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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...
09.01  Anaemias and some other blood disorders
09.01.01  Iron-deficiency anaemias
09.01.01.01  Oral iron
Ferrous Fumarate tablets
View adult BNF View SPC online View childrens BNF
First Choice
Green
 
Ferrous Fumarate liquid (Fersamal®)
View adult BNF View SPC online
Formulary
Green
 
   
Ferrous Gluconate
View adult BNF View SPC online View childrens BNF
Formulary
Green
  • If patient is intolerant of ferrous sulphate 
  •    
    Ferrous Sulphate
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Sodium Feredetate SF Elixir (Sytron®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Ferric maltol (Feraccru®)
    (30mg hard capsule)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • Approved on formulary December 2017
    • For the treatment of iron deficiency anaemia (IDA) in patients with inflammatory bowel disease (IBD), unable to tolerate other oral iron formulations.
    • Supported by a RICaD
     
    Link  Ferric maltol (Feraccru) RICaD
       
    09.01.01.01  Iron and folic acid
    Iron and Folic Acid (Pregaday®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    09.01.01.01  Compound iron preparations to top
    09.01.01.02  Parenteral iron
    Ferric Carboxymaltose (Ferinject®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    Hospital only 
    Link  Drug safety update: Intravenous iron and serious hypersensitivity reactions: strengthened recommendations
       
    Iron Isomaltoside (Monofer®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    Hospital only  
    Link  Drug safety update: Intravenous iron and serious hypersensitivity reactions: strengthened recommendations
       
    Iron Sucrose (Venofer®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    Hospital use only 
    Link  Drug safety update: Intravenous iron and serious hypersensitivity reactions: strengthened recommendations
       
    09.01.02  Drugs used in megaloblastic anaemias
    Folic Acid
    View adult BNF View SPC online View childrens BNF
    Formulary Green Traffic Light  Tablets
    Amber Traffic Light  SF solution- Lexpec® 
       
    Hydroxocobalamin 1mg/ml (injection)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    09.01.03  Drugs used in hypoplastic, haemolytic, and renal anaemias
    Hydroxycarbamide
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • For use in sickle cell 
  •    
    09.01.03  Erythropoietin
    Darbepoetin Alfa (Aranesp®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned for use in renal dialysis
  • Primary Care commissioned for use in cancer- in line with NICE TA323
     
  •    
    Epoetin beta (NeoRecormon®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned for use in renal dialysis
  • Primary Care commissioned for use in cancer- in line with NICE TA323
     
  •    
    Methoxy Polyethylene Glycol-Epoetin Beta (Mircera®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned for use in renal dialysis
  • Primary Care commissioned for use in cancer- in line with NICE TA323
     
  •    
    09.01.03  Iron overload to top
    Deferasirox (Exjade®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned 
  •    
    Deferiprone (Ferriprox®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned  
  •    
    Desferrioxamine Mesilate
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned 
  •    
    09.01.04  Drugs used in autoimmune thrombocytopenic purpura
    Anagrelide (Xagrid®)
    (capsules)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Eltrombopag (Revolade®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
    Link  NICE TA293: Eltrombopag for treating chronic immune (idiopathic) thrombocytopenic purpura (review of technology appraisal 205)
       
    Romiplostim (Nplate®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
    Link  NICE TA221: Thrombocytopenic purpura - romiplostim
       
    09.01.05  G6PD deficiency
    09.01.06  Drugs used in neutropenia
    Filgrastim
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
  • Hospital only 
  •    
    Lenograstim
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
  • Hospital only 
  •    
    Pegfilgrastim
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
  • Hospital only. 
  •    
    09.01.07  Drugs used to mobilise stem cells
    09.02  Fluids and electrolytes to top
    09.02.01  Oral preparations for fluid and electrolyte imbalance
    09.02.01.01  Oral potassium
    Potassium Chloride efferevescent tablets (Sando-K®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Potassium Chloride MR tablets (Slow-K®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Potassium Chloride SF syrup (Kay-Cee-L®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation 
  •    
    09.02.01.01  Potassium removal
    Polystyrene Sulphonate Resins
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    09.02.01.02  Oral sodium and water
    Sodium Chloride
    View adult BNF View SPC online View childrens BNF
    Formulary


