Version May 2024
Dystrophic epidermolysis bullosa, wound treatment:
Note: Application should be performed by a health care professional.
Infants ≥6 months, Children, and Adolescents: Topical: Apply appropriate amount of gel based on wound size (see table) to wounds once weekly until wounds closed. Do not exceed maximum weekly dose. It may not be possible to treat all wounds at each treatment visit; treat wounds until closed before selecting new wound(s); if wounds reopen, prioritize treating previously treated areas.
Dose by wound size:
|
Wound area |
Dosea |
Volume |
|---|---|---|
|
a Do not exceed age-based maximum weekly dose. b PFU = plaque-forming units. | ||
|
<20 cm2 |
4 × 108 PFUb |
0.2 mL |
|
20 to <40 cm2 |
8 × 108 PFU |
0.4 mL |
|
40 to 60 cm2 |
1.2 × 109 PFU |
0.6 mL |
|
>60 cm2 |
Calculate dose based on areas above, not to exceed age-based weekly maximum. | |
Maximum weekly doses:
Infants ≥6 months and Children <3 years: Topical: Maximum dose: 1.6 × 109 PFU (0.8 mL) once weekly.
Children ≥3 years and Adolescents: Topical: Maximum dose: 3.2 × 109 PFU (1.6 mL) once weekly.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Beremagene geperpavec: Drug information")
Dystrophic epidermolysis bullosa: Topical: Maximum weekly dose: Apply up to 3.2 × 109 plaque-forming units (maximum weekly volume: 1.6 mL) once weekly. Note: It may not be possible to apply gel to all wounds at each treatment visit. The total dose for wounds >60 cm2 should be calculated by wound size until the maximum weekly dose is reached.
|
Wound area |
Dose |
Volume |
|---|---|---|
|
<20 cm2 |
4 × 108 plaque-forming units |
0.2 mL |
|
20 to <40 cm2 |
8 × 108 plaque-forming units |
0.4 mL |
|
40 to 60 cm2 |
1.2 × 109 plaque-forming units |
0.6 mL |
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Reported adverse reactions are for adults and pediatrics.
1% to 10%:
Dermatologic: Erythema of skin (6%), pruritus (10%), skin rash (6%)
Nervous system: Chills (10%)
Respiratory: Cough (6%), rhinorrhea (6%)
There are no contraindications listed in the manufacturer's labeling.
Other warnings/precautions:
• Accidental exposure: Avoid direct contact (eg, scratching, touching) with treated wounds and dressing for ~24 hours after application; if exposure occurs, flush exposed area with clean water for at least 15 minutes. Wear protective gloves when administering, changing dressing, and disposing.
• Appropriate use: To be applied by a health care professional only in a health care setting.
Vyjuvek: FDA approved May 2023; availability anticipated in 3rd quarter of 2023.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Gel, External:
Vyjuvek: Beremagene geperpavec-svdt 5 billion PFU/2.5 mL (2.5 mL)
No
Gel (Vyjuvek External)
5000000000PFU/2.5ML (per mL): $11,640.00
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Topical: Application should be performed by a health care professional. Using prepared treatment syringes, apply to wound(s) in droplets spaced evenly within the wound (~1 cm by 1 cm apart); droplet pattern should loosely resemble a grid. Avoid touching administration syringe to the skin. After administering, apply a nonadherent hydrophobic dressing that is slightly larger than the wound; place a slightly larger standard dressing on top of the hydrophobic dressing; do not change dressing within ~24 hours of gel application; direct contact with treated wounds and their dressings should be avoided for ~24 hours. Clean all surfaces that have come in contact with the gel with a virucidal agent (eg, 70% isopropyl alcohol, 6% hydrogen peroxide, <0.4% ammonium chloride); dispose of all material in a biohazard bag or container.
Topical: For topical use only. Apply to wounds until they are closed before selecting new wounds to treat; it may not be possible to apply gel to all wounds at each treatment visit. If wounds reopen, prioritize open wounds for retreatment. Apply using prepared treatment syringes to wound(s) in droplets spaced evenly within the wound (~1 cm by 1 cm apart); droplet pattern should loosely resemble a grid (see dosing table for dose per approximate wound size). Avoid touching administration syringe to the skin. After administering, apply a slightly larger than the wound nonadherent hydrophobic dressing; place a slightly larger standard dressing on top of the hydrophobic dressing; do not change dressing within ~24 hours of gel application. Clean all surfaces and that have come in contact with the gel with a virucidal (eg, 70% isopropyl alcohol, 6% hydrogen peroxide, or <0.4% ammonium chloride). Dispose of all material (eg, syringe, cleaning material, unused administration syringes containing gel) in a biohazard bag or container.
Store carton at −15°C to −25°C (5°F to −13°F). If a freezer is not available, the carton can be refrigerated (2ºC to 8ºC [35.6ºF to 46.4ºF]) for up to 1 month. Do not refreeze once thawed. Once prepared, administration syringes containing the gel may remain at 20°C to 25°C (68°F to 77°F) for up to 8 hours. If immediate use is not possible, administration syringes can be stored for up to 48 hours in the refrigerator (2ºC to 8ºC [35.6ºF to 46.4ºF]). Discard if storage requirements are not met.
Treatment of wounds in patients with dystrophic epidermolysis bullosa with mutation(s) in the collagen type VII alpha 1 chain (COL7A1) gene (FDA approved in ages ≥6 months and adults).
None known.
There are no known significant interactions.
Patients who may become pregnant should use effective contraception during therapy.
Animal reproduction studies have not been conducted. Patients who are pregnant should avoid direct contact with treated dressings and treated wounds, and should not prepare or apply beremagene geperpavec gel to others.
Observe for chills and itching.
Beremagene geperpavec is a herpes simplex virus-1 (HSV-1) vector-based gene therapy that may transduce both keratinocytes and fibroblasts within the nucleus of the cells allowing for production and secretion of COL7; the COL7 molecules form anchoring fibrils, which hold the epidermis and dermis together and help maintain skin integrity. Patients with dystrophic epidermolysis bullosa have lower than normal or no functioning anchoring fibrils.
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