North East and North Cumbria
ICS Formulary
 
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9 Nutrition and blood
09-08-01 Drugs used in metabolic disorders

Asfotase alfa Strensiq®
Formulary
  • 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

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Betaine
Formulary

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Calcium Levomefolate Prefolic
Formulary

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Copper histidinate
Formulary
  • 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

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Nitisinone
Formulary

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Sebelipase alfa Kanuma®
Formulary
  • 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

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Ubiquinone Coenzyme Q10, Ubidecarenone
Formulary
  • 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.

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Velmanase alfa Lamzede®
Formulary
  • 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

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Chenodexoycholic acid Xenbilox®
Formulary
  • 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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Sebelipase alfa Kanuma®
Non Formulary
  • 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)

Not Recomended