    Green Traffic Light  Sodium chloride M/R Tablets


    Red Traffic Light  Sodium chloride 5mmol solution


    Amber Traffic Light  Sodium chloride 1mmol/mL oral solution. Amber Specialist recommendation

     
       
    09.02.01.02  Oral rehydration therapy (ORT) to top
    Oral Rehydration Salts
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
    Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    09.02.01.03  Oral bicarbonate
    Sodium Bicarbonate capsules
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation 
  •    
    09.02.02  Parenteral preparations for fluid and electrolyte imbalance
    09.02.02.01  Electrolytes and water
    09.02.02.01  Intravenous sodium
    09.02.02.01  Intravenous glucose to top
    09.02.02.01  Intravenous potassium
    09.02.02.01  Bicarbonate and lactate
    09.02.02.01  Water
    Water for Injection
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    09.02.02.02  Plasma and plasma substitutes
    09.02.02.02  Plasma substitutes to top
    Gelatin (Gelofusine®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red

    For Endoscopy use only

     
       
    09.03  Intravenous nutrition
    09.03  Supplementary preparations
    09.04  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))
    View adult BNF View SPC online View childrens BNF
    First Choice
    Green

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

     
    Foodlink® Complete
    (Powder)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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®
    View adult BNF View SPC online View childrens BNF
    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
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • Special additives for conditions of intolerance
    • Vitamin and mineral deficiency and as adjunct in synthetic diets
     
       
    Fortijuce®
    (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 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)
    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
    • 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
    Amber
    • 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.
     
       
    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® 3:1
    (Powder)
    View adult BNF View SPC online View childrens BNF
    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.
     
       
    Kindergen®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    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
    • Enteral feed or nutritional supplement for children with chronic renal failure receiving peritoneal rapid overnight dialysis.
     
       
    Lipistart®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    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
    • 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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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
     
       
    Polycal®
    (Liquid)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    • Hospital use only. For patients with high protein needs, low phosphate requirements.
    • Used for renal patients.
    • Used in liver/hepatobiliary diseases/conditions
    • Dietitians to review and consider Renapro powder prior to discharge.
     
       
    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.
     
       
    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.
     
       
    Renilon® 7.5
    (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
    • 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))
    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
    • 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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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))
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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  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)
    View adult BNF View SPC online View childrens BNF
    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  Glutaric aciduria (type 1) to top
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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  Glycogen storage disease
    Corn flour and corn starch
    (Powder)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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  Homocystinuria or hypermethioninaemia
     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)
    View adult BNF View SPC online View childrens BNF
    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)
    View adult BNF View SPC online View childrens BNF
    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  Hyperlysinaemia
    09.04  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
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    • 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  Maple syrup urine disease to top
     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
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    • 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
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    • 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)
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    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  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)
    View adult BNF View SPC online View childrens BNF
    Formulary
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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)
    View adult BNF View SPC online View childrens BNF
    Formulary
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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  Other inborn errors of metabolism
    Cystine500®
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    Formulary
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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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    Formulary
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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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    Formulary
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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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    Formulary
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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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    Formulary
    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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    Formulary
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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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    Formulary
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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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    Formulary
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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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    Formulary
    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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    Formulary
    Amber
    • 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  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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    Formulary
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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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    Formulary
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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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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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    Formulary
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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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    Formulary
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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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    Formulary
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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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    Formulary
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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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    Formulary
    Amber
    • 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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    Formulary
    Amber
    • 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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    Formulary
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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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    Formulary
    Amber
    • 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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    Formulary
    Amber
    • 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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    Formulary
    Amber
    • 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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    Formulary
    Amber
    • 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 gel®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber

    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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber

    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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • For use in the dietary management of phenylketonuria.
    • Amino acid supplement. Without phenylalanine for infants with phenylketonuria.
     
