Lancashire and South Cumbria
Formulary
Formulary
9 Nutrition and blood
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.
For more information about the formulary development process, please see: https://www.lancsmmg.nhs.uk/lancashire-and-south-cumbria-icb-formulary-development/
09-08-01 Drugs used in metabolic disorders
| Asfotase alfa Strensiq® |
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Formulary
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Solution for injection Tertiary Centre Only. |
 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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Solution for injection pre-filled syringe 25mg/0.5ml 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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Links
LSCMMG shared care guideline - penicillamine- 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: Penicillamine shared care guideline
- LSCMMG: PKU - Prescribing of Multivitamins & Phenylalanine-free amino acid substitutes for adults and children
- Medicines for children: Carnitine for metabolic disorders
- NICE CG32: Nutrition support for adults: oral nutrition support, enteral tube feeding and parenteral nutrition
- NICE HST23 Asfotase alfa for treating paediatric-onset hypophosphatasia
- NICE HST26 Eladocagene exuparvovec for treating aromatic L-amino acid decarboxylase deficiency
- NICE NG20: Coeliac disease: recognition, assessment and management
- NICE NG203: Chronic kidney disease: assessment and management
- NICE TA 915:Pegunigalsidase alfa for treating Fabry diease
- NICE TA1020: Eplontersen for treating hereditary transthyretin-related amyloidosis
- NICE TA821: Avalglucosidase alfa for treating Pompe disease
- NICE TA821: Avalglucosidase alfa for treating Pompe disease
- NICE TA868 Vutrisiran for treating hereditary transthyretin-related amyloidosis
- NICE TA868: Vutrisiran for treating hereditary transthyretin-related amyloidosis
- NICE TA915: Pegunigalsidase alfa for treating Fabry disease
- NICE: Asfotase alfa for treating paediatric-onset hypophosphatasia