netFormulary Coventry and Warwickshire Area Prescribing Committee NHS
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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
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Formulary
Red SO
Long-term enzyme replacement therapy in patients with paediatric-onset hypophosphatasia
 
   
Betaine
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Formulary
Red SO
Adjunctive treatment of homocystinuria
 
   
Biotin (Vitamin H)
(Unlicensed)
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Formulary
Red SO
Treatment of isolated carboxylase defects and defects of biotin metabolism
 
   
EliglustatRestricted Item
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Formulary
Red SO
Long-term treatment of adult patients with Gaucher disease type 1 (GD1)
 
Link  NICE HST5: Eliglustat for treating type 1 Gaucher disease
   
Nitisinone
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Formulary
Red SO
Treatment of hereditary tyrosinaemia type 1
 
   
Sebelipase alfa
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Formulary
Red SO
Replacement therapy in lysosomal acid lipase deficiency
 
   
Sodium Dichloroacetate
(Unlicensed)
Formulary
Red SO
Treatment of pyruvate dehydrogenase defects
 
   
Ubidecarenone (Ubiquinone, Co-enzyme Q10)
(Unlicensed )
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Formulary
Red SO
Treatment of mitochondrial disorders in children
 
   
09.08.01  Expand sub section  Wilsons disease
Penicillamine
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Amber SC
Treatment of Wilson's disease
Specialist to provide a share care agreement as required 
   
Penicillamine
(children)
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Formulary
Red SO
Treatment of Cystinuria Ė dissolution and prevention of cystine stones in children
 
   
Trientine dihydrochloride
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Formulary
Red SO
Treatment of Wilsonís disease
 
   
Zinc Acetate
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Formulary
Red SO
Treatment of portal hypertension in children
 
   
09.08.01  Expand sub section  Carnitine deficiency
Levocarnitine
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Formulary
Red SO
Treatment of primary and secondary carnitine deficiency
 
   
09.08.01  Expand sub section  Fabry's disease
Agalsidase Alfa
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Formulary
Red SO
Long-term enzyme replacement therapy in Fabry's Disease (α-galactosidase A deficiency)
 
   
Agalsidase Beta
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Formulary
Red SO
Long-term enzyme replacement therapy in patients with Fabry's Disease (α-galactosidase A deficiency)
 
   
09.08.01  Expand sub section  Gaucher's disease to top
Imiglucerase
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Formulary
Red SO
Treatment of Gaucher's disease
 
   
Miglustat
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Formulary
Red SO
Treatment of type 1 Gaucher's disease and Niemann-Pick type C disease
 
   
Velaglucerase alfa
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Formulary
Red SO
Treatment of Gaucherís disease type 1 in children
 
   
09.08.01  Expand sub section  Mucopolysaccharidosis I
Elosulfase alpha
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Formulary
Red SO
Treatment of mucopolysaccharidosis type†IVa
 
   
Galsulfase
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Formulary
Red SO
Treatment of Mucopolysaccharidosis VI
 
   
Idursulfase
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Formulary
Red SO
Treatment of mucopolysaccharidosis II
 
   
Laronidase
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Formulary
Red SO
Treatment of mucopolysaccharidosis 1
 
   
09.08.01  Expand sub section  Pompe disease
Alglucosidase Alfa
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Formulary
Red SO
Long term enzyme replacement therapy in Pompe disease
 
   
09.08.01  Expand sub section  Nephropathic cystinosis
Mercaptamine
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Formulary
Red SO
Treatment of nephropathic cystinosis
 
   
Mercaptamine (cysteamine) eye drops
(Unlicensed)
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Formulary
Red SO
Treatment of cystinosis
 
   
09.08.01  Expand sub section  Urea cycle disorders
Arginine
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Formulary
Red SO
Acute/maintenance treatment of hyperammonaemia in:
carbamylphosphate synthetase deficiency, ornithine carbamyl transferase deficiency, citrullinaemia, arginosuccinic aciduria
 
   
Arginine
(Unlicensed)
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Red SO
Treatment of urea cycle disorders
 
   
Carglumic Acid
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Formulary
Red SO
Treatment of hyperammonaemia
 
   
Sodium Benzoate
(Unlicensed)
Formulary
Red SO
Treatment of hyperammonaemia due to urea cycle disorders and non-ketotic hyperglycinaemia
 
   
Sodium Phenybutyrate
Formulary
Red SO
Adjunct in long-term treatment of urea cycle disorders. It is indicated as adjuvant in all patient with neonatal onset disease and in those with late onset disease who have a history of hyperammonaemic encephalopathy
 
   
 ....
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
click to search medicines.org.uk
Link to adult BNF
click to search medicines.org.uk
Link to children's BNF
click to search medicines.org.uk
Link to SPCs
SMC
Scottish Medicines Consortium
Cytotoxic Drug
Cytotoxic Drug
CD
Controlled Drug
High Cost Medicine
High Cost Medicine
Homecare
Homecare

Traffic Light Status Information

Status Description

Preferred

Clinicians are free to prescribe these drugs. They are regarded as cost effective choices which have evidence to support their use.  

Second Line

These drugs should only be prescribed in the scenarios described in the formulary after preferred drug(s) have been ruled out.  

Non Formulary

Non-formulary choice - the vast majority of a patientís needs should be met by use of formulary preferred choices and second line options when appropriate. Therefore the Clinician should only use a non-formularly drug if this can be justified and it is recommended that all such instances are recorded in the patient record.  

Green SA

Specialist Advised Ė Specialists may simply advise a patientís GP to initiate these drugs themselves after they have made an initial assessment. Note: SA drugs can be initiated by the specialist according to patient need and also depending on local commissioning arrangements which may mandate, through the contract between provider and commissioner, that all SA drugs are treated as SI.  

Green SI

Specialist Initiation - These drugs must be initiated, i.e. the first dose prescribed, by the specialist and then may be continued when appropriate by the patientís GP following communication from the specialist.  

Amber SC

Shared Care - Responsibility for prescribing may be transferred from secondary to primary care with the agreement of an individual GP and when agreed shared care arrangements have been established. The specialist MUST stabilize the patient before asking for care to be transferred. Only specialists should initiate these drugs. Prescribing should be transferred to GPs according to an Shared Care Agreement [SCA]   

Red SO

Specialist Only - These drugs are deemed to be not appropriate for prescribing by GPs. Specialists should not ask GPs to prescribe these drugs.   

Black BL

Black Listed Medicines - These drugs are considered inappropriate for prescribing in Coventry & Warwickshire as they are either not cost-effective or of proven benefit or both.   

Not Recommended

Not Recommended - These are specialist drugs that are not recommended for use.  

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