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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Nitisinone
Formulary

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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
Link  NICE HST29: Velmanase alfa for treating alpha-mannosidosis

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Chenodexoycholic acid Xenbilox®
Formulary

  • 250mg capsules


    • 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)

    • Approved for the treatment of inborn errors of bile acid synthesis (all ages) in line with NHS England Commissioning Policy




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