| Formulary Chapter 9: Nutrition and blood - Full Chapter
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| Chapter Links... |
 County Durham and Tees Valley Guidelines for recognition and management of non- IgE cow’s milk allergy in children |
| Guidance for the Prescribing of Vitamins and Minerals in Primary Care in County Durham and Tees Valley |
 North of Tyne, Gateshead and North Cumbria - Recommendations for symptom management in renal patients (including symptom management at the end of life) |
| South Tees Pathway for the Management of Undernutrition (ADULTS) |
| South Tees Pathway for the Management of Undernutrition (Nursing/Care Homes) |
| Details... |
| 09.08.01 |
Drugs used in metabolic disorders |
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Asfotase alfa (Strensiq®)
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Formulary
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- 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
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NICE HST23: Asfotase alfa for treating paediatric-onset hypophosphatasia
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Betaine
(Homocystinuria)
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Formulary
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Calcium Levomefolate (Prefolic)
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Formulary
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Copper histidinate
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Formulary
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- 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
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NHSE Specialised Commissioning Policy 2577: Subcutaneous copper histidinate injections for presymptomatic neonates with classical Menkes disease
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Nitisinone
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Formulary
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Sebelipase alfa (Kanuma®)
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Formulary
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- 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
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NICE HST30: Sebelipase alfa for treating Wolman disease
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Ubiquinone (Coenzyme Q10, Ubidecarenone)
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Formulary
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- 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
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Velmanase alfa (Lamzede ®)
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Formulary
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- 10mg powder for solution for infusion
- Approved for treating alpha-mannosidosis in line with NICE and NHS England Specialised Commissioning Policy
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NICE HST29: Velmanase alfa for treating alpha-mannosidosis
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Chenodexoycholic acid (Xenbilox®)
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Formulary
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- 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)
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| 09.08.01 |
Wilsons disease |
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Penicillamine
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Formulary
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- 125mg & 250mg tablets - also used for the treatment ofcystinuria and rheumatoid arthritis – section 10.1.3
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Zinc Acetate (Wilzin®)
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Formulary
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Trientine Dihydrochloride
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Formulary
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- 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)
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Tiopronin
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 Unlicensed
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- 500mg tablets - for use as 2nd line treatment for cystinuria in
patients who fail to tolerate/respond to penicillamine unlicensed.
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| 09.08.01 |
Carnitine deficiency |
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Carnitine
(Levocarnitine)
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Formulary
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- 30% oral solution
- 1g in 5ml injection
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| 09.08.01 |
Fabry's disease |
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Agalsidase Alfa
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Formulary
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Agalsidase Beta
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Formulary
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Migalastat (Galafold ®)
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Formulary
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- Approved for the treatment of Fabry's disease in line with NICE
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Highly specialised technologies guidance HST4: Migalastat for treating Fabry disease
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Pegunigalsidase alfa (Elfabrio®)
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Formulary
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- 20mg/10ml concentrate for solution for infusion
- Approved for treating Fabry disease in line with NICE and NHSE Specialised Commissioning guidance
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NICE TA915: Pegunigalsidase alfa for treating Fabry’s disease
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| 09.08.01 |
Gaucher's disease |
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Imiglucerase (Cerezyme®)
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Formulary
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Velaglucerase
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Formulary
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| 09.08.01 |
Mucopolysaccharidosis I |
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Elosulfase alfa
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Formulary
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- 5mg/5ml concentrate for solution for infusion:
- treating mucopolysaccharidosis type 4A in people of all ages
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NICE HST19 - Elosulfase alfa for treating mucopolysaccharidosis type 4A
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Galsulfase (Naglazyme®)
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Formulary
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Idursulfase
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Formulary
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Laronidase (Aldurazyme®)
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Formulary
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| 09.08.01 |
Pompe disease |
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Alglucosidase Alfa (Myozyme®)
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Formulary
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Avalglucosidase alfa (Nexviadyme®)
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Formulary
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- 100mg powder for concentrate for solution for infusion
- Approved for treating Pompe disease in line with NICE
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NICE TA821: Avalglucosidase alfa for treating Pompe disease
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Cipaglucosidase alfa (CIPA) (Pombiliti®)
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Formulary
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- 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
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NICE TA912: Cipaglucosidase alfa with miglustat for treating late-onset Pompe disease
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| 09.08.01 |
Nephropathic cystinosis |
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Mercaptamine
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Formulary
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| 09.08.01 |
Urea cycle disorders |
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Arginine
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Formulary
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Carglumic Acid (Carbaglu®)
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Formulary
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Sodium Benzoate
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Formulary
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Sodium Phenylbutyrate
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Unlicensed
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- 500mg tablets - Licensed formulation marketed as Ammonaps
- 1g in 5ml & 2g in 10ml injections
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| Non Formulary Items |
Sebelipase alfa (Kanuma®)
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Non Formulary
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- For treating lysosomal acid lipase deficiency that is not Wolman disease - terminated appraisal
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NICE TA961: Sebelipase alfa for treating lysosomal acid lipase deficiency that is not Wolman disease (terminated appraisal)
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Key |
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Cytotoxic Drug
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Controlled Drug
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High Cost Medicine
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NHS England |
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Homecare |
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CCG |
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Traffic Light Status Information
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Description |
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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. |
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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. |
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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. |
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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. |
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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. |
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UNDER REVIEW: drugs whose current formulary status or RAG status is currently under review. |
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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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