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 Formulary Chapter 9: Nutrition and blood - Full Chapter
Notes:

 Principles of using Oral Nutritional Supplements (ONS)

 

FIRST LINE Option

ONS Option

Food First

[Dietary measures (food fortification, increased frequency, nutritious snacks and drinks - using everyday ordinary foods)]

 Please note:- hygiene considerations/infection control

Except where patient has special dietary

requirements/ restrictions

·         Supplements should be given in-between meals and snacks NOT INSTEAD OF MEALS.

 

·         Most supplements contain milk – in diagnosed /milk intolerance – refer to dietitian

 

 

 

Homemade milkshake

 

Exceptions:-

·         Lactose intolerance

·         Milk allergy or intolerance

·         CKD 4 and 5

·         Tube feed patients (not licensed)

·         Cannot be prepared (dexterity issues, lack of carer support, hygiene considerations/infection control)

·         Liver/hepatobiliary and pancreatic disease/conditions.

 

 

Preferred option: Powdered Feeds

Alternative option, where powdered feed is unsuitable:

Ready-made/ready to drink feeds (e.g. bottled)

Make up with whole (full fat) milk

Exceptions:

·         Lactose intolerance

·          Milk allergy or intolerance

·         CKD 4 and 5

·         Tube feed patients (not licensed)

·         Cannot be prepared (dexterity issues, lack of carer support, hygiene considerations/infection control)

Note – can be made in a smaller volume, on the advice of a dietitian, for volume intolerance.

·         Unable to tolerate large amounts of lactose

·         Cannot be prepared (dexterity issues, lack of carer support, hygiene considerations/infection control - while in acute setting)

Dietary advice sheets and guidance available from local nutrition and dietetic services.

Please note- Not all patients will be able to meet their specific nutritional requirements with food first measures alone and supplements are required for some conditions e.g. very high protein requirement in liver transplantation, sarcopenia. The supplements will be recommended by the specialist dietitian.

Review criteria

·         All patients on ONS should be monitored and for adult patients an appropriate nutritional screening tools should be used (e.g. MUST, Birmingham learning disability screening tool).

·         Review the MUST score at least every 3 months or as advised by specialist dietitian

·         Patients on active dietitian case load will be monitored upto the point of discharge.

·         If no dietetic input, other healthcare professionals should monitor ONS for its effectiveness.

·         Please contact your local dietetic service for advice, guidance or referral criteria.

 

 

 

 

 Details...
09.04  Expand sub section  Oral nutrition
Enshake®
(Powder)
View adult BNF View SPC online View childrens BNF
First Choice
Amber
  • Protein, fat, and carbohydrate
  • First choice for patients with very high energy requirement eg cystic fibrosis
  • In adults with cystic fibrosis, nutritional advice and interventions should be aimed at maintaining a BMI of 22kg/m2 in women and 23kg/m2 in men, as these are associated with better lung function (Nutritional management of CF – Consensus document – 2016)
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement. Not suitable for child under 1 year; use with caution in child 1–6 years.
 
Food First
(Dietary advice sheets and guidance available from local nutrition and dietetic services))
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First Choice
Green

Where clinically appropriate encourage a nutrient dense food and fluid intake for patients at risk of malnutrition.

 
Foodlink® Complete
(Powder)
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First Choice
Green
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • First line product
  • Not suitable for CKD 4-5, renal pts.
  • Not suitable for lactose intolerance
  • Not suitable for bile acid malabsorption or fat maldigestion associated with biliary diseases/conditions
  • To be made up with whole milk.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
Foodlink® Complete with Fibre
(Powder)
View adult BNF View SPC online View childrens BNF
First Choice
Green
  • First line with added fibre
  • Not suitable for CKD 4-5, renal pts.
  • Not suitable for lactose intolerance
  • Not suitable for bile acid malabsorption or fat maldigestion associated with biliary conditions
  • To be made up with whole milk.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
Homemade milkshake
(Where clinically appropriate encourage a nutrient dense food and fluid intake for patients at risk of malnutrition.)
View adult BNF View SPC online View childrens BNF
First Choice
Green

Where clinically appropriate encourage a standardised home-made nutritious
drink at least equivalent to a standard 1.5KCal/ml prescribed bottle supplement
in terms of calories and protein

 
Scandishake® Mix
(Powder)
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Second Choice
Amber
  • Protein, fat, and carbohydrate
  • Second choice for patients with very high energy requirement eg cystic fibrosis
  • In adults with cystic fibrosis, nutritional advice and interventions should be aimed at maintaining a BMI of 22kg/m2 in women and 23kg/m2 in men, as these are associated with better lung function (Nutritional management of CF – Consensus document – 2016)
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement. Not suitable for child under 3 years.
 
   
Altraplen Compact® (formally known as Nutriplen®)
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Alternative to second line supplement choice
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–6 years.
 
   
Aptamil Pepti® 1
(Powder)
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Formulary
Amber
  • Infant and child: Hydrolysate formula
  • Whey hydrolysate formula containing lactose. Use in infants (from birth – 6 mths) who have cow’s milk protein intolerance / allergy but no lactose intolerance. Tube or oral feed.
  • Established cows' milk protein intolerance, with or without secondary lactose intolerance
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Aptamil Pepti® 2
(Powder)
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Formulary
Amber
  • Infant and child: Hydrolysate formula
  • Whey hydrolysate containing lactose. Use in infants (from 6 mths) who have cow’s milk protein intolerance / allergy but no lactose intolerance. Tube or oral feed
  • Established cows' milk protein allergy or intolerance. Not suitable for child under 6 months.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Aptamil Pepti® Junior
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Formulary
Amber
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Atraplen® Protein (formally known as Nutriplen® Protein)
(Liquid (sip feed))
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Formulary
Amber
  • Used in cases where increased protein is required
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula.
  • Not suitable for child under 3 years; use with caution in child 3–6 years
 
   
Aymes® Complete
(Liquid (sip feed))
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Formulary
Green
  • 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Second line choice for 200ml ready-made. If powder is not tolerated or contraindicated
 
   
Aymes® Creme
(Semi-solid)
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Formulary
Amber
  • More than 1.5 kcal/ml and 5g (or more) protein/100ml
  • Low energy per serving as a semi-solid supplement where low volume is required only.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, preoperative
    preparation of malnourished patients, dysphagia, proven inflammatory bowel
    disease, following total gastrectomy, short-bowel syndrome, bowel fistula Not suitable for child
    under 3 years; use with caution in child 3–6 years.
 
   
Aymes® Shake/Savoury
(Powder)
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Formulary
Green
  • In line with food first
  • Not suitable for CKD 4-5, renal pts.
  • Not suitable for lactose intolerance
  • Not suitable for bile acid malabsorption or fat maldigestion associated with biliary diseases/conditions
  • To be made up with whole milk.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula . Use with caution in child 1–6 years.
 
   
Basecal®
(Powder)
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Formulary
Amber
  • Fat and Carbohydrate
  • Over one year of age. Metabolic disorder pts
  • Also these are the only protein free (containing fat, carbohydrate, vitamins and minerals) powders aimed at children ≥1-year-old and adults with inborn errors of protein metabolism (particularly patients with urea cycle disorders and organic acidaemias). Basecal 100 and Basecal 200 are commonly added as an energy source in low protein module tube feeds. The dose will depend on energy requirements and tolerance to natural protein. It is commonly used in combination with a controlled amount of natural protein e.g. measured dose of standard enteral tube feed. It will help meet essential energy and nutrient requirements. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
 
   
Betaquick®
(Liquid)
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Formulary
Amber
  • High-energy supplements: fat
  • 21% MCT emulsion, used orally and in cooking as an energy source for children and adults on a ketogenic diet
 
   
Calogen®
(Liquid (emulsion))
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Formulary
Amber
  • High-energy supplements: fat
  • Used as a source of LCT, as an energy supplement and additive to feed to enhance energy density.
  • Does not contain protein
  • Also this is the only LCT fat source that can be used as a fat energy source in patients with organic acidaemias e.g. propionic acidaemia and methyl malonic acidaemia and urea cycle disorders. It may also be used as part of a modular feed or protein-free energy source. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat (or fat and carbohydrate) supplement. Liquid supplements
 
   
Calogen® Extra
(Liquid)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Only to be used on dietetic advice for oral and tube feeds
  • Used where energy, protein and micronutrients required.
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement. Not suitable for child under 3 years; use with caution in child 3–6 years. May require dilution for child 3–5 years.
 
   
Calogen® Extra Shots
(Liquid)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Used on dietetic advice only
  • Used where hygiene concerns/infection control – single use sterile dose – where Calogen Extra bottle is not appropriate
  • Used where energy, protein and micronutrients required in small volume
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement.
  • Not suitable for child under 3 years; use with caution in child 3–6 years. May require dilution for child 3–5 years.
 
   
Carbzero
(Liquid)
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Formulary
Amber
  • High-energy supplements: fat
  • 20% LCT emulsion, used as an energy source for children and adults on a ketogenic diet, may be used orally or in cooking
  • For use in ketogenic diets. Long chain triglyceride liquid emulsion used as a milk replacement (when diluted) for some patients with PKU on very low protein diets who cannot tolerate other low protein milk replacements. It will help meet essential energy requirements. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
 
   
Cow & Gate Pepti-Junior®
(Powder)
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Formulary
Amber
  • Infant and child: Hydrolysate formula
  • Whey hydrolysate. Use in infants who have malabsorption problems and require a peptide and MCT containing feed. Eg after gut surgery. Use as tube or oral feed
  • Disaccharide and/or whole protein intolerance, or where amino acids and peptides are indicated in conjunction with medium chain triglycerides.
 
   
Dialamine®
(Powder)
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Formulary
Amber
  • Protein and carbohydrate
  • This is a source of essential amino acids for use in low protein diets with urea cycle disorders. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Hypoproteinaemia, chronic renal failure, wound fistula leakage with excessive protein loss, conditions requiring a controlled nitrogen intake, and haemodialysis. Not suitable for child under 6 months.
 
   
Duocal® Super Soluble
(Powder)
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Formulary
Amber
  • Fat and Carbohydrate
  • For renal children with fluid restriction
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat (or fat and carbohydrate) supplement.
 
   
Energivit®
(Powder)
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Formulary
Amber
  • Fat and Carbohydrate
  • Infant feed - Metabolic disorder pts
  • Also protein-free (containing fat, carbohydrate, vitamins and minerals) powder necessary for infants with inborn errors of protein metabolism. It is the only protein-free infant feed for patients with urea cycle disorders and organic acidaemias. It is commonly used in combination with a measured amount of natural protein e.g. expressed breast milk. It will help meet essential energy and nutrient requirements. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • For children requiring additional energy, vitamins, minerals, and trace elements following a protein-restricted diet
 
   
FlavourPac®
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Formulary
Amber
  • Flavouring preparations
  • For flavouring unpalatable flavouring amino acid supplements or feeds in inborn errors of metabolism
  • Essential for flavouring different unflavoured amino acid supplements in inborn errors of metabolism particularly for non PKU conditions. They offer common children’s favourite flavours: e.g. orange or raspberry. Flavourings are important, as amino acid formulations are unpalatable and unacceptable particularly for patients with late diagnosed conditions. They cannot be purchased from a supermarket.
  • For use with Vitaflo's range of unflavoured protein substitutes for metabolic diseases; not suitable for child under 3 years.
 
   
Forceval caps and soluble tabs
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Formulary
Amber
  • Special additives for conditions of intolerance
  • Vitamin and mineral deficiency and as adjunct in synthetic diets
 
   
Fortijuce®
(Liquid (sip feed))
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Formulary
Amber
  • More than 1 kcal/mL and less than 5 g protein/100 mL
  • Juice style fat free, low K+
  • Patients who dislike milky tasting drinks
  • Caution advised – not milk free and high sugar content
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula Not suitable for child under 3 years
 
   
Fortini®
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1 kcal/ml and less than 4g protein/100ml
  • Sip feed for children over 8kg with faltering growth
  • Disease-related malnutrition and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Fortini® Compact Multifibre
(Liquid)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Child: More than 1 kcal/mL and less than 4 g protein/100 mL
  • Low volume supplement
  • Supporting an unmet paediatric nutritional need
  • No other product is current available to meet this need.
 
   
Fortini® Creamy Fruit
(Semi-solid)
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Formulary
Amber
  • Child: More than 1 kcal/mL and less than 4 g protein/100 mL
  • High energy fruit pudding for children with faltering growth
 
   
Fortini® Multifibre
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Child: More than 1 kcal/mL and less than 4 g protein/100 mL
  • Sip feed for children over 8kg with faltering growth, with added fibre
  • Disease-related malnutrition and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Fortini® Smoothie Multifibre
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Child: More than 1 kcal/mL and less than 4 g protein/100 mL
  • Sip feed for children over 8kg with faltering growth
 
   
Fortisip® 2kcal
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • For bolus tube feeding only
  • Only on dietetic advice sip feed in small volume.
  • Nutritionally complete in 2 bottles.
 