       
    09.04  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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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  ALD to top
    GTO oil
    (Liquid)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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  Other powder feeds
    Meritene energis
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    • Prescribed for liver patients, at request of UHB liver/dietetic team only
    • Liver/hepatobiliary and pancreatic conditions.
    • Hospital supply only.
     
       
    09.04  Rarely Used
    09.04.01  Foods for special diets
    Bread
    (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. 1 slice ordinary bread = 4g protein and is not allowed in low protein diets.

     
       
    Cake, biscuits, and snacks
    (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.

     
       
    Cereals
    (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. 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)
    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.

     
       
    Flour mixes and egg substitutes
    (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. 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  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  
  •    
    09.05  Minerals to top
    09.05.01  Calcium and magnesium
    09.05.01.01  Calcium supplements
    Alliance Calcium Syrup®
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    Formulary
    Amber
  • Replacement for Calcium Sandoz ® (discontinued) 
  •    
    Calcichew®
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
     
       
    Sandocal-1000®
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
     
       
    09.05.01.02  Hypercalcaemia and hypercalciuria
    Cinacalcet (Mimpara®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
  • NHSE commissioned for dialysis only.
  • All prescribing retained by secondary care.  
  • Link  NICE TA117: Hyperparathyroidism - cinacalcet
       
    Etelcalcetide (Parsabiv®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    In line with NICE, NHSE commissioned
    Restricted Item Black Triangle 
    Link  NICE TA448: Etelcalcetide for treating secondary hyperparathyroidism
       
    09.05.01.03  Magnesium
    Magnaspartate ® sachets
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
     
       
    Magnesium citrate tablets
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Magnesium Glycerophosphate oral
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Magnesium Sulphate
    (injection)
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
  • Hospital only 
  • Link  UKMI Q&A: Can magnesium sulfate be given subcutaneously?
    Link  UKMI Q&A: Magnesium sulfate injection: converting between millimoles, milligrams and percentage w/v.
       
    09.05.02  Phosphorus to top
    09.05.02.01  Phosphate supplements
    Joulies Phosphate Solution
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    • paediatrics
     
       
    Phosphate supplements (Phosphate-Sandoz®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Potassium acid phosphate
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    09.05.02.02  Phosphate-binding agents
    Aluminium Hydroxide
    (capsules)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation 
  •    
    Calcium Salts
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation 
  •    
    Lanthanum
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • Specialist Recommendation
    • No ESCA
    • Common Practice
     
       
    Osvaren®
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation 
  •    
    Sevelamer
    (tablets)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
  • No ESCA
  • Common Practice 
  •    
    Sucroferric oxyhydroxide (Velphoro®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    NHS England

    2nd or 3rd line agent

    Specialist use only

     
       
    09.05.03  Fluoride
    Sodium Fluoride Mouthwash, BP
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
  • Dental prescribing only  
  •    
    Sodium Fluoride Toothpaste 0.619% (2800ppm), DPF
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    • Dental prescribing only
     
       
    Sodium Fluoride Toothpaste 1.1% (5000ppm), DPF
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    • Dental prescribing only
     
       
    09.05.04  Zinc
    Zinc Sulphate (Solvazinc®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    09.05.05  Selenium to top
    Selenium capsules/tablets (Selenase®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
     
  •    
    09.06  Vitamins
    09.06.01  Vitamin A
    Vitamins A 150,000 units
    (oral drops)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Vitamins A and D capsules
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
     
  •    
    Vitamins A drops
    (oral drops)
    View adult BNF View SPC online View childrens BNF
    Unlicensed Drug Unlicensed
    Red
  • Paediatric directorate only 
  •    
    09.06.02  Vitamin B group
    Nicotinamide
    (tablets)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    Pyridoxine Hydrochloride
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Riboflavin
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
  • Inherited metabolic disease 
  •    
    Thiamine
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Thiamine (Pabrinex®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    • Secondary care use only
     
