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.
  • 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®
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)

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