   
Fortisip® Bottle
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Green
  • Restricted for use where fluid volume is a benefit
  • Restricted for patients who are lactose intolerant
  • Maybe used as an alternative as a bolus feeds
  • Low fibre
  • Low osmolality.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
  • Second line choice for 200ml ready-made. If powder is not tolerated or contraindicated
 
   
Fortisip® Compact
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Second line supplements choice.
  • Low volume supplement and for bolus feeding
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
 
   
Fortisip® Compact Fibre
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Second line supplements choice for use when fibre is indicated as advised by dietitian only. Low volume supplement and for bolus feeding
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
 
   
Fortisip® Compact Protein
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Not to be used without dietetic advice that higher protein is clinically appropriate.
  • With added protein
  • Low volume supplement and for bolus feeding advised by dietitian only.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula.
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
 
   
Fortisip® Yogurt Style
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • For dysgeusia and flavour fatigue only
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula. Not suitable for child under 3 years
 
   
Fresubin® 2kcal
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • First line choice for 2kcal/ml sip feed
  • Small volume required and based on taste preference
  • Nutritionally complete in 2 bottles.
  • High Cal low Vol
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula; also CAPD, haemodialysis.
  • Not suitable for use in child under 1 year; use with caution in child 1–5 years.
 
   
Fresubin® 2kcal Fibre Drink
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Used where 2kcal/ml sip feed in small volume with fibre is required
  • High Cal low Vol with added fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula ; also CAPD, haemodialysis.Not suitable for use in child under 1 year; use with caution in child 1–5 years.
 
   
Fresubin® Juicy Drink
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • More than 1 kcal/mL and less than 5 g protein/100 mL
  • Juice style fat free, low K+
  • Patients who dislike milky tasting drinks 
  • Caution advised – not milk free and high sugar content
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula ; also CAPD, haemodialysis

 

 
   
Fresubin® Thickened
(Liquid (sip feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Used for dysphagia pts only
  • Dysphagia or disease-related malnutrition. Not suitable for child under 3 years; use with caution in child 3–4 years.
 
   
Fresubin® YOcreme
(Semi-solid)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • For dysgeusia and flavour fatigue only
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, preoperative
    preparation of malnourished patients, dysphagia, proven inflammatory bowel
    disease, following total gastrectomy, short-bowel syndrome, bowel fistula. Not suitable
    for child under 3 years
 
   
Fructose
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Special additives for conditions of intolerance
  • Used as a partial CHO source in modular feeds for infants and children with malabsorption secondary to short bowel syndrome or other malabsorption states
  • (Laevulose) For proven glucose/galactose intolerance
 
   
Galactomin 17®
(Powder)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Specialised formulas: Infant and child: Residual lactose formula
  • Lactose free feed for use in adults metabolic disease only
  • Used as a milk alternative in adults, lactose free with galactosaemia. This is not used for infants by Birmingham Children’s Hospital. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Proven lactose intolerance in pre-school children, galactosaemia, and galactokinase deficiency.
 
   
Galactomin 19®
(Powder)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Specialised formulas: Infant and child: Fructose-based formula
  • Disease specific fructose containing formula for infants and children with glucose galactose malabsorption.
  • Conditions of glucose plus galactose intolerance
 
   
Glucose
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Special additives for conditions of intolerance
  • Used as a partial CHO source in modular feeds for infants and children with malabsorption secondary to short bowel syndrome or other malabsorption states
  • (Dextrose monohydrate) For use as an energy supplement in sucrose-isomaltase deficiency
 
   
Hydrolysed whey protein maltodextrin powder
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • High-energy supplements: protein
  • Hydrolysed protein powder to be used in a modular feed.
 
   
Infatrini®
(Liquid (sip or tube feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • 1 kcal infant feed for faltering growth
  • Varying volume size available to support nutritional requirements of the patients.
  • Single use only in acute setting – risk of increased waste. Once open must be used within 24 hours.
  • Failure to thrive, disease-related malnutrition and malabsorption, in child from birth up to body-weight 8 kg. Up to 18 months.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Infatrini® Peptisorb
(Liquid (sip or tube feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Infant and child: Hydrolysate formula
  • Whey hydrolysate. Use in infants (birth – 18 mths) who are unable to tolerate Infatrini (eg gut intolerance) and also require a higher calorie feed for faltering growth. Use as tube or oral feed
  • Disease-related malnutrition, intractable malabsorption, proven inflammatory bowel disease, short bowel syndrome, bowel fistula, and intolerance to whole protein feeds
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
KetoCal® 2.5:1 (Liquid )
View adult BNF View SPC online View childrens BNF
Formulary
Amber
 
   
KetoCal® 3:1
(Powder)
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • High fat, low carbohydrate, nutritionally complete formula for infants and children from birth to 6 yrs, or as a supplementary feed for children over 6 years and adults on a ketogenic diet. May also be used in cooking
  • Enteral feed or nutritional supplement as part of ketogenic diet in management of drug resistant epilepsy or other conditions for which a ketogenic diet is indicated in children from birth to 6 years; as a nutritional supplement in children over 6 years.
 
   
KetoCal® 4.1
(Powder)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • High fat, low carbohydrate powdered formula for children on a ketogenic diet as oral or tube feed, or used in cooking or as a supplementary feed for adults on a ketogenic diet
  • Enteral feed or nutritional supplement as part of ketogenic diet in management of epilepsy resistant to drug therapy, in children over 1 year, only on the advice of secondary care physician with experience of ketogenic diet.
 
   
KetoCal® 4:1 LQ
(Liquid (sip or tube feed))
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • High fat, low carbohydrate nutritionally complete liquid for children over 1 year of age and adults on a ketogenic diet, suitable for oral or tube feeding
  • Enteral feed or nutritional supplement as part of ketogenic diet in management of drug resistant epilepsy or other conditions for which a ketogenic diet is indicated in children 1–10 years; as a nutritional supplement in children over 10 years.
 
   
Lipistart®
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: MCT-enhanced formula
  • Essential for infants/children with long chain fatty acid oxidation disorders who need a high intake of MCT but require normal proportions of carbohydrate e.g. CPT1 and CPT2 deficiency. High carbohydrate formulas are unnecessary.
  • Also essential for infants/children with long chain fatty acid oxidation disorders who need a high intake of MCT but require normal proportions of carbohydrate e.g. CPT1 deficiency (i.e. high carbohydrate formulas may be unnecessary). There should always be 2 powdered high MCT formulae in the formulary in case of supply issues. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Dietary management of fat malabsorption, long-chain fatty acid oxidation disorders, and other disorders requiring a high MCT, low LCT formula.
 
   
Liquigen®
(Liquid (emulsion))
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Formulary
Amber
  • High-energy supplements: fat
  • Used as a source of MCT when required for children and adults with LCT intolerance and on a low LCT diet and for adults and children on a ketogenic diet - ££
  • Also this is used as a milk replacement and as part of module tube feeds for patients with long chain fatty acid disorders such as LCHADD and VLCADD who require ≥10% fat calories from long chain fat and a diet supplemented with MCT. It is essential to provide essential energy on fat restricted diets if MCT is tolerated by the patient. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Steatorrhoea associated with cystic fibrosis of the pancreas, intestinal lymphangiectasia, intestinal surgery, chronic liver disease, liver cirrhosis, other proven malabsorption syndromes, ketogenic diet in epilepsy, and in type 1 lipoproteinaemia. Not suitable for child under 1 year
 
   
Locasol ®
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: Low calcium formula
  • Use in children with high blood calcium levels eg Noonan’s syndrome
  • Conditions of calcium intolerance requiring restriction of calcium and vitamin D intake
 
   
Loprofin SnoPro
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • Low protein, low potassium, low Phosphate milk substitute , used as a milk replacement in children with renal disease and IMD
  • Nutritional supplement for the dietary management of phenylketonuria, chronic renal failure and other inborn errors of amino acid metabolism.
 
   
Low fat 1+ module
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: MCT-enhanced formula
  • Children and adults with a fatty acid disorder
  • Also fat free preparation. Used by patients with long chain fatty acid oxidation disorders who cannot tolerate MCT and require a very low-fat diet. Suitable for children and adults with a fatty acid disorder. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
 
   
Maxijul® Super Soluble
(Powder)
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Formulary
Amber
  • High-energy supplements: carbohydrate
  • High calorie modular powder, to be added to other food, drink and feeds e.g. faltering growth, to increase carbohydrate content.
  • Also used as an additional energy supplement when IMD patients require a modified, low protein or low-fat feed or are on a glucose polymer based energy supplement and require other Nutricia products such as Monogen or Nutrini fibre, so it can be delivered using the same home delivery service so less burdensome for families. It is also used as a core ingredient for glucose based emergency feeds. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high or readily available carbohydrate supplement.
 
   
MCT Procal®
(Powder)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Used in children and adults on a ketogenic diet as an energy source for use with long chain fatty acid disorders and in metabolic disorders.
  • Used in liver/hepatobiliary disease/conditions
  • Essential for use with log chain fatty acid disorders such as LCHADD, VLCADD, CACT. It provides a pre-measured source of MCT energy at breakfast, midday and evening to ensure patients do not become energy depleted when performing usual daytime activities. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Dietary management of disorders of long-chain fatty acid oxidation, fat malabsorption, and other disorders requiring a low LCT, high MCT supplement.
  • Not suitable for child under 1 year.
 
   
Medium-chain Triglyceride (MCT) Oil
(Liquid)
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Formulary
Amber
  • High-energy supplements: fat
  • Used as a source of energy for children and adults requiring a low LCT/ High MCT diet
  • Also this is used as a MCT cooking oil to improve diet palatability in patients with long chain fatty acid disorders such as LCHADD and VLCADD who require ≥10% fat calories from long chain fat. It is used to make MCT margarine, add to toast or to cook potatoes and other vegetables. It is essential to provide essential energy on fat restricted diets if MCT is tolerated by the patient. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Nutritional supplement for steatorrhoea associated with cystic fibrosis of the pancreas, intestinal lymphangiectasia, intestinal surgery, chronic liver disease and liver cirrhosis, other proven malabsorption syndromes, ketogenic diet in management of epilepsy, type 1 hyperlipoproteinaemia
 
   
Modjul® Flavour System
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Formulary
Amber
  • Flavouring preparations
  • For flavouring unpalatable flavouring amino acid supplements or feeds in inborn errors of metabolism
  • Essential for flavouring different unflavoured amino acid supplements in inborn errors of metabolism- particularly for non PKU conditions. They offer alternative flavours to Vitaflo pacs. This is important as amino acid formulations are unpalatable and unacceptable particularly for patients with late diagnosed conditions. They cannot be purchased from a supermarket.
  • For use with unflavoured SHS products based on peptides or amino acids; not suitable for child under 6 months.
 
   
Modulen IBD®
(Powder)
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • For treatment of Crohn’s Disease
  • Crohn's disease active phase, and in remission if malnourished
 
   
Monogen®
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: MCT-enhanced formula
  • Used in infants who require high MCT feed eg following chylothorax and in inborn errors of metabolism where MCT is required
  • Also essential for infants/children with long chain fatty acid oxidation disorders who need a high intake of MCT and high intake of carbohydrate e.g. LCHADD and trifunctional protein deficiency (i.e. lower carbohydrate formulas are inappropriate). There should always be 2 powdered high MCT formulae in the formulary in case of supply issues. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Long-chain acyl-CoA dehydrogenase deficiency (LCAD), carnitine palmitoyl transferase deficiency (CPTD), primary and secondary lipoprotein lipase deficiency, chylothorax, and lymphangiectasia
 
   
Neocate® Junior
(Powder)
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Formulary
Amber
  • Infant and child: Amino acid-based formula
  • Replacement for Neocate Active and Neocate Advance on the formulary.
  • Use in children over 12 mths who require amino acid based feed / have multiple food allergies.
  • Used as tube or oral feed, provides complete nutritional support
  • For the dietary management of proven whole protein allergy, short bowel syndrome, intractable malabsorption and other gastrointestinal disorders where an amino acid diet is recommended, for children from 1 year onwards
  • ACBS Indications: Proven whole protein intolerance, short bowel syndrome, intractable malabsorption, or other gastro-intestinal disorders where an elemental diet is indicated
 
   
Neocate® LCP
(Powder)
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Formulary
Amber
  • Infant and child: Amino acid-based formula
  • Use in infants ( birth – 12 months) who have multiple food allergies / faltering growth / require amino acid based feed as unable to tolerate standard or peptide feed. Used as tube and oral feed.
  • Cows' milk allergy, multiple food protein intolerance, and conditions requiring an elemental diet
  • ACBS Indications: Proven whole protein intolerance, short bowel syndrome, intractable malabsorption, or other gastro-intestinal disorders where an elemental diet is indicated
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Neocate® Syneo
(Powder)
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Formulary
Amber
  • Infant and child: Amino acid-based formula - ACBS Indications:Proven whole protein intolerance, short bowel syndrome, intractable malabsorption, or other gastro-intestinal disorders where an elemental diet is indicated
  • Contains Bifidobacterium breve M-16V to combat gut flora dysbiosis, shown by infants with allergies.
  • Use in infants ( birth – 12 months) who have multiple food allergies / faltering growth / require amino acid based feed as unable to tolerate standard or peptide feed. Used as tube and oral feed.
  • Not suitable for premature infants or infants who maybe immunocompromised. Not for use in postpyloric tube feeding.
    Not recommended for infants with central venous catheter or SHORT BOWEL SYNDROME without full consideration of risks/benefits and monitoring by/on advice of a specialist.
  • Cows' milk allergy, multiple food protein intolerance, and conditions requiring an elemental diet
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Nepro®
(Liquid (sip or tube feed))
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • Used for renal in and outpatients – adults only Useful on occasion due to reduced Vitamin A content – adults only
  • Enteral feed or nutritional supplement in patients with chronic renal failure who are on haemodialysis or CAPD, or with cirrhosis, or other conditions requiring a high energy, low fluid, low electrolyte diet.Not suitable for child under 1 year; use with caution in child 1–5 years.
 
