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 Formulary Chapter 9: Nutrition and blood - Full Chapter
09.08.01  Expand sub section  Drugs used in metabolic disorders
Asfotase alfa (Strensiq®)
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Formulary
Red

For treating paediatric-onset hypophosphatasia as per NICE HST6.

Funded by specialised commissioning.

APCO September 2017.

 
   
Inotersen (Tegsedi®)
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Formulary
Red
High Cost Medicine
NHS England

Secondary care prescribing only for treating hereditary transthyretin amyloidosis as per NICE HST9.

Funded by specialised commissioning.

APCO July 2019.

 
Link  NICE HST9: Inotersen for treating hereditary transthyretin amyloidosis
   
Patisiran (Onpattro®)
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Formulary
Red
NHS England

For treating hereditary transthyretin amyloidosis in line with NICE HST10.

Funded by specialised commissioning. 

APCO September 2019. 

 
Link  NICE HST10: Patisiran for treating hereditary transthyretin amyloidosis
   
Biotin
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Unlicensed Drug Unlicensed
Red

Secondary care use only for children with metabolic disorders.

Prescribed by Paediatric Neurologists only.

APCO September 2013.

 
Link  Medicines for Children leaflet: Biotin for metabolic disorders
   
Tiopronin (Thiola®)
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Unlicensed Drug Unlicensed
Red

Secondary care use only for cystinuria.

APCO September 2018.

 
   
09.08.01  Expand sub section  Wilsons disease
Penicillamine
(Wilson's Disease)
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Formulary
Red

Secondary care use only.

 
   
09.08.01  Expand sub section  Carnitine deficiency
Levocarnitine (Carnitor®)
(Carnitine)
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Formulary
Red
High Cost Medicine

Secondary care use only to potentially reduce the usage of erythropoietin in patients on haemodialysis.

APCO January 2004.

 
   
09.08.01  Expand sub section  Fabry's disease
Migalastat (Galafold®)
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Formulary
Red

For treating Fabry disease as per NICE HST4. To be prescribed by specialist centres only.

APCO March 2017.

Funded by specialised commissioning.

 
Link  NICE HST4: Migalastat for treating Fabry disease
   
09.08.01  Expand sub section  Gaucher's disease to top
09.08.01  Expand sub section  Mucopolysaccharidosis I
09.08.01  Expand sub section  Pompe disease
09.08.01  Expand sub section  Nephropathic cystinosis
09.08.01  Expand sub section  Urea cycle disorders
Arginine
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Unlicensed Drug Unlicensed
Red

For the treatment of Urea Cycle Defects as per BIMDG adult emergency management guideline. 

To be initiated following discussion with Metabolic Medicine or as per patient’s care plan from the national centre.

 
   
Sodium Benzoate
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Unlicensed Drug Unlicensed
Red

For the treatment of Urea Cycle Defects as per BIMDG adult emergency management guideline. 

To be initiated following discussion with Metabolic Medicine or as per patient’s care plan from the national centre.

 
   
 ....
 Non Formulary Items
Agalsidase Alfa and Beta  (Fabrazyme®)

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Non Formulary
High Cost Medicine
 
Agalsidase Alfa and Beta  (Replagal®)

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Non Formulary
High Cost Medicine
 
Alglucosidase Alfa  (Myozyme®)

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Non Formulary
High Cost Medicine
 
Betaine
(Homocystinuria)

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Non Formulary
 
Carglumic Acid  (Carbaglu®)

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Non Formulary
High Cost Medicine
 
Chenodexoycholic acid  (Xenbilox®)

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Non Formulary
 
Eliglustat  (Cerdelga®)

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Non Formulary
High Cost Medicine
 
Galsulfase  (Naglazyme®)

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Non Formulary
 
Glycerol phenylbutyrate  (Ravicti®)

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Non Formulary
 
Imiglucerase  (Cerezyme®)

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Non Formulary
High Cost Medicine
 
Laronidase  (Aldurazyme®)

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Non Formulary
High Cost Medicine
 
Mercaptamine (Cysteamine)  (Cystagon®)

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Non Formulary
High Cost Medicine
 
Miglustat  (Zavesca®)

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Non Formulary
High Cost Medicine
 
Nitisinone

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Non Formulary
 
Sebelipase alfa  (Kanuma®)

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Non Formulary
 
Sodium Dichloroacetate

Non Formulary
 
Sodium Phenylbutyrate

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Non Formulary
 
Trientine Dihydrochloride

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Non Formulary
 
Ubidecarenone

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Non Formulary

Also known as ubiquinone and co-enzyme Q10.

Link  DTB review of co-enzyme Q10 for statin related myopathy
 
Zinc Acetate  (Wilzin®)

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Non Formulary
 
  
Key
note Notes
Section Title Section Title (top level)
Section Title Section Title (sub level)
First Choice Item First Choice item
Non Formulary Item Non Formulary section
Restricted Drug
Restricted Drug
Unlicensed Drug
Unlicensed
Track Changes
Display tracking information
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Link to adult BNF
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Link to children's BNF
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Link to SPCs
SMC
Scottish Medicines Consortium
Cytotoxic Drug
Cytotoxic Drug
CD
Controlled Drug
High Cost Medicine
High Cost Medicine
Cancer Drugs Fund
Cancer Drugs Fund
NHSE
NHS England
Homecare
Homecare
CCG
CCG

Traffic Light Status Information

Status Description

Red

Red: Medicines which should only be prescribed in secondary care by a specialist.  

Amber Continuation

Amber Continuation: Medicines which should be initiated or recommended by a specialist for continuation in primary care. The specialist must notify the GP that the prescribing responsibility has been transferred.   

Amber Shared Care Protocol

Amber Shared Care Protocol: Medicines which are appropriate to be initiated and stabilised by a specialist, once stabilised the medicine may be appropriate for responsibility to be transferred from secondary to primary care with the agreement of a GP and a formal ‘shared care’ agreement. The shared care protocol must be approved by the Area Prescribing Committee Oxfordshire (APCO).   

Green

Green: Medicines which are suitable for initiation and ongoing prescribing within primary care.   

Brown

Brown: Medicines which should only be prescribed in restricted circumstances.  

Black

Black: Medicines which are not recommended for use because of lack of evidence of clinical effectiveness, cost effectiveness or safety.  

OCCG Green

not used  

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