Formulary Chapter 9: Nutrition and blood - Full Chapter
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Notes: |
This chapter of the formulary is under continual development, please let the team know if you have any comments about the contents: mlcsu.lscformulary@nhs.net.
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Chapter Links... |
LSCMMG: Bariatric surgery guidelines for prescribing of vitamins and nutritional supplements: Position Statement |
LSCMMG: Gluten-Free food products: Position Statement |
LSCMMG: Oral Nutritional Supplements in Primary Care |
LSCMMG: PKU - Prescribing of Multivitamins & Phenylalanine-free amino acid substitutes for adults and children |
NICE CG32: Nutrition support for adults: oral nutrition support, enteral tube feeding and parenteral nutrition |
NICE NG20: Coeliac disease: recognition, assessment and management |
NICE NG203: Chronic kidney disease: assessment and management |
Details... |
09.08.01 |
Drugs used in metabolic disorders |
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Asfotase alfa (Strensiq® )
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Formulary

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Solution for injection
Tertiary Centre Only.
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NICE: Asfotase alfa for treating paediatric-onset hypophosphatasia
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Avalglucosidase alfa (Nexviadyme ®)
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Formulary

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Powder for concentrate for solution for infusion 100mg
Under expert supervision.
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NICE TA821: Avalglucosidase alfa for treating Pompe disease
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Eladocagene exuparvovec (Upstaza®)
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Formulary

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Solution for infusion 2.8 × 1011 vector genomes (vg)/0.5 mL
Tertiary Centre Only.
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Eplontersen (Wainzua®)
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Formulary

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Solution for injection 45 mg/0.8 ml
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NICE TA1020: Eplontersen for treating hereditary transthyretin-related amyloidosis
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Pegunigalsidase alfa (Elafbrio®)
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Formulary
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Concentrate for solution for infusion 20mg/10ml, 5mg/2.5ml
Under expert supervision.
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NICE TA915: Pegunigalsidase alfa for treating Fabry disease
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Vutrisiran (Amvuttra ®)
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Formulary

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Under expert supervision.
Females of childbearing potential should use effective contraception during treatment; if conception is planned, vutrisiran and vitamin A supplementation should be stopped and vitamin A levels monitored - consult product literature.
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NICE TA868: Vutrisiran for treating hereditary transthyretin-related amyloidosis
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09.08.01 |
Wilsons disease |
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Penicillamine
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Formulary
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Tablets 125mg, 250mg
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LSCMMG: Penicillamine shared care guideline
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09.08.01 |
Carnitine deficiency |
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09.08.01 |
Fabry's disease |
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09.08.01 |
Gaucher's disease |
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09.08.01 |
Mucopolysaccharidosis I |
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09.08.01 |
Pompe disease |
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09.08.01 |
Nephropathic cystinosis |
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09.08.01 |
Urea cycle disorders |
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Key |
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Restricted Drug |
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Unlicensed |
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Link to adult BNF
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Link to children's BNF
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Link to SPCs
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Scottish Medicines Consortium |
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Cytotoxic Drug |
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Controlled Drug |
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High Cost Medicine |
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Cancer Drugs Fund |
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NHS England |
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Homecare |
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ICB |
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Low carbon footprint |
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Medium carbon footprint |
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High carbon footprint |
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Status |
Description |

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Green:
Appropriate for initiation and ongoing prescribing in both primary and secondary care.
Generally, little or no routine drug monitoring is required. |

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Green (Restricted):
Appropriate for initiation and ongoing prescribing in both primary and secondary care provided:
Additional criteria specific to the medicine or device are met, or
The medicine or device is used following the failure of other therapies as defined by the relevant LSCMMG pathway.
Generally, little or no routine drug monitoring is required.
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Red medicines:
Medicine is supplied by the hospital for the duration of the treatment course.
Primary care initiation or continuation of treatment is not recommended unless exceptional circumstances such as specialist GP.
Red medicines are those where primary care prescribing is not recommended. These treatments should be initiated by specialists only and prescribing retained within secondary care. They require specialist knowledge, intensive monitoring, specific dose adjustments or further evaluation in use. If however, a primary care prescriber has particular specialist knowledge or experience of prescribing a particular drug for a particular patient it would not always be appropriate for them to expect to transfer that prescribing responsibility back to secondary care. There should be a specific reason and a specific risk agreement, protocol and service set up to support this.
Primary care prescribers may prescribe RED medicines in exceptional circumstances to patients to ensure continuity of supply while arrangements are made to obtain ongoing supplies from secondary care. |

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Amber level 0:
Suitable for prescribing in primary care following recommendation or initiation by a specialist.
Little or no specific monitoring required.
Patient may need a regular review, but this would not exceed that required for other medicines routinely prescribed in primary care.
Brief prescribing document or information sheet may be required.
Primary care prescribers must be familiar with the drug to take on prescribing responsibility or must get the required information.
When recommending or handing over care, specialists should ask primary care prescribers to take over prescribing responsibility, and should give enough information about the indication, dose, monitoring requirements, use outside product licence and any necessary dose adjustments to allow them to confidently prescribe. |

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Amber level 1 (with shared care):
Suitable for prescribing in primary care following recommendation or initiation by a specialist.
Minimal monitoring required.
Patient may need a regular review, but this would not exceed that required for other medicines routinely prescribed in primary care.
Full prior agreement about patient’s on-going care must be reached under the shared care agreement.
Primary care prescribers are advised not to take on prescribing of these medicines unless they have been adequately informed by letter of their responsibilities with regards monitoring, side effects and interactions and are happy to take on the prescribing responsibility. A copy of locally approved shared care guidelines should accompany this letter which outlines these responsibilities. Primary care prescribers should then tell secondary care of their intentions as soon as possible by letter so that arrangements can be made for the transfer of care. |

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Amber level 2 (with shared care and enhanced service):
Initiated by specialist and transferred to primary care following a successful initiation period.
Significant monitoring required on an on-going basis.
Full prior agreement about patient’s on-going care must be reached under the shared care agreement.
Suitable for enhanced service.
These medicines are considered suitable for GP prescribing following specialist initiation of therapy, as per shared care document which will be sent out with the request to prescribe, with on-going communication between the primary care prescriber and specialist. Amber Level 2 medicines require significant monitoring for which an enhanced service may be suitable. (Subject to local commissioning agreements). |

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Do not prescribe: NOT recommended for use by the NHS in Lancashire and South Cumbria.
Includes medicines that NICE has not recommended for use and terminated technology appraisals, unless there is a local need. |

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Grey medicines:
Medicines which have not yet been reviewed or are under the review process.
GPs and specialists are recommended not to prescribe these drugs.
This category includes drugs where funding has not yet been agreed.
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Refer to local guidance. |
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