   
Nutilis® Complete Stage 1
(Liquid (pre-thickened))
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Formulary
Amber
  • Used for dysphagia pts only
  • Where first line pre-thickened product – Slo Milkshake – is not suitable
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
 
   
Nutramigen® 1 with LGG
(Powder)
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Formulary
Green
  • Specialised formulas: Infant and child: Hydrolysate formula
  • Casein hydrolysate. Use in infants (from birth – 6 mths) who have cow’s milk protein intolerance / allergy. Tube or oral feed.
  • Nutramigen has all the fat as LCT and CHO as glucose polymer and is more suitable for CMPI
  • If patient has a cow’s milk protein intolerance / allergy GP may initiate Nutramigen® Lipil 1 and refer to dietetics – in line NICE CG 116
  • Disaccharide and/or whole protein intolerance where additional medium chain triglycerides are not included.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Nutramigen® 2 with LGG
(Powder)
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Formulary
Green
  • Specialised formulas: Infant and child: Hydrolysate formula
  • Casein hydrolysate. Use in infants from 6 mths who have cow’s milk protein intolerance/ allergy and soya intolerance/allergy. Tube or oral feed. If patient has a cow’s milk protein intolerance / allergy GP may initiate Nutramigen® Lipil 2 and refer to dietetics – in line NICE CG 116
  • Established disaccharide and/or whole protein intolerance (where additional chain triglycerides are not indicated) Not suitable for child under 6 months.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Nutramigen® Puramino
(Powder)
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Formulary
Amber
  • Infant and child: Amino acid-based formula
  • Use in infants (birth – 12 months) who have multiple food allergies / faltering growth / require amino acid based feed. Unlike other products contains 33% MCT fat so useful in children requiring amino acid feed who also have fat malabsorption. Used as tube and oral feed.
  • For use in the management of severe protein intolerance, multiple food intolerance and other gastro-intestinal disorders where an amino acid based diet is specifically indicated for infants and young children.
  • ACBS Indications: Proven whole protein intolerance, short bowel syndrome, intractable malabsorption, or other gastro-intestinal disorders where an elemental diet is indicated
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Nutricrem®
(Semi-solid)
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Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Lactose free diet
  • Low energy per serving as a semi-solid supplement where low volume is required only.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–6 years.
 
   
Nutrini®
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • 1 kcal feed without fibre, used in children 8-20kg. Up to 6 years.
  • Also essential low protein tube feed for children with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) aged 2 to 10y due to its low protein and amino profile as a source of natural protein. It is used when IMD children cannot tolerate fibre. To change to an alternative enteral feed with children with metabolic disorders would potentially require hospital admission to ensure stabilisation with a new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Nutrini® Energy
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml tube feed for children over 8kg
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg

 

 
   
Nutrini® Energy Multi Fibre
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml tube feed for children over 8kg with added fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Nutrini® Multi Fibre
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • 1 kcal feed with fibre, used in children 8-20kg. Up to 6 years.
  • Also, used in low protein tube feeds for children with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) aged 2 to 10y due to its protein and amino acid profile. It provides a source of natural protein. It is used in IMD children who can tolerate fibre. To change to an alternative enteral feed with children with metabolic disorders would potentially require hospital admission to ensure stabilisation with a new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Nutrini® Peptisorb
(Liquid (tube feed))
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Formulary
Amber

 

  • Hydrolysate formula
  • 1.0kcal/ml polymeric feed used 8 -20kg where hydrolysed protein is required
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg Follow trust guidance on use of acidic tube feeds.

 

 
   
Nutrini® Peptisorb Energy
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml hydrolysed protein feed used in children over 8kg where whole protein is not tolerated. Additional information re pH of the product?
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–6 years, body-weight 8–20 kg
 
   
Paediasure®
(Liquid (sip or tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • 1 kcal per ml low carbohydrate sip feed for children
  • Paediatric ACBS indications: Disease-related malnutrition, intractable malabsorption, growth failure, pre-operative preparation of malnourished patients, dysphagia, short-bowel syndrome, bowel fistula in child 1–10 years, body-weight 8–30 kg
 
   
Paediasure® Plus
(Liquid (sip or tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • Lower Carbohydrate , 1.5kcal/ml sip feed, used in children with CHO intolerance
  • Paediatric ACBS indications: Disease-related malnutrition, intractable malabsorption, growth failure, pre-operative preparation of malnourished patients, dysphagia, short-bowel syndrome, bowel fistula in child 1–10 years, body-weight 8–30 kg
 
   
Paediasure® Plus Fibre
(Liquid (sip or tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • Lower Carbohydrate , 1.5kcal/ml sip feed, used in children with CHO intolerance with added fibre
  • Paediatric ACBS indications: Disease-related malnutrition, intractable malabsorption, growth failure, pre-operative preparation of malnourished patients, dysphagia, short-bowel syndrome, bowel fistula in child 1–10 years, body-weight 8–30 kg. Not suitable for use in child under 1 year.
 
   
Paediasure® Peptide
(Liquid (sip or tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein
  • Peptide based sip feed used in pts with gut failure
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 1–10 years, body-weight 8–30 kg
 
   
Peptamen® Junior
(Powder)
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Formulary
Amber

 

  • Hydrolysate Formula
  • Powdered preparation only Used in children requiring hydrolysate, ability to concentrate or dilute to meet specific calorie density requirements
  • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula, in child 1–10 years
 
   
Peptamen® Junior
(Liquid)
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Formulary
Amber

 

  • Hydrolysate Formula
  • Ready to use, neutral pH, 1kcal/ml
  • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula, in child 1–10 years
 
   
Peptamen® Junior Advance
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml hydrolysed protein feed used in children over 8kg where whole protein is not tolerated. Increased MCT for use in liver failure.
  • Intractable malabsorption, short-bowel syndrome, bowel fistula, and proven inflammatory bowel disease in child 1–10 years
 
   
PKU Explore 10
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Formulary
Amber
 
   
PKU Explore 5
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Formulary
Amber
 
   
Polycal®
(Liquid)
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Formulary
Amber
  • High-energy supplements: carbohydrate
  • Ready to use. Requires no specific instruction to prepare e.g. energy supplement/ used as pre and post bolus with patients with glycogen storage disease to prevent hypoglycaemia, also used in fatty acid oxidation disorders
  • There is no other liquid glucose supplement on ACBS prescription.
  • Also energy supplements/ used as pre-and post-bolus tube feeds with patients with glycogen storage disease to prevent hypoglycaemia. Also use as a quick access, liquid energy supplement in patients with fatty acid oxidation disorders. There is no other liquid glucose supplement on ACBS prescription. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high or readily available carbohydrate supplement.
 
   
Pro-Cal® Shot
(Liquid)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Used where energy and protein is required in small volume – only on recommendation of dietitian
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement.
  • Not suitable for child under 3 years.
 
   
Pro-Cal® Singles
(Liquid)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Used where energy and protein is required in small volume, where measured doses are required – only on recommendation of dietitian
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement. Not suitable for child under 3 years.
 
   
Prosource® Jelly
(Semi-solid)
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Formulary
Amber
  • High-energy supplements: protein
  • Liver/hepatobiliary disease/conditions.
  • Other patients groups, based on clinical assessment.
  • Pts requiring increased protein in a low volume
  • Hypoproteinaemia. Not recommended for child under 3 years
 
   
Prosource® Liquid
(Liquid)
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Formulary
Amber
  • Protein and carbohydrate
  • Pts requiring low volume, low cal, low CHO protein supplement (10g/30ml) for oral and tube feeders. Useful in allergy to milk and soya proteins. For high protein requirements.
  • Liver/hepatobiliary disease/conditions.
  • Biochemically proven hypoproteinaemia.
  • Not recommended for child under 3 years.
 
   
ProSource® Plus
(Liquid)
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Formulary
Amber
  • Protein and carbohydrate
  • Pts requiring low volume, low cal, low CHO protein supplement (15g/30ml) for oral and tube feeders.
  • Useful in allergy to milk and soya proteins.
  • Liver/hepatobiliary disease/conditions.
  • Hypoproteinaemia.
  • Not recommended for child under 3 years
 
   
ProSource® TF
(Liquid)
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Formulary
Amber
  • Protein and carbohydrate
  • Pts requiring low volume, low cal, low CHO protein supplement (11g/30ml) specifically formulated for tube feeders. Useful in allergy to milk and soya proteins. Low osmolality.
  • Liver/hepatobiliary disease/conditions.
 
   
Protifar®
(Powder)
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Formulary
Amber
  • High-energy supplements: protein
  • High protein modular powder, to be added to other food, drink and feeds in a range of conditions
  • Nutritional supplement for use in biochemically proven hypoproteinaemia.
 
   
Renapro®
(Powder)
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • For patients with high protein needs, low volume, low phosphate requirements.
  • Used in renal patients.
  • Used in liver/hepatobiliary diseases/conditions.
 
   
Renapro® shot
(Liquid)
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Formulary
Amber
  • For patients with high protein needs, low phosphate requirements.
  • Used for renal patients.
  • Used in liver/hepatobiliary diseases/conditions
 
   
Renastart®
(Powder)
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Formulary
Amber
  • Specialised formulas for specific clinical conditions
  • Used in infants and children with renal disease when low Potassium and Low phosphate feed is required, can be given orally or as a tube feed
  • Dietary management of renal failure in child from birth to 10 years.
 
   
Renastart®
(Powder)
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Formulary
Amber
  • Used in infants and children with renal disease when low Potassium and Low phosphate feed is required, can be given orally or as a tube feed
  • Dietary management of renal failure in child from birth to 10 years.
 
   
Renastep®  (Liquid)
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Formulary
Amber
  • ACBS criteria: For the dietary management of renal failure from 3 years of age.
  • Patients with renal failure from 3 years of age.
  • Use under medical supervision.
  • Not suitable for use as a sole source of nutrition.
  • Suitable from 3 years of age onwards.
  • Regular monitoring of nutritional status and electrolyte levels is required.
  • For enteral use only
 
   
Renilon® 7.5
(Liquid (sip feed))
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Formulary
Amber
  • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
  • Low potassium, low phosphate sip feed
  • Dietetic advice
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
  • Not suitable for child under 3 years; use with caution in child 3–5 years.
 
   
Resource® Junior
(Liquid (sip feed))
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Formulary
Amber
  • Child: More than 1 kcal/mL and less than 4 g protein/100 mL
  • First Choice Alternative to Fortini, for patients who have taste fatigue - long term use
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula. Not suitable for use in child under 3 years
 
   
Resource® Optifibre
(Powder)
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Formulary
Amber
  • High-fibre supplements
  • A fibre source added to oral and tube feeds
  • This is added to low protein tube feeds for patients with propionic acidaemia, methyl malonic acidaemia to provide a source of fibre. Children with these conditions become easily constipated leading to metabolic instability and hospital admissions so an additional source extra to fibre content of existing enteral feeds is essential. this product cannot be purchased from the supermarket and is essential. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula except dysphagia
  • Not suitable for child under 5 years
 
   
S.O.S®
(Powder)
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Formulary
Amber
  • High-energy supplements: carbohydrate
  • Individual dose sachets, to be made as needed –pre measured dose to be made up by parents.
  • Essential for use in an emergency regimen in inborn errors of metabolism / module in tube feed for low amino acid or fat diets / energy supplement.
  • Also essential core ingredient in emergency feeds for inborn errors of metabolism / or used as an Individual carbohydrate module in tube feeds for low amino acid or fat diets / or as an energy supplement. Particularly important to use with non-English speaking families and vulnerable families as it is supplied in pre-measured sachets. Research as shown that it is safer to use than conventional glucose polymer powders. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • For use as an emergency regimen in the dietary management of inborn errors of metabolism in adults and children from birth.
 
   
Similac® Alimentum
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: Hydrolysate formula
  • Casein hydrolysate with MCT. Use in infants (from birth – 6 mths) ) who have cow’s milk intolerance / allergy and also malabsorption. Tube or oral feed. Similac has fat as 50% MCT and more complex CHO
  • Cows’ milk protein allergy and other conditions where an extensively hydrolysed formula is indicated.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Slo drinks milkshakes
(powdered)
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Formulary
Amber
  • 1.5 kcal/ml and 5g (or more) protein/100ml
  • First line For dysphagia pts only
  • Not suitable for CKD 4-5, renal pts.
  • Not suitable for lactose intolerance
  • Not suitable for bile acid malabsorption or fat maldigestion associated with biliary diseases/conditions
  • To be made up with whole milk.
  • Contains 24g protein per serving as served.
 
