substitute containing essential and non-essential amino acids, carbohydrate, vitamins, minerals, trace elements and docosahexaenoic acid (DHA).
Express paste – a few spoonfuls and it’s gone. Ideal for patients transitioning from gel.
Express mini drink – Low in volume. Quick to take in one go.
Express flexi drink – make up to individual’s own taste/volume. Ideal for those transitioning from a traditional protein substitute who want to make up to their own volume.
MSUD express plus is a food for special medical purposes and must be used under medical supervision.
MSUD express plus Datacard
