Cambridgeshire and Peterborough
Formulary
 
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9 Nutrition and blood
09-08-01 Drugs used in metabolic disorders

Asfotase alfa Strensiq®
Restricted

  • CUHFT only via Homecare - in line with NICE HST6 for treating paediatric-onset hypophosphatasia.

Link  NICE HST6: Asfotase alfa for treating paediatric-onset hypophosphatasia
 

Restricted Drug Red Hospital View adult BNF  View SPC online  View childrens BNF
Biotin
Restricted

Tablets Injection


Restricted Drug Red Hospital View adult BNF  View SPC online  View childrens BNF
Glycerol Phenylbutyrate Ravicti®
Restricted

  • CUHFT only - for urea cycle disorders. 


Restricted Drug Red Hospital View adult BNF  View SPC online  View childrens BNF
Nitisinone Orfadin®
Formulary

 

    • CUHFT only: Indicated for alkaptonuria and tyrosinaemia. 

 


Red Hospital View adult BNF  View SPC online  View childrens BNF
Ubidecarenone
Formulary

  • CUHFT only - Available as: -Capsules 10 mg, 30mg, 60mg -Oral Solution 50 mg in 5mL (Ubidecarenone & Vitamin E). Unlicensed medicine - named patient use only. Also known as ubiquinone and co-enzyme Q10. For paediatric metabolic disorders on specialist advice only. 60mg caps (Solgar vitamins) contain powder which can be dispersed in water 

  • Non formulary at all other trusts and in Primary Care


 


Red Hospital View adult BNF  View SPC online  View childrens BNF
Eliglustat Cerdelga®
Restricted

  • CUHFT only -  in line with NICE HST5 for treating type 1 Gaucher disease. 

Link  NICE HST5: Eliglustat for treating type 1 Gaucher disease
 

Restricted Drug Red Hospital View adult BNF  View SPC online  View childrens BNF  HCD
Sodium Dichloroacetate
Non Formulary
Powder
  • Unlicensed special, named patient use only
  • Ongoing manufacturing problem, Jan 2011
  • Available for pyruvate dehydrogenase deficiency

  • Non Formulary