   
SMA LF® Lactose Free Formula (previous name SMA® LF)
(Powder)
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Formulary
Green
  • Specialised formulas: Infant and child: Residual lactose formula
  • Can be purchased over the counter.
  • Only prescribable for patients who currently do not pay for formula feeds (eg healthy start scheme)
  • For infants with lactose intolerance
  • Proven lactose intolerance
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
Tentrini®
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • Polymeric feed, used in children 20-45kg as tube feed, no added fibre
  • Also essential low protein tube feeds for teenagers with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) due to its protein profile. It provides a natural protein source. It is used when IMD teenagers cannot tolerate fibre. To change to an alternative enteral feed with teenagers with metabolic disorders would potentially require hospital admission to ensure stabilisation with a new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 7–12 years, body-weight 21–45 kg
 
   
Tentrini®
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • Polymeric feed, used in children 20-45kg as tube feed, no added fibre
  • Also essential low protein tube feeds for teenagers with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) due to its protein profile. It provides a natural protein source. It is used when IMD teenagers cannot tolerate fibre. To change to an alternative enteral feed with teenagers with metabolic disorders would potentially require hospital admission to ensure stabilisation with a new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 7–12 years, body-weight 21–45 kg
 
   
Tentrini®
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • Polymeric feed, used in children 20-45kg as tube feed, no added fibre
  • Also essential low protein tube feeds for teenagers with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) due to its protein profile. It provides a natural protein source. It is used when IMD teenagers cannot tolerate fibre. To change to an alternative enteral feed with teenagers with metabolic disorders would potentially require hospital admission to ensure stabilisation with a new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure in child 7–12 years, body-weight 21–45 kg
 
   
Tentrini® Energy
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml tube feed for children 20-45kg
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula and growth failure, in child 7–12 years, body-weight 21–45 kg
 
   
Tentrini® Energy Multi Fibre
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1.5 kcal/mL and more than 4 g protein/100 mL
  • 1.5kcal/ml tube feed for children 20-45kg with added fibre
  • Paediatric ACBS indications: Disease-related malnutrition, intractable malabsorption, growth failure, pre-operative preparation of malnourished patients, dysphagia, short-bowel syndrome, bowel fistula and proven inflammatory bowel disease, in child 7–12 years, body-weight 21–45 kg
 
   
Tentrini® Multi Fibre
(Liquid (tube feed))
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Formulary
Amber
  • Child: 1 kcal/ml and less than 4g protein/100ml
  • Polymeric feed, used in children 20-45kg as tube feed, added fibre
  • Also essential in low protein tube feeds for teenagers with inborn errors of protein metabolism (e.g. organic acidaemias, urea cycle disorders) due to its protein profile. It provides a natural protein source. It is used when IMD teenagers cannot tolerate fibre. To change to an alternative enteral feed with teenagers with metabolic disorders would potentially require hospital admission to ensure stabilisation with new enteral feed. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula except bowel fistula, and growth failure in child 7–12 years body-weight 21–45 kg

 

 
   
Vitasavoury®
(Powder)
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Formulary
Amber
  • Protein, fat, and carbohydrate
  • Taste fatigue to sweet products
  • Disease-related malnutrition, malabsorption states, or other conditions requiring fortification with a high fat or carbohydrate (with protein) supplement.
  • Not suitable for child under 3 years.
 
   
Wysoy®
(Powder)
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Formulary
Amber
  • Specialised formulas: Infant and child: Soya-based formula
  • Essential for galactosaemia. The only completely lactose free formula, soya based formula suitable for these infants, recommended in international galactosaemia guidelines for feeding infants. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Proven lactose and associated sucrose intolerance in pre-school children, galactokinase deficiency, galactosaemia, and proven whole cows' milk sensitivity.
 
Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
   
09.04  Expand sub section  Nutritional supplements for metabolic diseases
 note 

Disorders of lysine metabolism


Glutaric aciduria Type 1 (GA1) is a very rare organic acidaemia with an inability to metabolise the amino acids lysine and tryptophan. Without attentive and appropriate dietary treatment, it can lead to severe movement problems, irreversible brain damage and death. There are < 5 patients in the Birmingham area on dietary treatment. Treatment involves a low protein diet (commonly less than 1g/kg body weight/ day natural protein), and supplementation with a protein substitute free of lysine and low in tryptophan (and usually supplemented with vitamins, minerals and DHA). Patients require access to low protein special foods. The protein substitutes are essential: not only do they supply essential amino acids, they help prevent entry of lysine across the blood brain barrier. Please note poor adherence or lack of access to protein substitute could lead to an encephalopathic crisis, irreversible brain damage and death. The dose of protein equivalent prescribed per/ kg body weight/day is: 1 -1.5g/kg bodyweight/day.

Protein substitutes are usually taken at least 3 times daily.
The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.

NB: Please note that these products have a poor taste and smell. It is necessary to have a wide choice of products available to find a single product that a patient may be able to tolerate.

In addition, patients with GA1 need to strictly follow an emergency regimen during illness, which is associated with catabolism of lysine and has potential to cause irreversible encephalopathic crisis. The emergency regimen is a special feed consisting of glucose polymer, lysine-free, low tryptophan amino acid supplements. The emergency feed is commonly administered via a nasogastric/gastrostomy tube at home. A suitable
lysine-free, low tryptophan amino acid that can be administered via a tube is required. This may be a different supplement to their maintenance L-amino acid supplement. The dose and frequency of administration will be determined by the specialist dietitian.

Pyridoxine responsive epilepsy (PDE) is a rare disorder causing metabolic epilepsy, neurodevelopmental disabilities including developmental delay and intellectual disability. There is only 1 patient in the Birmingham area. The epilepsy responds to pharmacological doses of pyridoxine but inadequate degradation of lysine leads to accumulation of neurotoxic compounds associated with developmental disability. Therefore, this condition is also treated with a low protein diet and protein substitute using the GA1 protein substitutes. The dose of protein equivalent prescribed per/ kg body weight/day is: 1 -1.5g/kg bodyweight/day. Patients require access to low protein special foods

Paediatric Seravit®
(Powder)
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Formulary
Amber
  • Vitamin and Mineral supplements
  • Used in children and low weight adults on very restricted diets who are not meeting vitamin and mineral requirements and in enteral feed patients.
  • For use in infants - unflavoured vitamin and mineral supplement given to infants and young children on low protein or low-fat diets who require vitamin and mineral supplementation. There are no other powdered preparations available that meet the requirements of infants on very limited diets with inherited metabolic disorders of metabolism. It cannot be purchased from a supermarket. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient
  • Vitamin, mineral, and trace element supplement in infants and children with restrictive therapeutic diets
 
   
09.04  Expand sub section  Glutaric aciduria (type 1)
GA Amino 5
(Powder)
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Formulary
Amber
  • Amino acid supplement without lysine – low in tryptophan for infants with GA1 - used for tube feeding in emergencies
  • The only pre-measured amino acid supplement without lysine, low in tryptophan for children with GA1 that can be administered via a tube in emergency feeds. It is essential it is easy to use in an emergency and pre-measured is safer, particularly for non-English speaking families. It can also be used in pyridoxine responsive epilepsy.
 
   
GA Anamix Junior
(Powder)
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Formulary
Amber
  • An unthickened amino acid supplement without lysine, low in tryptophan for children with GA1 that can be given as a drink. It can also be used in pyridoxine responsive epilepsy.
 
   
GA Express 15 (not ACBS approved)
(Powder)
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Formulary
Amber
  • Amino acid supplement without lysine and low in tryptophan for children and adults with GA1
  • The only low volume, vitamin and mineral amino acid supplement without lysine, low in tryptophan for use in patients > the age of 6 years’ children and adults with GA1 and pyridoxine responsive epilepsy.
 
   
GA Gel®
(Powder)
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Formulary
Amber
  • Amino acid supplement without lysine – low in tryptophan for infants with GA1
  • The only weaning (thickened) amino acid supplement without lysine, low in tryptophan for children with GA1. It can also be used in pyridoxine responsive epilepsy.
  • Nutritional supplement for dietary management of type 1 glutaric aciduria in children 6 months–10 years.
 
   
GA1 Anamix® Infant
(Powder)
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Formulary
Amber
  • Amino acid supplement without lysine – low in tryptophan for infants with GA1
  • The only amino acid supplement without lysine, low in tryptophan for infants with GA1 and pyridoxine responsive epilepsy.
  • Nutritional supplement for the dietary management of proven glutaric aciduria (type 1) in children from birth to 3 years.
 
   
09.04  Expand sub section  Glycogen storage disease
Corn flour and corn starch
(Powder)
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Formulary
Amber
  • For glycogen storage disease
  • Slow release CHO to prevent hypoglycaemia in GSD
  • Slow release carbohydrate to prevent hypoglycaemia in all types of GSD. Cheaper than glycosade and easier to mix. Used in less severe forms of GSD. Between 1 to 2g/kg body weight is prescribed dependent on age, weight and clinical symptoms of hypoglycaemia. Doses may be given at regular intervals during the day and night or at bedtime only. The dose and frequency of dose should always be advised by the specialist dietitian according to the needs of the patient.
 
   
Glycosade®
(Powder)
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Formulary
Amber
  • Slow release CHO to prevent hypoglycaemia in GSD
  • The only specially formulated slow release carbohydrate to prevent hypoglycaemia in severe forms of GSD e.g. Type I and III which allows extended fasting time. The dosage is dependent on age, weight and clinical symptoms of hypoglycaemia. Doses may be given at regular intervals during the day and night or at bedtime only. The dose and frequency of dose should always be advised by the specialist dietitian according to the needs of the patient. Glycosade is in a pre-measured sachet. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • A nutritional supplement for use in the dietary management of glycogen storage disease and other metabolic conditions where a constant supply of glucose is essential. Not suitable for use in children under 2 years.
 
   
09.04  Expand sub section  Homocystinuria or hypermethioninaemia to top
 note 

Homocystinuria (HCU) is a very rare amino acid disorder (1 in 344 000 in classical HCU) with an inability to metabolise the amino acid methionine. There are only 5 patients in the Birmingham area on dietary treatment. Treatment involves a life-long low protein diet and supplementation with a protein substitute free of methionine (and usually supplemented with vitamins, minerals and DHA). Patients require access to low protein special
foods. The protein substitutes are essential: not only do they supply essential amino acids, they help maintain metabolic control and usually provide a source of other nutrients. Please note poor adherence leads or lack of access to protein substitute to loss of metabolic control, potentially stroke and even death.
The dose of protein equivalent from amino acid supplement prescribed per/ kg body weight/day (also considering natural protein tolerance) is:


Children 0-3 years: 3g/kg body weight /day
Children 4-6 years: 2.5g/kg body weight/day
Children 7 – 12 years: 1.5-2g/kg body weight/day
Over 12 years and adults: 1-1.5g/kg body weight/day usually to an upper amount of 80g day (unless extreme needs/athletes)
Pregnancy: >70g/day total
Protein substitutes are usually taken at least 3 times daily.
The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.

NB: Please note that these products have a poor taste and smell. It is necessary to have a wide choice of products available to find a single product that a patient may be able to tolerate.

Blood cysteine concentrations may be low and it may be necessary to supplement with extra cystine supplements. The dose and type of phenylalanine powder should always be advised by the specialist dietitian according to the needs of the patient

HCU Anamix® Infant
(Powder)
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Formulary
Amber
  • Amino acid supplement without methionine for infants with homocystinuria
  • The only amino acid supplement without methionine for infants with homocystinuria. It is given to screened infants for HCU. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Nutritional supplement for the dietary management of proven vitamin B6 non-responsive homocystinuria or hypermethioninaemia in children from birth to 3 years.
 
   
HCU Cooler® 10
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for children and adults with homocystinuria
  • The only liquid amino acid supplement without methionine for children with homocystinuria aged 3 to 6 years. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements.
 
   
HCU Cooler® 15
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for teenagers and adults with homocystinuria
  • The only liquid amino acid supplements without methionine for children with homocystinuria aged 7 to 12 years. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements.
 
   
HCU Cooler® 20
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for teenagers and adults with homocystinuria
  • One of two liquid amino acid supplements without methionine for children with homocystinuria aged >12 years. They need the larger sachets (20g protein equivalent) meet their non-methionine protein requirements.
 
   
HCU Express® 15
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for teenagers and adults with homocystinuria
  • The only powdered amino acid supplement without methionine that provides 15g protein equivalent per sachet for children with homocystinuria aged 7 to 12 years. Larger sachets are unacceptable for this age group of children and provide in excess of protein requirements.
  • A methionine-free protein substitute for use as a nutritional supplement in children over 8 years with homocystinuria
 
   
HCU Express® 20
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for teenagers and adults with homocystinuria
  • One of 2 powdered amino acid supplements without methionine that provides 20g protein equivalent per sachet for teenagers with homocystinuria aged >12 years. They need the larger pouches to meet their non-methionine protein requirements. It is important we have a choice of more than 1 product for this age group as adherence is poor.
  • A methionine-free protein substitute for use as a nutritional supplement in children over 8 years with homocystinuria.
 
   
HCU gel®
(Powder)
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Formulary
Amber
  • Amino acid supplement without methionine for children with homocystinuria
  • The only weaning (thickened) amino acid supplement without methionine for children with homocystinuria.
  • A methionine-free protein substitute for use as a nutritional supplement for the dietary management of children 1–10 years with homocystinuria.
 