       
    09.06.02  Oral vitamin B complex preparations
    Vitamin B Tablets, Compound Strong
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
  • Do Not Use Vitamin B Compund tablets 
  •    
    09.06.02  Other compounds to top
    09.06.03  Vitamin C
    Ascorbic Acid
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    09.06.04  Vitamin D
    Colecalciferol oral
    View adult BNF View SPC online View childrens BNF
    First Choice
    Green
    Please see guidance below to support prescribing

    Please prescribe generically as listed below

    Oral Capsules or Tablets
    Colecalciferol 800 unit capsules
    Colecalciferol 800 unit tablets
    Colecalciferol 1,000unit capsules
    Colecalciferol 1,000unit tablets
    Colecalciferol 3,200unit capsules
    Colecalciferol 4,000unit tablets
    Colecalciferol 20,000unit capsules
    Colecalciferol 25,000unit tablets
    Colecalciferol 40,000unit capsules

    Oral Solution
    Colecalciferol 2,740units/ml oral drops sugar free
    Colecalciferol 10,000units/ml oral drops sugar free
    Colecalciferol 10,000units/ml oral solution sugar free
    Colecalciferol 15,000units/5ml oral solution
    Colecalciferol 25,000units/1ml oral solution unit dose ampoules sugar free
    Colecalciferol 50,000units/1ml oral solution unit dose ampoules sugar free


    Licensed brands currently available (updated March 2017)
    Aviticol 800 IU Capsules
    Aviticol 1,000 IU Capsules (Listed in the Drug Tariff)
    Aviticol 20,000 IU Capsules

    Desunin 800 IU Tablets (Listed in the Drug Tariff)
    Desunin 4,000 IU Tablets (Listed in the Drug Tariff)

    Fultium-D3 800 IU Capsules (Listed in the Drug Tariff)
    Fultium-D3 3,200 IU Capsules (Listed in the Drug Tariff)
    Fultium-D3 20,000 IU Capsules (Listed in the Drug Tariff)
    Fultium-D3 2740 IU/ml oral drops, solution (Listed in the Drug Tariff)

    InVita D3 800 IU Capsules
    InVita D3 2,400 IU/ml oral drops, solution
    InVita D3 25,000 IU oral solution (Listed in the Drug Tariff)
    InVitaD3 50,000 IU oral solution (Listed in the Drug Tariff)

    Plenachol 20,000 IU Capsules
    Plenachol 40,000 IU Capsules (Listed in the Drug Tariff)

    Stexerol-D3 1,000 IU Tablets (Listed in the Drug Tariff)
    Stexerol-D3 25,000 IU Tablets (Listed in the Drug Tariff)

    STRIVIT-D3 800 IU Capsules

    THORENS 10 000 I.U. /ml oral drops, solution (Listed in the Drug Tariff)
    THORENS 25 000 I.U. /2.5 ml oral solution

     
    Calcium and Vit D preparations
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
  • Follow local guidance 
  •    
    Alfacalcidol injection
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
  • Hospital only 
  •    
    Alfacalcidol oral preparations
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Calcitriol
    (capsules)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Ergocalciferol
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    09.06.05  Vitamin E
    Vitamin E Suspension 100mg/ml
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Alpha tocopheryl acetate oral suspension 100mg/ml
  • £££ Specialist Recommendation
     
  •    
    09.06.06  Vitamin K
    Menadiol Sodium Phosphate
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
    Link  UKMI Q&A: What guidance is there available on the use of vitamin K for the management of obstetric cholestasis?
       
    Phytomenadione (Konakion® MM)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
       
    Phytomenadione (Konakion® MM Paediatric)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
    Link  UKMI Q&A: What guidance is there available on the use of vitamin K for the management of obstetric cholestasis?
       
