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 Formulary Chapter 9: Nutrition and blood - Full Chapter
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09.08.01  Expand sub section  Drugs used in metabolic disorders
Asfotase alfa (Strensiq®)
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Formulary
Red
High Cost Medicine
NHS England
  • 40mg/ml & 100mg/ml solution for injection
  • Approved for the treatment of paediatric-onset and juvenile-onset hypophosphatasia in line with NICE and NHS England Commissioning Policy
 
Link  NICE HST23: Asfotase alfa for treating paediatric-onset hypophosphatasia
 
Betaine
(Homocystinuria)
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Formulary
Red
 
 
Calcium Levomefolate (Prefolic)
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Formulary
Red
 
 
Copper histidinate
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Formulary
Red
  • Subcutaneous copper histidinate injections
  • Approved for presymptomatic neonates with classical Menkes disease in line with NHSE Specialised Commissioning Policy
  • The following providers are commissioned to provide this service within North East:
    • The Newcastle upon Tyne Hospitals NHS Trust
 
Link  NHSE Specialised Commissioning Policy 2577: Subcutaneous copper histidinate injections for presymptomatic neonates with classical Menkes disease
 
Nitisinone
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Formulary
Red
 
 
Sebelipase alfa (Kanuma®)
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Formulary
Red
High Cost Medicine
NHS England
BlueTeq
  • 20mg/10ml concentrate for solution for infusion
  • Approved for long-term enzyme replacement therapy in Wolman disease (rapidly progressive lysosomal acid lipase deficiency), only if people are aged 2 years or under when treatment starts, in line with NICE and NHSE Specialised Commissioning Policy.
  • Services are only commissioned at:
    • Birmingham Women and Childrens NHS Foundation Trust
    • Great Ormond Street Hospital for Children NHS Foundation Trust
    • Manchester University Hospitals NHS Foundation Trust
 
Link  NICE HST30: Sebelipase alfa for treating Wolman disease
 
Ubiquinone (Coenzyme Q10, Ubidecarenone)
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Formulary
Green plus
  • 10mg, 30mg, & 100mg capsules 
  • 50mg in 5ml & 30mg in 1ml (150mg in 5ml) oral solution
    • nutritional supplement and antioxidant approved for use in the
      management of mitochondrial disorders.
  • Approved for use under specialist consultant supervision in the
    management of patients with severe hyperlipidaemia who are not tolerating statins due to myopathy. Use in statin induced myopathy is subject to treatment being initiated by a consultant. 
  • Lipid clinic initiation only
 
 
Velmanase alfa (Lamzede ®)
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Formulary
Red
High Cost Medicine
NHS England
BlueTeq
  • 10mg powder for solution for infusion
  • Approved for treating alpha-mannosidosis in line with NICE and NHS England Specialised Commissioning Policy
 
Link  NICE HST29: Velmanase alfa for treating alpha-mannosidosis
 
Chenodexoycholic acid (Xenbilox®)
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Formulary
Red
High Cost Medicine
  • 250mg capsules
  • Approved for the treatment of inborn errors of bile acid synthesis (all ages) in line with NHS England Commissioning Policy
  • Approved for the treatment of Cerebrotendinous Xanthomatosis in line with NHS Commissioning Policy. NHS England will commission this drug for patients who are currently being treated (April 2017)
 
 
09.08.01  Expand sub section  Wilsons disease
Penicillamine
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Formulary
Red
  • 125mg & 250mg tablets - also used for the treatment ofcystinuria and rheumatoid arthritis – section 10.1.3
 
 
Zinc Acetate (Wilzin®)
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Formulary
Red
 
 
Trientine Dihydrochloride
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Formulary
Red
High Cost Medicine
  • 300mg capsules
    • Approved for the treatment of Wilsons disease in line with NHS England Commissioning Policy. NHS England will commission the treatment for patients who are currently being treated (April 2017)
 
 
Tiopronin
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Unlicensed Drug Unlicensed
Red
  • 500mg tablets - for use as 2nd line treatment for cystinuria in
    patients who fail to tolerate/respond to penicillamine unlicensedunlicensed.
 
 
09.08.01  Expand sub section  Carnitine deficiency
Carnitine
(Levocarnitine)
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Formulary
Red
High Cost Medicine
  • 30% oral solution
  • 1g in 5ml injection
 
 
09.08.01  Expand sub section  Fabry's disease
Agalsidase Alfa
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Formulary
Red
High Cost Medicine
 
 
Agalsidase Beta
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Formulary
Red
High Cost Medicine
 
 
Migalastat (Galafold ®)
Formulary
Red
  • Approved for the treatment of Fabry's disease in line with NICE 
 
Link  Highly specialised technologies guidance HST4: Migalastat for treating Fabry disease
 
Pegunigalsidase alfa (Elfabrio®)
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Formulary
Red
  • 20mg/10ml concentrate for solution for infusion
  • Approved for treating Fabry disease in line with NICE and NHSE Specialised Commissioning guidance
 
Link  NICE TA915: Pegunigalsidase alfa for treating Fabry’s disease
 
09.08.01  Expand sub section  Gaucher's disease to top
Imiglucerase (Cerezyme®)
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Formulary
Red
High Cost Medicine
  • 400iu injection
 