   
HCU Lophlex® LQ 20
(Liquid)
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Formulary
Amber
  • Amino acid supplement without methionine for teenagers and adults with homocystinuria
  • One of 2 powdered amino acid supplements without methionine that provides 20g protein equivalent per sachet for teenagers with homocystinuria aged >12 years. They need the larger pouches to meet their non-methionine protein requirements. It is important we have a choice of more than 1 product for this age group as adherence is poor.
  • Nutritional supplement for the dietary management of homocystinuria in children over 3 years.
 
   
09.04  Expand sub section  Hyperlysinaemia
09.04  Expand sub section  Isovaleric acidaemia
 note 

Isovaleric acidaemia is a very rare organic acidaemia with an inability to metabolise leucine. The incidence is unknown and there are <10 patients in the Birmingham area. The condition is mainly treated by a low protein diet but a leucine-free L-amino acid infant feed may be used in infancy to supplement protein restriction and to aid growth. It is now unusual to use a leucine free L-amino acid supplement in patients over the age of 1 year as they should be able to tolerate safe levels of natural protein intake (WHO/FAO/UNU 2007).

IVA Anamix® Infant
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine for infants with isovaleric acidaemia
  • The only infant amino acid supplement without leucine, for infants with isovaleric acidaemia. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • Nutritional supplement for the dietary management of proven isovaleric acidaemia or other proven disorders of leucine metabolism in children from birth to 3 years.
 
   
09.04  Expand sub section  Maple syrup urine disease
 note 

Maple syrup urine disease (MSUD) is a very rare amino acid disorder (1 in 116 000) with an inability to metabolise the amino acids leucine, valine and isoleucine. Without attentive and appropriate dietary treatment, it can lead to irreversible
brain damage and death. There is only 1 patient in the Birmingham area on dietary treatment. Treatment involves a life-long low protein diet and supplementation with a protein substitute free of leucine, valine and isoleucine (and usually
supplemented with vitamins, minerals and DHA). Patients require access to low protein special foods. The protein substitutes are essential: not only do they supply essential amino acids, they help maintain metabolic control and usually
provide a source of other nutrients. Please note poor adherence leads or lack of access to protein substitute to loss of metabolic control, irreversible brain damage and even death.
The dose of protein equivalent from amino acid supplement prescribed per/ kg body weight/day (also considering natural protein tolerance) is:

Children 0-3 years: 3g/kg body weight /day
Children 4-6 years: 2.5g/kg body weight/day
Children 7 – 12 years: 1.5-2g/kg body weight/day
Over 12 years and adults: 1-1.5g/kg body weight/day usually to an upper amount of 80g day (unless extreme needs/athletes)
Pregnancy: >70g/day total
Protein substitutes are usually taken at least 3 times daily.
The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.

NB: Please note that these products have a poor taste and smell. It is necessary to have a wide choice of products available to find a single product that a patient may be able to tolerate.

Patients ability to tolerate valine and isoleucine is usually better than their tolerance of ability to leucine. It is not uncommon for children to have low plasma valine and isoleucine levels. If this is not corrected it can lead to poor metabolic control so valine and isoleucine supplements are commonly required. The dose and frequency of administration will be determined by the specialist dietitian.

In addition, patients with MSUD need to go on an emergency regimen during illness, which is associated with catabolism of leucine and poor metabolic control. The emergency regimen is a special feed consisting of glucose polymer, the
MSUD L-amino acid supplement with additional valine and isoleucine. It is commonly administered via a nasogastric/gastrostomy tube at home. A suitable MSUD amino acid that can be administered via a tube is commonly required.
This may be a different supplement to their maintenance L-amino acid supplement. The dose and frequency of administration will be determined by the specialist dietitian.

MSUD Amino 5
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for infants/children with MSUD
  • The only pre-measured amino acid supplement, without leucine, valine, isoleucine for infants/children with maple syrup urine disease used for emergency regimens and tube feeds. This is particularly important as most of our families with MSUD do not speak English as their first language.
 
   
MSUD Anamix® Infant
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for infants with MSUD
  • The only infant amino acid supplement without leucine, valine, isoleucine for maple syrup urine disease.
  • Nutritional supplement for the dietary management of proven maple syrup urine disease in children from birth to 3 years.
 
   
MSUD Anamix® Junior
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for children with MSUD
  • The only infant amino acid supplement without leucine, valine, isoleucine for maple syrup urine disease.
  • Nutritional supplement for the dietary management of maple syrup urine disease in children 1–10 years.
 
   
MSUD Anamix® Junior LQ
(Liquid)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for children with MSUD. Can be used in adults
  • An unthickened powdered amino acid supplement without leucine, valine, isoleucine for children aged 1 to 10y with maple syrup urine disease which can be given as a drink.
  • Nutritional supplement for the dietary management of maple syrup urine disease in children 1–10 years.
 
   
MSUD cooler® 10
(Liquid)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for children and adults with MSUD
  • An oral liquid amino acid supplement without leucine, valine, isoleucine formulated for children aged 3 to 6 years with maple syrup urine disease. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements
 
   
MSUD cooler® 15
(Liquid)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for teenagers with MSUD
  • An oral liquid amino acid supplement without leucine, valine, isoleucine for children aged 7 to 12 years with maple syrup urine disease. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements.
 
   
MSUD cooler® 20
(Liquid)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for teenagers and adults with MSUD
  • One of 2 oral flavoured liquid amino acid supplements without leucine, valine, isoleucine for teenagers over 12 years of age and adults. This age group need the larger pouches to meet their non-leucine protein requirements. It is important we have a choice of more than 1 product as compliance is poor. It is essential that patients are offered choice of more than 1 protein substitute.
 
   
MSUD express® 15
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for teenagers and adults with MSUD
  • The only powdered amino acid supplement without leucine, valine, isoleucine for 7 to 12-year olds with maple syrup urine disease. Larger sachets are unacceptable for this age group of children and provide in excess of protein requirements.
  • Nutritional supplement for the dietary management of maple syrup urine disease in children over 8 years and adults.
 
   
MSUD express® 20
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for teenagers and adults with MSUD
  • The only powdered amino acid supplement without leucine, valine, isoleucine for teenagers and adults with maple syrup urine disease that will met their higher BCAA-free amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-methionine protein requirements
  • Nutritional supplement for the dietary management of maple syrup urine disease in children over 8 years and adults.
 
   
MSUD Gel®
(Powder)
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Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for children with MSUD
  • The only weaning (thickened) amino acid supplement without leucine, valine, isoleucine for infants/young children with maple syrup urine disease.
  • Nutritional supplement for the dietary management of maple syrup urine disease in children 1–10 years.
 
   
MSUD Lophlex® LQ 20
(Liquid)
View adult BNF View SPC online View childrens BNF
Formulary
Amber
  • Amino acid supplement without leucine, valine and isoleucine for teenagers and adults with MSUD
  • One of 2 oral flavoured amino acid supplement without leucine, valine, isoleucine for teenagers over 12 years of age and adults with maple syrup urine disease. They need the larger pouches to meet their BCAA-free amino acid requirements. It is important we have a choice of more than 1 product for this age group as compliance is poor. It is essential that patients are offered choice of more than 1 protein substitute.
  • Nutritional supplement for the dietary management of maple syrup urine disease in children over 3 years.
 
   
09.04  Expand sub section  Methylmalonic or propionic acidaemia
 note 

Methylmalonic acidaemia and propionic acidaemia are very rare organic acidaemias (estimates vary but approximately for each condition: 1 in 50,000). Combined, there are less than 10 patients in the Birmingham area with these
conditions. Both conditions have an inability to metabolise methionine, valine, threonine and isoleucine. The conditions are mainly treated by a low protein diet. However, a methionine, valine, threonine and isoleucine L-amino acid infant
feed may be used in infancy to supplement protein restriction and to aid growth. An amino acid supplement may be used in older children if metabolic control is poor or children tolerate less than the safe levels of natural protein intake
(WHO/FAO/UNU 2007). The dosage used usually provides no more than 20% of total protein requirements. The dose and frequency of administration will be determined by the specialist dietitian.

MMA/PA amp 5
(Powder)
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  • Methionine, threonine and valine free, and low isoleucine aa supplement
  • The only pre-measured sachet of methionine, threonine, valine free and low isoleucine amino acid supplement for patients with propionic acidaemia and methyl malonic acidaemia reliant on tube feeding. A pre-measured sachet is particularly important as most of our families with MSUD do not speak English as their first language so require pre-measured products.
 
   
MMA/PA Anamix® Infant
(Powder)
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  • Amino acid supplement without methionine, threonine, valine and isoleucine for infants with propionic acidaemia and methyl malonic acidaemia
  • The only infant amino acid supplement without methionine, threonine, valine and isoleucine for patients with propionic acidaemia and methyl malonic acidaemia
  • Nutritional supplement for the dietary management of proven methylmalonic acidaemia or propionic acidaemia in children from birth to 3 years.
 
   
09.04  Expand sub section  Other inborn errors of metabolism to top
Cystine500®
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  • To correct cysteine deficiency in homocystinuria
  • The only pre-measured sachet of cysteine to correct cysteine deficiency in homocystinuria. The dose and frequency of administration will be determined by the specialist dietitian.
  • Nutritional supplement for the dietary management of inborn errors of amino acid metabolism in adults and children from birth.
 
   
DocOmega®
(Powder)
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  • DHA supplement for low chain fatty acid and low protein diets
  • The only ACBS prescribed DHA supplement for low protein diets. The dose and frequency of administration will be determined by the specialist dietitian.
  • Nutritional supplement for the dietary management of inborn errors of metabolism for adults and children from birth.
 
   
EAA® Supplement
(Powder)
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  • Consists of essential amino acids for the treatment of urea cycle disorders
  • Essential amino acid supplement for use in urea cycle disorders and the cerebral creatine deficiency syndrome: guanidinoacetate methyltransferase (GAMT) deficiency. The only pre-measured sachet of essential amino acids with added vitamins and minerals for the treatment of urea cycle disorders. This is particularly important as most of our families with UCD do not speak English as their first language so require pre-measured products. Unmeasured use of essential amino acid supplements in UCD could lead to hyperammonaemia, acute brain encephalopathy and ITU admission. The dose and frequency of administration will be determined by the specialist dietitian.
  • Nutritional supplement for the dietary management of disorders of protein metabolism including urea cycle disorders in children over 3 years.
 
   
Isolecuine50®
(Powder)
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  • To correct isoleucine deficiency in MSUD
  • The only pre-measured sachet of isoleucine that provides 50 mg/dose to correct isoleucine deficiency in MSUD. A pre-measured sachet is particularly important as most of our families with MSUD do not speak English as their first language so require pre-measured products. The dose and frequency of administration will be determined by the specialist dietitian.
  • Nutritional supplement for use in the dietary management of inborn errors of amino acid metabolism in adults and children from birth.
 
   
Isoleucine 1000
(Powder)
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Red
  • Used as an inpatient only
  • Used at the time of diagnosis when on haemofiltration and an isoleucine supplement is necessary.
 
   
KeyOmega®
(Powder)
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  • Long chain fatty acid supplementation in very low fat diets
  • Nutritional supplement for the dietary management of inborn errors of metabolism.
 
   
ProZero®
(Liquid)
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  • Low protein milk replacer in children and adults with inborn errors of protein metabolism on low protein diets
  • A protein-free milk in children and adults with inborn errors of protein metabolism on low protein diets. It contains equal calories per 100 ml to cow’s milk. It is particularly suitable for MSUD patients. Available in 1 litre /250 ml cartons. The dose and frequency of administration will be determined by the specialist dietitian
  • A protein-free nutritional supplement for the dietary management of inborn errors of metabolism in children over 6 months and adults.
 
   
UCD Amino5
(Powder)
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  • Essential amino acid supplement for use in urea cycle disorders
  • Essential amino acid supplement for use in urea cycle disorders and the cerebral creatine deficiency syndrome: guanidinoacetate methyltransferase (GAMT) deficiency. The only pre-measured sachet of essential amino acids without vitamins and minerals (designed for tube feeding) for the treatment of urea cycle disorders. This is particularly important as most of our families with urea cycle disorders do not speak English as their first language so require pre-measured products. Unmeasured use of essential amino acid supplements in UCD could lead to hyperammonaemia, acute brain encephalopathy and ITU admission. The dose and frequency of administration will be determined by the specialist dietitian
 
   
Valine 1000®
(Powder)
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Red
  • Used as an inpatient only
  • Used at the time of diagnosis when on haemofiltration and a valine supplement is necessary
 
   
Valine50®
(Powder)
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  • To correct valine deficiency in MSUD
  • The only pre-measured sachet of valine that provides 50 mg/dose to correct valine deficiency in MSUD. A pre-measured sachet is particularly important as most of our families with MSUD do not speak English as their first language so require pre-measured products. The dose and frequency of administration will be determined by the specialist dietitian
  • Nutritional supplement for the dietary management of inborn errors of amino acid metabolism in adults and children from birth.
 