    Phytomenadione 1mg
    (capsule)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
     
       
    09.06.07  Multivitamin preparations to top
    Healthy Start Vitamins
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
  • Available free of charge from designated centres 
  • Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    Multivitamin preparations (Abidec®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
  • contains arachis ( peanut) oil 
  • Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    Multivitamin preparations (Dalivit®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
    Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    Mutivitamins
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
     
    Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    09.06.07  Vitamin and mineral supplements and adjuncts to synthetic diets
    FruitiVits®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • For patients with inborn errors of metabolism and for enteral feed patients.
    • For use in children 3 years and over with inborn errors of metabolism. This is a small volume, flavoured, carbohydrate free vitamin/mineral supplement used in conditions like citrin deficiency. There are no other carbohydrate free preparations available. 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 children 3–10 years with restrictive therapeutic diets
     
       
    Multivitamin Supplement (Paravit-CF®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber

    Capsule, liquid.

    • Specialist initiation or recommendation for GP initiation by specialist in cystic fibrosis.
     
       
    Paediatric Seravit®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
    • 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
     
       
    Renavit®
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
    • For renal patients as per APC formulary
    • Dietary management of water-soluble vitamin deficiency in adults with renal failure on dialysis.
    • Dietary management of water-soluble vitamine deficiency in adults with renal failure on dialysis
     
       
    Vitamin and mineral supplements (Forceval Soluble®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation Check Availability 
  • Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    Vitamin and mineral supplements (Forceval®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
     
  • Link  BSOL CCG: Conditions for which over the counter items (OTC) should not routinely be prescribed in primary care
    Link  Conditions for which over the counter items should not routinely be prescribed in primary care: Guidance for CCGs
       
    09.07  Bitters and tonics
    09.08  Metabolic disorders
    09.08.01  Drugs used in metabolic disorders
    Asfotase alfa
    View adult BNF View SPC online View childrens BNF
    Restricted Drug Restricted
    Red
    High Cost Medicine
    In line with NICE Highly Specialised Technology guidance 
    Link  NICE HST6: Asfotase alfa for treating paediatric-onset hypophosphatasia
       
    09.08.01  Wilsons disease to top
    Penicillamine
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Wilson disease: Specialist Recommendation with ESCA (in development)
     
  •    
    Trientine Dihydrochloride
    View adult BNF View SPC online View childrens BNF
    Formulary
    Amber
  • Specialist Recommendation
     
  •    
    09.08.01  Carnitine deficiency
    09.08.01  Fabry's disease
    09.08.01  Gaucher's disease
    Eliglustat (Cerdelga®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    High Cost Medicine
    In line with NICE Highly Specialised Technology guidance- NHSE commissioned 
    Link  NICE HST5: Eliglustat for treating type 1 Gaucher disease
       
    09.08.01  Mucopolysaccharidosis I
    09.08.01  Pompe disease to top
    09.08.01  Nephropathic cystinosis
    Mercaptamine (Cystagon®)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Red
    • Secondary care use only
     
       
    09.08.01  Urea cycle disorders
    09.08.02  Acute porphyrias
    09.08.02  Drugs unsafe for use in acute porphyrias
    09.09  Feed thickeners and pre-thickened drinks to top
    Instant Carobel®
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green

     

    • Used as a feed thickener for infants with GORD and in children with swallowing issues who require thickened fluids
    • Used in dysphagic patients or those requiring thickened feeds
    • For thickening feeds in the treatment of vomiting
     
    Link  Guidelines for Prescribing Specialist Infant Formula in Primary Care - November 2018 v2
       
    Nutilis® Clear
    (Powder)
    View adult BNF View SPC online View childrens BNF
    Formulary
    Green
    • Recommended by SALTs for patients with dysphagia requiring thickened fluids
    • For thickening of liquids or foods in dysphagia. Not suitable for children under 3 years.
    • Gum based products are generally preferred as they thicken to a more stable end point (unlike starch based products, they do not continue thickening over time) and provide a smoother texture, which improves acceptability and palatability for patients.