 
Velaglucerase
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Formulary
Red
 
 
09.08.01  Expand sub section  Mucopolysaccharidosis I
Elosulfase alfa
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Formulary
Red
  • 5mg/5ml concentrate for solution for infusion:
    • treating mucopolysaccharidosis type 4A in people of all ages
 
Link  NICE HST19 - Elosulfase alfa for treating mucopolysaccharidosis type 4A
 
Galsulfase (Naglazyme®)
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Formulary
Red
 
 
IdursulfaseBlack Triangle
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Formulary
Red
 
 
Laronidase (Aldurazyme®)
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Formulary
Red
High Cost Medicine
 
 
09.08.01  Expand sub section  Pompe disease
Alglucosidase Alfa (Myozyme®)
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Formulary
Red
High Cost Medicine
 
 
Avalglucosidase alfa (Nexviadyme®)
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Formulary
Red
High Cost Medicine
NHS England
  • 100mg powder for concentrate for solution for infusion
  • Approved for treating Pompe disease in line with NICE
 
Link  NICE TA821: Avalglucosidase alfa for treating Pompe disease
 
Cipaglucosidase alfa (CIPA) (Pombiliti®)
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Formulary
Red
  • 105mg powder for concentrate for solution for infusion vials
  • Approved with with miglustat (Opfolda) for treating late-onset Pompe disease in adults in line with NICE and NHSE Specialised Commissioning guidance


For information only - as there are no providers within the North East & Cumbria area that are commissioned to provide this service

 
Link  NICE TA912: Cipaglucosidase alfa with miglustat for treating late-onset Pompe disease
 
09.08.01  Expand sub section  Nephropathic cystinosis
Mercaptamine
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Formulary
Red
High Cost Medicine
 
 
09.08.01  Expand sub section  Urea cycle disorders
Arginine
View adult BNF View SPC online View childrens BNF
Formulary
Red
 
 
Carglumic Acid (Carbaglu®)
View adult BNF View SPC online View childrens BNF
Formulary
Red
High Cost Medicine
 
 
Sodium Benzoate
Formulary
Red
 
 
Sodium Phenylbutyrate
Unlicensed Drug Unlicensed
Red
  • 500mg tablets - Licensed formulation marketed as Ammonaps
  • 1g in 5ml & 2g in 10ml injections
 
 
 ....
 Non Formulary Items
Sebelipase alfa  (Kanuma®)

Non Formulary
Black
  • For treating lysosomal acid lipase deficiency that is not Wolman disease - terminated appraisal
Link  NICE TA961: Sebelipase alfa for treating lysosomal acid lipase deficiency that is not Wolman disease (terminated appraisal)
  
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
NHSE
NHS England
Homecare
Homecare
CCG
CCG

Traffic Light Status Information

Status Description

Red

Drugs for hospital use only. The responsibility for initiation and monitoring treatment should rest with an appropriate hospital clinician and the drug should be supplied through the hospital throughout the duration of treatment. In some very exceptional circumstances (e.g. due to distance from the hospital, storage, supply or mobility/transport problems) it may be appropriate for the GP to be asked to prescribe a Red drug. This should be negotiated on an individual patient basis and should only be done with the GP’s prior informed agreement where the roles of the GP and hospital services are clearly defined and agreed. The GP should not feel under pressure to prescribe in these circumstances. For all RED drugs automatically added to the formulary in response to a positive NICE TA: Prescribers need to ensure that local Trust new drug governance procedures and pharmacy processes are followed before any prescribing.  

Amber

Drugs initiated by hospital specialist, but where continuing treatment by GPs may be appropriate under a shared care arrangement. The specialist should send the GP a copy of the shared care agreement to sign. The GP should sign the shared care agreement, or indicate they do not want to be part of such an agreement, and return a copy back to the specialist. Shared care guidelines are available or are being developed for most of the drugs listed as Amber. If no shared care guideline is available, the hospital specialist should provide the patient’s GP with sufficient information and support to allow treatment to be continued and managed safely in primary care.  

Green plus

Drugs normally recommended or initiated by a specialist (hospital or GP with an extended role https://www.rcgp.org.uk/gpwer), but can be safely maintained in primary care with very little or no monitoring required. In some cases there may be a further restriction for use outlined - these will be defined in each case. Provision of additional information, or an information leaflet, may be appropriate in some cases to facilitate continuing treatment by GPs.  

Green

Drugs where prescribing by GPs is appropriate. Can be initiated and prescribed in all care settings, and if appropriate, discontinued without recourse to secondary care.  

Black

NOT APPROVED: Drugs that have been considered by NTAG or the NENC ICB Medicines Subcommittee (or other approved body) and are not approved for prescribing within the North East and North Cumbria.   

Brown

UNDER REVIEW: drugs whose current formulary status or RAG status is currently under review.  

Not Recomended

NOT REVIEWED: Drugs that haven not been reviewed yet. This usually means that an application is in progress. These drugs are not normally considered appropriate for prescribing in the North East and North Cumbria until such time that a decision is taken on their formulary status.  

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