   
09.04  Expand sub section  Phenylketonuria
 note 

Phenylketonuria (PKU)
In phenylketonuria (PKU), a lifelong, low phenylalanine diet (consisting of severe restriction of natural protein, supplementation with phenylalanine free protein substitute and low protein specialist foods), is the only proven treatment that
prevents irreversible brain damage, significant delays in development, and hyperactive behaviour with autistic features in children. In adults it prevents neuropsychiatric co-morbidities and maternal PKU syndrome.
It is a very rare amino acid disorder (1 in 12, 600 in West Midlands) with an inability to metabolise the amino acid phenylalanine, leading to irreversible brain damage if untreated or if treatment is sub-optimal. There are only 80 patients in
the Birmingham area. The protein substitutes are essential: not only do they supply essential amino acids; they help maintain metabolic control and usually provide a source of other nutrients. Poor adherence leads to loss of metabolic
control. In patients with classical PKU, they are likely to provide > 80% of protein requirements.
The dose of protein equivalent from amino acid supplement prescribed per/ kg body weight/day (also considering natural protein tolerance) is:

Children 0-3 years: 3g/kg body weight /day
Children 4-6 years: 2.5g/kg body weight/day
Children 7 – 12 years: 1.5-2g/kg body weight/day
Over 12 years and adults: 1-1.5g/kg body weight/day usually to an upper amount of 80g day (unless extreme needs/athletes)
Pregnancy: >70g/day total
Protein substitutes are usually taken at least 3 times daily.
The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.

There is a subsection of older PKU adults that have stopped diet because they were advised that it was safe in teenage years (in the 1970’s -80’s) or they could not tolerate the traditional L-amino acid products. More untreated adults are
now presenting with high phenylalanine concentrations and symptoms such as headaches, tiredness, lack of concentration, low mood, anxiety, short term memory loss and lack of executive function.

They have white matter changes on the brain MRI scans concurrent with high brain phenylalanine concentrations. All adult patients are now encouraged to return to a low phenylalanine diet. We estimate that there are 30 patients who
need to return to a low phenylalanine diet in the Birmingham area. L-amino acid supplements have a poor taste and smell. It is necessary to have a wide choice of products available to find a single product that a patient may be able to
tolerate. Please note inability to take the protein substitute or lack of access to supply could lead too irreversible brain damage.
Also high blood phenylalanine levels during pregnancy have a teratogenic effect on the developing foetus that can result in growth retardation, microcephaly, intellectual disabilities and birth defects, including congenital heart defects. It is
particularly important that women maintain a very strict diet pre-conception and throughout pregnancy.

Dalia
(Liquid)
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  • Low protein milk for children and adults with phenylketonuria and other disorders of protein metabolism
  • Low protein milk (similar energy content to cow’s milk) for children and adults with phenylketonuria and other disorders of protein metabolism.
 
   
Glytactin Bettermilk 15
(Powder)
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  • A low phenylalanine supplement derived from whey protein for children and adults with phenylketonuria. It is more palatable than amino acid supplements and associated with better utilisation of protein. Very useful for adult patients trying to return to diet or children / adults struggling to take their amino acid product because of its taste. Suitable from aged 4 years
 
   
Glytactin Bettermilk RTD 15 (original and chocolate)
(Liquid)
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  • A low phenylalanine supplement derived from whey protein for children and adults with phenylketonuria. It is more palatable than amino acid supplements and associated with better utilisation of protein. Very useful for adult patients trying to return to diet or children / adults struggling to take their amino acid product because of its taste. Suitable from aged 4 years
 
   
Lophlex®
(Powder)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • One of 2 powdered amino acid supplement without phenylalanine for teenagers and adults with phenylketonuria that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-phenylalanine protein requirements. It is important we have a choice of more than 1 product for this age group as compliance is reported to be poor and the potential outcome of poor compliance is poor executive functioning. The 2 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of proven phenylketonuria in children over 8 years and adults including pregnant women.
 
   
Loprofin® PKU Drink
(Liquid)
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  • Low protein milk for children and adults with phenylketonuria and other disorders of protein metabolism
  • Low energy protein milk for children and adults with phenylketonuria and other disorders of protein metabolism who are overweight
  • Nutritional supplement for the dietary management of phenylketonuria in children over 1 year and adults.
 
   
Loprofin® Sno-Pro
(Liquid)
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  • Specialised formulas for specific clinical conditions
  • Low protein, low potassium, low Phosphate milk substitute , used as a milk
    replacement in children with renal disease and IMD
  • Nutritional supplement for the dietary management of phenylketonuria, chronic renal failure and other inborn errors of amino acid metabolism.
 
   
Phlexy-10® Exchange System
(Powder/Tablets)
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  • Tablets maybe used in pregnancy, when suffering from morning sickness
  • Nutritional supplement for the dietary management of phenylketonuria.
 
   
Phlexy-Vits®
(Powder/Tablets)
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  • Vitamin and mineral supplement for teenagers and adults with phenylketonuria and other inborn errors of protein metabolism
  • The only suitable low volume (powder and tablets) comprehensive vitamin and mineral supplement for teenagers and adults that has been formulated for patients with phenylketonuria and other inborn errors of protein metabolism requiring very restrictive diets. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
  • For use as a vitamin and mineral component of restricted therapeutic diets in children over 11 years and adults with phenylketonuria and similar amino acid abnormalities.
 
   
PKU Air 15
(Liquid)
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  • A liquid amino acid which is lower in calories for children and adults with phenylketonuria
 
   
PKU Air 20
(Liquid)
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  • A supplement which is lower in calories for children and adults with phenylketonuria
 
   
PKU Anamix first spoon
(Powder)
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  • A supplement without phenylalanine for children with phenylketonuria
  • One of 2 powdered weaning (thickened) amino acid supplement without phenylalanine for infants/young children with phenylketonuria. The consistency can be adjusted to suit the developmental age of the older baby/toddler. This is our preferable product because it has added long chain fatty acids.
  • Nutritional supplement for the dietary management of proven phenylketonuria in children from 6 months to 5 years.
 
   
PKU Anamix® Infant
(Powder)
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  • A supplement without phenylalanine for infants with phenylketonuria
  • The only infant amino acid supplement without phenylalanine for infants with phenylketonuria
  • Nutritional supplement for the dietary management of proven phenylketonuria in children from birth to 3 years.
 
   
PKU cooler 10®
(Liquid)
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  • A supplement without phenylalanine for children and adults with phenylketonuria
  • An oral liquid amino acid supplement without phenylalanine formulated for children aged 3 to 6 years with phenylketonuria. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements. The 3 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of phenylketonuria in children over 3 years
 
   
PKU cooler 15®
(Liquid)
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  • A supplement without phenylalanine for children and adults with phenylketonuria
  • One of three oral liquid amino acid supplements without phenylalanine for children aged 7 to 12 years with phenylketonuria. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements.
 
   
PKU cooler 20®
(Liquid)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • One of 3 liquid amino acid supplement without phenylalanine for teenagers and adults (>12 years of age) with phenylketonuria that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-phenylalanine protein requirements. It is important there is a choice of more than 1 product for this age group as compliance is poor and the potential outcome of poor compliance. The 3 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of phenylketonuria in children over 3 years.
 
   
PKU express15®
(Powder)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • The only powdered amino acid supplement without phenylalanine for 7 to 12-year olds with phenylketonuria. Larger sachets are unacceptable for this age group of children and provide in excess of protein requirements.
  • Nutritional supplement for the dietary management of phenylketonuria. Not recommended for children under 3 years.
 
   
PKU express20®
(Powder)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • One of 2 powdered amino acid supplement without phenylalanine for teenagers and adults with phenylketonuria that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-phenylalanine protein requirements. It is important there is a choice of more than 1 product for this age group as compliance is poor. The 2 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of phenylketonuria. Not recommended for children under 3 years.
 
   
PKU Lophlex
(Powder)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • One of 2 powdered amino acid supplement without phenylalanine for teenagers and adults with phenylketonuria that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-phenylalanine protein requirements. It is important we have a choice of more than 1 product for this age group as compliance is reported to be poor and the potential outcome of poor compliance is poor executive functioning. The 2 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of proven phenylketonuria in children over 8 years and adults including pregnant women.
 
   
PKU Lophlex® LQ 10
(Liquid)
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  • A supplement without phenylalanine for children and adults with phenylketonuria
  • One of three liquid amino acid supplement without phenylalanine formulated for children aged 3 to 6 years with phenylketonuria. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements. The 3 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of phenylketonuria in children over 4 years and adults including pregnant women.
 
   
PKU Lophlex® LQ 20
(Liquid)
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  • A supplement without phenylalanine for teenagers and adults with phenylketonuria
  • One of 3 liquid amino acid supplements without phenylalanine for teenagers and adults (>12 years of age) with phenylketonuria that will met their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-phenylalanine protein requirements. It is important there is a choice of more than 1 product for this age group as compliance is poor. The 3 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of phenylketonuria in children over 4 years and adults including pregnant women.
 
   
PKU Sphere 15
(Powder)
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A low phenylalanine supplement derived from whey protein for children and adults with phenylketonuria. It is more palatable than amino acid supplements and associated with better utilisation of protein. Very useful for adult patients trying to return to diet or children / adults struggling to take their amino acid product because of its taste. Suitable from aged 4 years

 
   
PKU Sphere 20
(Powder)
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A low phenylalanine supplement derived from whey protein for children and adults with phenylketonuria. It is more palatable than amino acid supplements and associated with better utilisation of protein. Very useful for adult patients trying to return to diet or children / adult struggling to take their amino acid product because of its taste. Suitable from aged 4 years

 
   
PKU Start®
(Liquid)
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  • For use in the dietary management of phenylketonuria.
  • Amino acid supplement. Without phenylalanine for infants with phenylketonuria.
 
   
09.04  Expand sub section  Tyrosinaemia
 note 

Tyrosinaemia Type I, II, and III are very rare amino acid disorders with an inability to metabolise the amino acids tyrosine and phenylalanine. There are less than 10 patients in the Birmingham area. Treatment involves a low protein diet and
supplementation with a protein substitute free of tyrosine and phenylalanine (and usually supplemented with vitamins, minerals and DHA). Patients require access to low protein special foods. The protein substitutes are essential: not only do
they supply essential amino acids, they help maintain metabolic control and usually provide a source of other nutrients. Poor adherence leads to loss of metabolic control. In patients with classical Tyrosinaemia, they are likely to provide > 80%
of protein requirements.
The dose of protein equivalent from amino acid supplement prescribed per/ kg body weight/day (also considering natural protein tolerance) is:

Children 0-3 years: 3g/kg body weight /day
Children 4-6 years: 2.5g/kg body weight/day
Children 7 – 12 years: 1.5-2g/kg body weight/day
Over 12 years and adults: 1-1.5g/kg body weight/day usually to an upper amount of 80g day (unless extreme needs/athletes)
Pregnancy: >70g/day total
Protein substitutes are usually taken at least 3 times daily.
The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.

NB: Please note that these products have a poor taste and smell. It is necessary to have a wide choice of products available to find a single product that a patient may be able to tolerate.

In infants and young children, blood phenylalanine concentrations may be low, and additional phenylalanine supplements may be necessary. The dose and type of phenylalanine powder should always be advised by the specialist dietitian
according to the needs of the patient.

TYR Anamix® Infant
(Powder)
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  • A supplement without tyrosine and phenylalanine for infants with tyrosinaemia
  • The only infant amino acid supplement without tyrosine and phenylalanine for infants with tyrosinaemia
  • Nutritional supplement for the dietary management of proven tyrosinaemia where plasma-methionine concentrations are normal in children from birth to 3 years.
 
   
TYR Anamix® Junior
(Powder)
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  • A supplement without tyrosine and phenylalanine for children with tyrosinaemia
  • An unthickened powdered amino acid supplement without tyrosine and phenylalanine for children aged 1 to 10y with tyrosinaemia
  • Nutritional supplement for the dietary management of proven tyrosinaemia in children 1–10 years.
 
   
TYR Anamix® Junior LQ
(Liquid)
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  • A supplement without tyrosine and phenylalanine for children with tyrosinaemia
  • A liquid amino acid supplement without tyrosine and phenylalanine for children aged 1 to 10y with tyrosinaemia. Ready to use. Can be delivered via a tube.
  • Nutritional supplement for the dietary management of tyrosinaemia type 1 (when nitisinone (NTBC) is used, see "nitisinone"), type II, and type III, in children over 1 year.
 
   
TYR cooler® 10
(Liquid)
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  • A supplement without tyrosine and phenylalanine for children and adults with tyrosinaemia
  • An oral liquid amino acid supplement without tyrosine and phenylalanine formulated for children aged 3 to 6 years with tyrosinaemia. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements. The 2 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of tyrosinaemia in children over 3 years and adults.
 
   
TYR cooler® 15
(Liquid)
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  • A supplement without tyrosine and phenylalanine for teenagers and adults with tyrosinaemia
  • One of two oral liquid amino acid supplements without tyrosine and phenylalanine for children aged 7 to 12 years with tyrosinaemia. Larger pouches are unacceptable for this age group of children and provide in excess of protein requirements.
  • Nutritional supplement for the dietary management of tyrosinaemia in children over 3 years and adults.
 
   
TYR cooler® 20
(Liquid)
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  • A supplement without tyrosine and phenylalanine for teenagers and adults with tyrosinaemia
  • One of 2 liquid amino acid supplements without tyrosine and phenylalanine for teenagers and adults (>12 years of age) with tyrosinaemia that will meet their higher amino acid requirements. This age group needs the larger sachets (20g protein equivalent) to meet their non-tyrosine/phenylalanine protein requirements. It is important we have a choice of more than 1 product for this age group as compliance is poor. The 2 different products have a different degree of sweetness and range of flavours.
 
