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

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Electrolyte Guideline in Emergencies Handbook

Use intravenous fluids consistent with NICE guidelines: (summary algorithms adult, children).  As most intravenous fluid use is for maintenance, remember key principles such as in adults’ adequate glucose (dextrose) in each 24 hour period to prevent ketosis and choice of fluids to prevent hyponatraemia (especially in children) and hypernatraemia. 

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09.08.01  Expand sub section  Drugs used in metabolic disorders
09.08.01  Expand sub section  Wilsons disease
Penicillamine
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Restricted Drug Restricted
Amber 1
Tablets
 
   
09.08.01  Expand sub section  Carnitine deficiency
 note 
Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
Carnitine (Carnitor®)
(levocarnitine)
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Restricted Drug Restricted
RedRed
NHS status commissioned by NHS England for canitine deficiency via highly specialised service criteria monitored by database
  • PBR excluded drug, no paperwork required as suitable for shared care with primary care.
  • Also known as levocarnitine

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded) 
  •    
    09.08.01  Expand sub section  Fabry's disease
     note 
    Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
    09.08.01  Expand sub section  Gaucher's disease to top
     note 
    Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
    09.08.01  Expand sub section  Mucopolysaccharidosis I
     note 
    Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
    09.08.01  Expand sub section  Pompe disease
     note 
    Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
    09.08.01  Expand sub section  Nephropathic cystinosis
    09.08.01  Expand sub section  Urea cycle disorders
     note 
    Star All products in this section are high cost drugs reimbursed separately from NHS England tariff (PBR excluded)
     ....
     Non Formulary Items
    Agalsidase Alfa and Beta  (Fabrazyme®)

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    Non Formulary
    RedRed
    NHS status Fabry's disease Specialist centre only, NHS England commissioned

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Agalsidase Alfa and Beta  (Replagal®)

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    Non Formulary
    RedRed
    NHS status Fabry's disease Specialist centre only, NHS England commissioned

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Alglucosidase Alfa  (Myozyme®)

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    Non Formulary
    RedRed
    NHS status Pompe disease: NHS England specialised centre only

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Alipogene Tiparvovec

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Black
    Lipoprotein lipase deficiency
    NHS status  Access only via NHS England IFR via specialised centre

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Arginine

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    Non Formulary
     
    Betaine
    (trimethylglycine)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England commissioned specialised service only in homocystinuria with highly specialised criteria and prescribing


    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Biotin

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    Non Formulary
     
    Carglumic Acid  (Carbaglu®)

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    Non Formulary
    RedRed
    NHS status commissioned by NHS England for urea cycle disorders via highly specialised service criteria

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Eliglustat  (Cerdelga®)
    (Gaucher's disease )

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England commissioned specialised service only.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Elosulfase alfa
    (Mucopolysaccharidosis IVA)

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    Non Formulary
    Black
    NHS status Not routinely commissioned by NHS England

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Galsulfase  (Naglazyme®)
    (mucopolysaccharidosis VI [MPS VI])

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England commissioned specialised service only.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Idursulfase  (Elaprase®)
    (Hunter syndrome, MPS-II, mucopolysaccharidosis-II)

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    Non Formulary
    RedRed

    Black triangle drugNHS continuation

    • NHS England specialised commissioned service only.

      Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Imiglucerase  (Cerezyme®)
    (Gaucher's disease)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England specialised commissioned service only

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Laronidase  (Aldurazyme®)
    (Mucopolysaccharidosis type 1, MPS I, Hurler syndrome, Hurler-Scheie syndrome, Scheie syndrome)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England specialised commissioned service only

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Mercaptamine  (Cystagon®)
    (Nephropathic cystinosis)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England specialised commissioned service only.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Migalastat  (Amigal® )
    (Fabry's disease)

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    Non Formulary
    RedRed

    Black triangle drugNHS continuation

    • NHS England specialised commissioned service only

      Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Miglustat  (Zavesca®)
    (Gaucher's disease/ Niemann-Pick)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England specialised commissioned service only.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Nitisinone  (Orfadin®)
    (Tyrosinaemia)

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    Non Formulary
    RedRed
    NHS continuation
  • NHS England specialised commissioned service only.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Sebelipase alfa
    (Recombinant human lysosomal acid lipase, SBC-102)

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    Non Formulary
    Black

    Black triangle drugNHS status Not routinely commissioned by NHS England for lysosomal acid lipase deficiency.

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)

     
    Sodium Benzoate

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    Non Formulary
     
    Sodium Dichloroacetate

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    Non Formulary
     
    Sodium Phenybutyrate  (Ammonaps®)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
     
    Taliglucerase alfa  (Elelyso®)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    RedRed
    Unlicensed 
    NHS continuation
  • NHS England specialised commissioned service only

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Tetrahydrobiopterin
    (Phenylketonuria, hyperphenylalaninemia)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    RedRed
    NHS continuation
  • For use by the NHS England specialist commissioned service only

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
  •  
    Trientine Dihydrochloride

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    Link  SSC 2019 letter Trientine for Wilson Disease NHS England Reference 170094P.pdf
     
    Ubidecarenone

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    Non Formulary
     
    Velaglucerase alfa  (VPRIV®)
    (Gaucher's Disease)

    View adult BNF View SPC online View childrens BNF
    Non Formulary
    RedRed
    NHS continuationNHS England will fund use in type 1 Gaucher's Disease consistent with specialised service specification criteria

    Star High cost drug reimbursed separately from NHS England tariff (PBR excluded)
     
    Zinc Acetate  (Wilzin®)

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

    Traffic Light Status Information

    Status Description

    Amber

    Requires to be initiated by specialist for repeat GP Prescribing (Purple as per Eclipse East Kent Primary Care Formulary, may be top sliced) If a downloadable EKPG Prescribing Information Sheet exists in the formulary entry for the drug, it, or an URL (shortcut to webpage/document) should be sent to the GP. See also "Hints in how to incorporate documents for medicines information into clinical communications to GPs".  

    Amber 1

    Shared Care (Orange as per Eclipse East Kent Primary Care Formulary). See the Principles of Shared Care Agreements and Shared Care Agreements: Assessment against agreed Principles of Shared Care documents. If a downloadable template document exists in the formulary entry for this drug, it should be sent to the GP (and patient) once patient specific details are incorporated. See "Hints in how to incorporate shared care documents for medicines into clinical communications to GPs".  

    Amber 2

    Shared Care and topsliced (Orange as per Eclipse East Kent Primary Care Formulary) See the Principles of Shared Care Agreements and Shared Care Agreements: Assessment against agreed Principles of Shared Care documents. If a downloadable template document exists in the formulary entry for this drug, it should be sent to the GP (and patient) once patient specific details are incorporated. See "Hints in how to incorporate shared care documents for medicines into clinical communications to GPs".  

    Black

    A decision has been made by either or both the local or national NHS not to routinely commission this preparation for its licensed indications  

    Blue

    No comment on drug in East Kent Primary Care Formulary (Blue as per Eclipse East Kent Primary Care Formulary)  

    Brown

    Exceptional Use Only (Brown as per Eclipse East Kent Primary Care Formulary)   

    Green

    On East Kent Primary Care Formulary (Pink or Green as per Eclipse East Kent Primary Care Formulary)  

    Grey

    Not assessed/Not recommended (As per Eclipse East Kent Primary Care Formulary)  

    Red

    Hospital Only (As per Eclipse East Kent Primary Care Formulary)  

    RedRed

    Known to be commissioned for local NHS patients but only available to them from another provider than EKHUFT  

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