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Sephience is a brand name for Sepiapterin. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: Sephience is indicated for the treatment of hyperphenylalaninaemia (HPA) in adult and paediatric patients with phenylketonuria (PKU).
Verbatim from this product's EMA label. Tap a section to expand.
Treatment with Sephience must be initiated and supervised by a physician experienced in the treatment of PKU. Posology The recommended dose (mg/kg/day) of Sephience to be administered orally once daily is based on age and body weight (see Table 1).
The maximum recommended dose is 60 mg/kg/day. The recommended dose of Sephience in patients ≥ 2 years of age is 60 mg/kg/day. However, the dose may be adjusted to a lower dose if the treating physician considers it necessary or appropriate.
5, 15, 30, and 60 mg/kg/day). 4 5 * Instead of four 250 mg sachets, one full 1 000 mg sachet can be mixed with 36 mL of water or apple juice. This mixture should be administered with a syringe, according to the volume to be administered detailed in Table 5.
Recommended dose of Sephience oral powder in sachet by body weight for patients 2 years and older and weighing more than 16 kg The recommended dose is 60 mg/kg/day. The calculated daily dose should be rounded to the nearest multiple of 250 mg or 1 000 mg, as appropriate.
For instance, a calculated dose of 1 251 to 1 374 mg should be rounded down to 1 250 mg corresponding to 1×250 mg sachet and 1×1 000 mg sachet. A calculated dose of 1 375 to 1 499 mg should be rounded up to 1 500 mg corresponding to 2×250 mg sachets and 1×1000 mg.
Missed dose A missed dose should be taken as soon as possible. The normal dosing schedule should be resumed the following day. Discontinuation of treatment In the pivotal Phase 3 clinical study, a threshold of 15% or greater reduction in blood phenylalanine (Phe) levels was utilised for determination of response.
No controlled efficacy and safety data are available in patients who do not experience a reduction of 15% or greater reduction in blood Phe levels after receiving sepiapterin for 14 days. The determination of responsiveness for a patient with PKU and the discontinuation of the medicinal product is at the discretion of the treating physician.
Special populations Elderly The safety and efficacy of Sephience in patients 65 years of age and older have not been established. Caution should be exercised when prescribing in patients 65 years of age and older. Renal impairment The safety and efficacy of Sephience in patients with renal impairment have not yet been established.
4 5 * Instead of four 250 mg sachets, one full 1 000 mg sachet can be mixed with 36 mL of water or apple juice. This mixture should be administered with a syringe, according to the volume to be administered detailed in Table
Dietary intake Patients treated with Sephience should undergo regular clinical assessments to align with their health care provider on appropriate dietary Phe intake (such as monitoring of blood Phe and tyrosine levels and nutritional intake).
g. 5). 8 for adverse reactions evaluated to date for sepiapterin). Excipients with known effect Sodium content This medicinal product contains less than 1 mmol sodium (23 mg) per sachet, that is to say essentially ‘sodium-free’. 7 Isomalt content Patients with rare hereditary problems of fructose intolerance should not take this medicinal product.
1.
Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
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2). Hepatic impairment The safety and efficacy of Sephience in patients with hepatic impairment have not yet been established. 2). 8). Method of administration Oral use. Sephience should be administered once daily with a meal, using mg/kg dosing.
Sephience oral powder comes in individual sachets of 250 mg or 1 000 mg and should be mixed in water, apple juice, or a small amount of soft food such as apple sauce and jams. Sephience is intended for long-term use. 6 Patients weighing 16 kg or less Sephience should be mixed with water or apple juice (9 mL for each 250 mg sachet; 36 mL for each 1 000 mg sachet), and a portion of this mixture corresponding to a required dose should be administered orally via an oral dosing syringe.
The preparation should be mixed well for at least 30 seconds until uniform and free of lumps, before drawing into the dosing syringe. Once mixed, the dose should be administered immediately. If not administered immediately, the liquid mixture can be administered within 6 hours or 24 hours, when stored at room temperature (below 25 °C) or in a refrigerator (2 °C - 8 °C), respectively.
The preparation should be mixed once again for at […]