   
TYR express15®
(Powder)
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  • A supplement without tyrosine and phenylalanine for teenagers and adults with tyrosinaemia
  • The only powdered amino acid supplement without tyrosine and phenylalanine for 7 to 12-year olds with tyrosinaemia. Larger sachets are unacceptable for this age group of children and provide in excess of protein requirements.
  • Nutritional supplement for the dietary management of tyrosinaemia in children over 8 years and adults.
 
   
TYR express20®
(Powder)
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  • A supplement without tyrosine and phenylalanine for teenagers and adults with tyrosinaemia
  • One of 2 powdered amino acid supplements without tyrosine phenylalanine for teenagers and adults with phenylketonuria that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-tyrosine/phenylalanine protein requirements. It is important we have a choice of more than 1 product for this age group as compliance is poor. The 2 different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of tyrosinaemia in children over 8 years.
 
   
TYR Gel®
(Powder)
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  • A supplement without tyrosine and phenylalanine for teenagers with tyrosinaemia
  • The only weaning (thickened) amino acid supplement without tyrosine and phenylalanine for infants/young children with tyrosinaemia.
  • Nutritional supplement for the dietary management of tyrosinaemia in children 1–10 years.
 
   
TYR Lophlex® LQ 20
(Liquid)
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  • A supplement without tyrosine and phenylalanine for teenagers with tyrosinaemia
  • One of 2 liquid amino acid supplements without tyrosine and phenylalanine for teenagers and adults (>12 years of age) with tyrosinaemia that will meet their higher amino acid requirements. This age group need the larger sachets (20g protein equivalent) to meet their non-tyrosine/phenylalanine protein requirements. It is important we have a choice of more than 1 product for this age group as compliance is reported to be poor. different products have a different degree of sweetness and range of flavours.
  • Nutritional supplement for the dietary management of tyrosinaemia in children over 3 years and adults.
 
   
09.04  Expand sub section  ALD
GTO oil
(Liquid)
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  • A cooking oil for dietary treatment of adrenoleukodystrophy. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
 
   
Lorenzo’s oil
(Liquid)
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  • For dietary treatment of adrenoleukodystrophy. The dose and type of product should always be advised by the specialist dietitian according to the needs of the patient.
 
   
09.04  Expand sub section  Other powder feeds
Meritene energis
(Powder)
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Red
  • Prescribed for liver patients, at request of UHB liver/dietetic team only
  • Liver/hepatobiliary and pancreatic conditions.
  • Hospital supply only.
 
   
09.04  Expand sub section  Rarely Used to top
09.04.01  Expand sub section  Foods for special diets
Bread
(Low-protein foods)
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Essential protein-free basic products. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein. 1 slice ordinary bread = 4g protein and is not allowed in low protein diets.

 
   
Cake, biscuits, and snacks
(Low-protein foods)
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Important protein-free foods to provide important energy, assist dietary adherence and provide variety when natural protein tolerance is very limited. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein.

 
   
Cereals
(Low-protein foods)
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Essential protein-free basic products. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein. 2 regular Weetabix = 4.5g protein and this protein amount is more than the amount of protein that may be tolerated.

 
   
Desserts
(Low-protein foods)
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Important protein-free foods to provide important energy, assist dietary adherence and provide variety when natural protein tolerance is very limited. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein.

 
   
Flour mixes and egg substitutes
(Low-protein foods)
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Formulary
Amber

Essential protein-free basic products. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein. 1 ordinary egg = 6g protein. 100g flour = 10g protein.

 
   
Gluten free foods
View adult BNF View SPC online View childrens BNF
Formulary
Amber

Department of Health and Social Care (DHSC) guidance – 

  • GF, GF & Wheat Free (WF) bread (includes rolls, part baked bread, and pizza bases)
  • GF, GF&WF mixes

Follow Coeliac UK prescribing guidance for monthly quantities (based on age)

 
Link  Prescribing gluten-free foods in primary care - FAQs
Link  Prescribing gluten-free foods in primary care: Guidance for CCGs
   
Pasta
(Low-protein foods)
View adult BNF View SPC online View childrens BNF
Formulary
Amber

Essential protein-free basic products. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein. 100g pasta = 10g protein.

 
   
Pizza bases
(Low-protein foods)
View adult BNF View SPC online View childrens BNF
Formulary
Amber

Important protein-free foods to provide important energy, assist dietary adherence and provide variety when natural protein tolerance is very limited. Necessary for children and adults with inborn errors of protein metabolism who do not tolerate very little natural protein. One regular pizza likely to provide 20g + protein.

 
   
Sapropterin Dihydrochloride  (Kuvan®)
View adult BNF View SPC online View childrens BNF
Formulary
Red
 
   
Savoury meals and mixes
(Low-protein foods)
View adult BNF View SPC online View childrens BNF
Formulary
Amber

Important protein-free foods to provide important energy, assist dietary adherence and provide variety when natural protein tolerance is very limited. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein-. 1 ordinary meal is likely to provide ≥25g protein.

 
   
Spreads
(Low-protein foods)
View adult BNF View SPC online View childrens BNF
Formulary
Amber

Important protein-free foods to provide important energy, assist dietary adherence and provide variety when natural protein tolerance is very limited. Necessary for children and adults with inborn errors of protein metabolism who tolerate very little natural protein. 1 portion ordinary spread likely to provide 2-5g protein.

 
   
09.04.02  Expand sub section  Enteral nutrition
Food First
(dietary sheets, fact sheets, recipe cards)
First Choice
Green
Where clinically appropriate encourage a nutrient dense food and fluid intake for patients at risk of malnutrition. 
Homemade milkshake
(recipe card and fact sheet)
First Choice
Green
Where clinically appropriate encourage a standardised home-made nutritious drink equivalent to a standard 1.5 KCal/mL prescribed bottle supplement in terms of calories and protein.
 
Nutrison®
(Liquid - tube feed)
Formulary
Amber

First line

  • 1 kcal/mL and less than 5 g protein/100 mL
  • Standard 1 kcal/mL, No fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison®Multi Fibre
(Liquid (sip or tube feed))
Formulary
Amber

First line

  • 1 kcal/mL and less than 5 g protein/100 mL
  • Standard 1 kcal/mL, With fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Jevity®
(Liquid - tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Pt requires 1 kcal/mL vegetarian feed fish oil free (contains fibre)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula except bowel fistula. Not suitable for child under 2 years
 
   
Osmolite®
(Liquid - tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Pt requires 1 kcal/mL, vegetarian feed fish oil free (Nil fibre)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison® Soya
(Liquid - tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Soya protein formula
  • Dietary restriction or intolerance to standard feeds
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison® Soya Multi Fibre
(Liquid - tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Soya protein formula
  • Dietary restriction or intolerance to standard feeds requiring fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Peptamen®
(Liquid -sip or tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Peptide-based formula
  • Where hydrolysate is required
  • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula
 
   
Nutrison Peptisorb®
(Liquid - tube feed)
Formulary
Amber
  • 1 kcal/mL and less than 5 g protein/100 mL
  • Peptide-based formula
  • Pts with malabsorption
  • Short bowel syndrome, intractable malabsoprtion, proven inflammatory bowel disease, bowel fistula.
  • Follow Trust guidance on use of acidic tube feeds
 
   
Elemental 028®Extra
(Liquid -sip or tube feed)
Formulary
Amber
  • Less than 1 kcal/mL and less than 5 g protein/100 mL
  • Amino acid formula (essential and non-essential amino acids)
  • Pts with IBD, multiple allergy
  • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula
 
   
Elemental 028®Extra
(Powder sachets)
Formulary
Amber
  • Less than 1 kcal/mL and less than 5 g protein/100 mL
  • Amino acid formula (essential and non-essential amino acids)
  • Pts with IBD, multiple allergies. For tube feed and oral feeding. Alter concentration of feed to support individual patient’s nutritional needs.
  • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula
 
   
Fresubin®HP Energy
(Liquid - tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Only available product with high protein and high MCT (burns pts, tumour pts, and liver pts.)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula ; also CAPD and haemodialysis
 
   
Fresubin®HP Energy Fibre
(Liquid - tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Only available product with high protein and high MCT with fibre (burns pts, tumour pts, liver pts)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula ; also CAPD and haemodialysis
 
   
Jevity®1.5 kcal
(Liquid - tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • High energy vegetarian feed, fish oil free with fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison®Energy
(Liquid - tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • High energy feed
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison®Energy Multi Fibre
(Liquid - sip or tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • High energy feed with fibre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Osmolite®1.5 kcal
(Liquid - sip or tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • High energy vegetarian fish oil free (Nil fibre)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Resource®Energy
(Liquid - sip feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Paediatrics and specialists adults use only
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Vital®1.5 kcal
(Liquid - sip or tube feed)
Formulary
Amber
  • 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Hydrolysate nutritionally complete in 1 litre
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Jevity®Plus
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Pt requires 1.2kcal/mL, fibre, vegetarian feed fish oil free
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Jevity®Plus HP
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Pt requires 1.3kcal/mL high protein fibre vegetarian as fish oil free
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Jevity®Promote
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Pt requires 1 kcal/mL, fibre, vegetarian feed fish oil free
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison®MCT
(Liquid - tube feed)
Formulary
Red
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Pts with chyle leaks following upper GI surgery
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Nutrison®Protein Plus
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Lower calorie, high protein feed. Fibre free
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula

 

 
   
Nutrison®Protein Plus Multi Fibre
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Lower calorie, high protein feed. Added fibre
 
   
Nutrison®800 Complete Multi Fibre
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • 800 kcal/L, nutritionally complete in 1 litre, low volume
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula except bowel fistula
  • Not suitable for child under 6 years; not recommended for child 6-12 years
 
   
Nutrison®1000 Complete Multi Fibre
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • 1000 kcal/L, nutritionally complete in 1 litre, low volume
  • Disease related malnutrition in patients with low energy and/or low fluid
    requirements
 
   
Nutrison®1200 Complete Multi Fibre
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • 1200 kcal/L, nutritionally complete in 1 litre, low volume
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Osmolite®Plus
(Liquid - tube feed)
Formulary
Amber
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Alternative standard feed, vegetarian fish oil free (1200 kcal/L) (Nil fibre)
  • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula
 
   
Peptamen®AF
(Liquid - tube feed)
Formulary
Red
  • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
  • Used in critical care settings 
  •    
    Peptamen®HN
    (Liquid - tube feed)
    Formulary
    Amber
    • Less than 1.5 kcal/mL and 5g (or more) protein/100 mL
    • Step down from Peptamen® AF
    • Intolerance to other feed
    • Hydrolysate 1300 kcal/L nutritionally complete in 1 litre
    • For patients with malabsorption where requirements (high protein/low volume) cannot be met with a standard 1Kcal/ml MCT peptide feed. Used in liver/hepatobiliary disease/conditions
    • Short bowel syndrome, intractable malabsorption, proven inflammatory bowel disease, bowel fistula. Not suitable for child under 3 years
     
       
    Ensure®Twocal
    (Liquid - sip feed)
    Formulary
    Amber
    • More than 1.5 kcal/mL and 5g (or more) protein/100 mL
    • High calorie vegetarian fish oil free
    • Standard ACBS indications: Disease-related malnutrition, intractable malabsorption, pre-operative preparation of malnourished patients, dysphagia, proven inflammatory bowel disease, following total gastrectomy, short-bowel syndrome, bowel fistula ; also haemodialysis and CAPD
     
       
    Nutrison®Concentrated
    (Liquid )
    Formulary
    Amber
    • More than 1.5 kcal/mL and 5g (or more) protein/100 mL
    • High calorie (2000 kcal/L), low volume, low potassium
    • First line in CF and renal patients
     
       
    Nutrini® Low Energy Multi Fibre
    (Liquid - tube feed)
    Formulary
    Amber

    Child under 12 years

    • Low energy with fibre. 8-20kg
    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • Paediatric ACBS indications: Disease-related malnutrition, intractable malabsorption, growth failure, pre-operative preparation of malnourished patients, dysphagia, short-bowel syndrome, bowel fistula except bowel fistula, in child 1–6 years, body-weight 8–20 kg
     
       
    Nutriprem® 1
    (Liquid - sip feed)
    Formulary
    Red

    Child under 12 years

    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • Low birth - weight formula
    • For premature infant up to 2kg
     
       
    Nutriprem® 2
    (Powder tin)
    Formulary
    Amber

    Child under 12 years

    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • Post discharge formula for pre-term infants <34 weeks to aid growth
    • Catch-up growth in pre-term infants (less than 35 weeks at birth) and small for gestational-age infants up to 6 months corrected age.
     
    Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
       
    Nutriprem® 2
    (Liquid - sip feed)
    Formulary
    Red

    Child under 12 years

    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • Post discharge formula for pre-term infants for immunocompromised infants
    • Catch-up growth in pre-term infants (less than 35 weeks at birth) and small for gestational-age infants up to 6 months corrected age.
     
       
    SMA® PRO Gold Prem 2
    (Powder (sip feed))
    Formulary
    Amber

    Child under 12 years

    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • Post discharge formula for pre term infants <34 weeks to aid growth
    • Powder feed
    • Partially hydrolysed
    • For catch-up growth in preterm infants and small for gestational age babies on discharge from hospital until 6 months corrected age. It may be used as the sole source of nutrition until complementary feeding commences.
     
    Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
       
    SMA® PRO High Energy
    (Liquid - sip feed)
    Formulary
    Amber

    Child under 12 years

    • Less than 1 kcal/mL and less than 4g protein/100 mL
    • High energy feed for faltering growth
    • Single use only in acute setting – risk of increased waste.
    • Once open must be used within 24 hours
    • Disease-related malnutrition, malabsorption, and growth failure in infancy.
     
    Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
       
    Fortini® 1.0 Multi Fibre
    (Liquid - sip feed)
    Formulary
    Amber
    Child under 12 years
  • 1 kcal/mL and less than 4g protein/100 mL
  • Standard 1 kcal/mL with fibre  
  •    
     ....
     Non Formulary Items
    Add-Ins®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Discontinued

     
    Alfamino®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Alicalm®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Althera®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Caloreen®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Discontinued by manufacturer

     
    Calshake®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Caprilon®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    No longer available (as per UHBP)

     
    Clinutren® Dessert

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Clinutren® Dessert

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Colief®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Complan® Shake
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Use Aymes Shake or Foodlink Complete as alternatives

     
    Easiphen®
    (Liquid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Emsogen®
    (Powder (sip feed or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Enfamil® AR
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Enfamil® O-Lac
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure®
    (can)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Compact
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Commence
    (Starter Pack)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Creme
    (Semi-solid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Fibre
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Juce
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Milkshake
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Savoury
    (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Ensure® Plus Yoghurt
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Enteral feeds: non-disease specific

    Non Formulary
    Black
  • Fresubin® Original
  • Fresubin® Original Fibre
  • Fresubin® 1500 Complete
  • Novasource® GI Control
  • Fresubin® Soya Fibre
  • SurvimedFresubin® OPD
  • Fresubin® 2250 Complete
  • Fresubin® Energy
  • Fresubin® Energy Fibre
  • Novasource® GI Forte
  • Fresubin® 1000 Complete
  • Fresubin® 1200 Complete
  • Fresubin® 1800 Complete
  • Perative®
  • Emsogen®
  • Frebini® Original
  • Frebini® Original Fibre




  •  
    Flavour Mix®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Forticare®
    (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Forticreme Complete
    (Semi-solid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Not recommended by SALTs for use as a dysphagia product.
    Low energy per serving as a semi-solid supplement where low volume is required only.

     
    Fortisip® Savoury Multi Fibre

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Discontinued

     
    Frebini® Energy Drink
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Frebini® Energy Fibre
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Frebini® Energy Fibre
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Frebini® Original
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Frebini® Original Fibre
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 1000 Complete
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 1200 Complete
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 1500 Complete
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 1800 Complete
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 2250 Complete
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® 5 kcal Shot

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Creme
    (Semi-solid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Energy
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Energy Fibre
    (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Original
    (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Original Fibre
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Powder
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Protein Energy Drink
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Fresubin® Soya Fibre
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Generaid®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Generaid® Plus
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    HCU LV®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without methionine for children and adults with homocystinuria
    • NOT USED - We have had no success in patients taking this product.
    • Nutritional supplement for the dietary management of hypermethioninaemia or vitamin B6 non-responsive homocystinuria in children over 8 years.
     
    Heparon® Junior
    (Powder)

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    Non Formulary
    Black
     
    HYPER LYS Anamix® Infant
    (Powder)

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    Non Formulary
    Black
    • Amino acid supplement without lysine for infants with proven hyperlysinaemia
    • Rarely used - The only infant amino acid supplement without lysine, for infants with proven hyperlysinaemia.
    • Nutritional supplement for the dietary management of proven hyperlysinaemia in children from birth to 3 years.
     
    Ketocal® 2.5:1  (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Amber
    • ACBS indication: Enteral feed or nutritional supplement as part of ketogenic diet in management of drug resistant epilepsy or other conditions for which a ketogenic diet is indicated in children 1–10 years; as a nutritional supplement in children over 10 years. 
     
    Keyo®  (semi-solid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Non-formulary November 2018

     
    Kindergen®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Non-formulary September 2019

    • Specialised formulas for specific clinical conditions
    • Used in infants and children with renal disease when low potassium and low phosphate feed is required, can be given orally or as a tube feed
    • Enteral feed or nutritional supplement for children with chronic renal failure receiving peritoneal rapid overnight dialysis.
     
    Leucine 100®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Low protein drink
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    L-Tyrosine
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Lysine 4000 (not ACBS approved)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    MCT Duocal®

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    Non Formulary
    Black
     
    MCT Pepdite®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Methionine-free TYR Anamix® Infant
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Milupa PKU 1-prima®

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    Non Formulary
    Black
     
    Milupa PKU 2-Seconda®

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    Non Formulary
    Black
     
    Milupa PKU 3-advanta®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Multi-thick®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Neocate® Active
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black

    Discontinued – April 2018 (replaced by Neocate Junior)

     
    Neocate® Advance
    (Powder)

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    Non Formulary
    Black

    Discontinued – April 2018 (replaced by Neocate Junior)

     
    Neocate® Spoon
    (Powder)

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    Non Formulary
    Black
     
    Novasource® GI Control
    (Liquid (tube feed))

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    Non Formulary
    Black
     
    Novasource® GI Forte

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    Non Formulary
    Black
     
    Nutilis® Complete Stage 2
    (Semi-solid)

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    Non Formulary
    Black
    • More than 1.5 kcal/mL and 5 g (or more) protein/100 mL
    • Not recommended by SALTs for use as a dysphagia product.
      Low energy per serving as a semi-solid supplement where low volume is required only.
     
    Nutilis® Complete Stage 2
    (Semi-solid)

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    Non Formulary
    Black
     
    Nutilis® Fruit Stage 3
    (Semi-solid)

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    Non Formulary
    Black
     
    Nutilis® Powder
    (Powder)

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    Non Formulary
    Black
     
    Oral Impact®
    (Powder)

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    Non Formulary
    Black
     
    Paediasure® Fibre
    (Liquid (sip or tube feed))

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    Non Formulary
    Black
     
    Peptide®
    (Powder)

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    Non Formulary
    Black
     
    Peptide® 1+
    (Powder)

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    Non Formulary
    Black
     
    Perative®
    (Liquid (tube feed))

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    Non Formulary
    Black
     
    Phenylalanine50®
    (Powder)

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    Non Formulary
    Black
     
    Phlexy-10® Exchange System
    (Capsule)

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    Non Formulary
    Black
     
    PK Aid-4®
    (Powder)

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    Non Formulary
    Black
     
    PKU Anamix® Junior
    (Powder)

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    Non Formulary
    Black
     
    PKU Anamix® Junior LQ
    (Liquid)

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    Non Formulary
    Black
     
    PKU gel®
    (Powder)

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    Non Formulary
    Black

    Replaced by PKU Explore - September 2019

     

    • A supplement without phenylalanine for children with phenylketonuria
    • One of 2 powdered weaning (thickened) amino acid supplement without phenylalanine for infants/young children with phenylketonuria. The consistency can be adjusted to suit the developmental age of the older baby/toddler. This was our product of choice 5 years ago. However, we have also older patients taking this protein substitute who refuse to change. Children with PKU are very neophobic about their protein substitutes and do not change easily. Without protein substitute it would cause low IQ and executive issues.
    • For use as part of the low-protein dietary management of phenylketonuria in children 1–10 years
     
    PKU Lophlex® Sensation 20
    (Semi-solid)

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    Non Formulary
    Black
     
    PKU Squeezie®
    (Liquid)

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    Non Formulary
    Black
     
    Pregestimil® Lipil
    (Powder)

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    Non Formulary
    Black
     
    Preparations  (Forceval Protein Powder®)

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    Non Formulary
     
    Pro-Cal®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    ProSure®
    (Liquid (sip or tube feed))

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    Non Formulary
    Black
     
    ProvideXtra® Juice Drink

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    Non Formulary
    Black
     
    Renamil®
    (Powder (sip feed or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® 2.0 Fibre
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® Desser Fruit

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® Dessert Energy
    (Semi-solid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® Fruit
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® Protein

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® Thickened Drink
    (Liquid)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Resource® ThickenUp Clear
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Respifor®
    (Liquid (sip feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Sapropterin dihydrochloride 100mg dispersible tablets

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Similac® High Energy
    (Liquid (sip or tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    SMA® Staydown
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Suplena®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Supportan®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Survimed® OPD
    (Liquid (tube feed))

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Thick and Easy®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Thicken Aid®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Tyrosine 1000®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Vegenat® -med Balanced Protein
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Vegenat® -med High Protein
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Vitajoule®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Vitapro®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    Vitaquick®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    VSL#3®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XLYS Maxamaid® (1)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without lysine for children with proven hyperlysinaemia
    • Rarely used - The only child amino acid supplement without lysine, for children with proven hyperlysinaemia..
    • Nutritional supplement for the dietary management of hyperlysinaemia.
     
    XLYS, Low TRY, Maxamaid® (1)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without lysine – low in tryptophan for infants with GA1
    • NOT USED - This is an old product and patients will not adhere to taking it. We have a long history of trying.
    • Nutritional supplement for the dietary management of type 1 glutaric aciduria.
     
    XLYS, TRY Glutaridon®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without lysine – low in tryptophan for infants with GA1 on emergency regimes
    • NOT USED - Unmeasured. Difficult to measure accurately.
    • Nutritional supplement for the dietary management of type 1 glutaric aciduria in children and adults; requires additional source of vitamins, minerals, and trace elements.
     
    XMET Homidon®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without methionine for children and adults with homocystinuria
    • NOT USED - This is an old product and is unacceptable to patients. They are unable to take it.
    • Nutritional supplement for the dietary management of hypermethioninaemia or homocystinuria in children and adults.
     
    XMET Maxamaid® (1)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without methionine for children and adults with homocystinuria
    • NOT USED - This is an old product and patients will not adhere to taking it. We have a long history of trying.
    • Nutritional supplement for the dietary management of hypermethioninaemia or homocystinuria.
     
    XMET Maxamum® (2)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without methionine for children and adults with homocystinuria
    • NOT USED - This is an old product and patients will not adhere to taking it. We have a long history of trying.
    • Nutritional supplement for the dietary management of hypermethioninaemia or homocystinuria.
     
    XMTVI Asadon®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    • Amino acid supplement without methionine, threonine, valine and isoleucine for infants with propionic acidaemia and methyl malonic acidaemia.
    • NOT USED - This is difficult to measure out with accuracy and is no longer used. We rarely use methionine, valine, threonine and isoleucine free L-amino acid supplements for children over 1 year of age with MMA/PA.
    • Nutritional supplement for the dietary management of methylmalonic acidaemia or propionic acidaemia in children and adults.
     
    XMTVI mAXAMAID® (1)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XMTVI Maxamum® (2)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XP Maxamaid® (2)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XP Maxamum®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XPHEN TYR Maxamaid® (1)
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XPHEN TYR Maxamum®

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XPHEN TYR Tyrosidon®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
    XPTM Tyrosidon®
    (Powder)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
     
      
    Key
    note Notes
    Section Title Section Title (top level)
    Section Title Section Title (sub level)
    First Choice Item First Choice item
    Non Formulary Item Non Formulary section
    Restricted Drug
    Restricted Drug
    Unlicensed Drug
    Unlicensed
    Track Changes
    Display tracking information
    click to search medicines.org.uk
    Link to adult BNF
    click to search medicines.org.uk
    Link to children's BNF
    click to search medicines.org.uk
    Link to SPCs
    Cytotoxic Drug
    Cytotoxic Drug
    CD
    Controlled Drug
    High Cost Medicine
    High Cost Medicine
    Cancer Drugs Fund
    Cancer Drugs Fund
    NHSE
    NHS England
    Homecare
    Homecare
    CCG
    CCG

    Traffic Light Status Information

    Status Description

    Prescribing in children

    The APC notes that the informed use of unlicensed medicines or of licensed medicines for unlicensed applications (‘off-label’ use) is often necessary in paediatric practice.

    The APC advises GPs to consider specialist prescribing recommendations for Green and Amber medicines that are not subject to ESCAs or RICaDs in combination with the information provided in the BNFC which goes beyond that of marketing authorisations. The BNFC has been designed for rapid reference and the information presented has been carefully selected to aid decisions on prescribing.

      

    Green

    Medicines which are suitable for initiation and maintenance prescribing by primary and secondary care clinicians. These medicines should be initiated and prescribed within their licensed indications.  

    Amber

    Initiation and maintenance of prescribing by Specialists and transfer to Primary Care prescribing when appropriate, or initiation and maintenance of prescribing in Primary Care following recommendation from a Specialist.

    Some amber medicines require agreement with the local (internal) medicines committee prior to initiation; others may require a framework to support safe transfer and maintenance of care such as a RICaD or ESCA. The Formulary will be annotated to reflect these requirements.   

    Red

    Medicines for initiation and maintenance prescribing by Specialists only  

    Black

    Non-formulary medicines- medicines not recommended for routine primary care prescribing.  

    Grey

    Positive NICE TA and /or awaiting local clarification on place in therapy ; Please contact your Medicines Optimisation team for more information.  

    netFormulary