Dystrophic epidermolysis bullosa

Active Ingredient: Beremagene geperpavec

Indication for Beremagene geperpavec

Population group: only infants (40 days - 1 year old) , children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 years old or older)
Therapeutic intent: Curative procedure

Beremagene geperpavec is indicated for the treatment of wounds in patients with dystrophic epidermolysis bullosa (DEB) with mutation(s) in the collagen type VII alpha 1 chain (COL7A1) gene, from birth.

For this indication, competent medicine agencies globally authorize below treatments:

For patients up to <3 years old 2 × 10⁹ PFU maximum weekly dose and for patients ≥3 years old 4 × 10⁹ PFU maximum weekly dose

For:

Dosage regimens

Regimen A, in case that patient age in years is < 3

Cutaneous, 2,000,000,000 plaque-forming units beremagene geperpavec, divided weekly.

Regimen B, in case that patient age in years is ≥ 3

Cutaneous, 4,000,000,000 plaque-forming units beremagene geperpavec, divided weekly.

Detailed description

Beremagene geperpavec is applied cutaneously to wound(s) once a week in small droplets in a grid-like pattern, approximately 1-cm by 1-cm apart. All wounds may not be possible to be treated at each treatment visit.

The recommended total maximum weekly dosing for children from birth up to 3 years old is 2×109 PFU. The recommended total maximum weekly dosing for children above 3 years of age, adolescents, and adults is 4×109 PFU.

Beremagene geperpavec should be applied to wounds until they are closed before selecting new wound(s) to treat. Weekly treatment of previously treated wounds should be prioritised if they re-open. If no wounds are present, beremagene geperpavec should not be administered.

The table below provides a reference on dose per approximate size of the wound in children, adolescents, and adults.

Table 1. Dose by wound area:

Wound area (cm2)*Dose (PFU)a
<20<4×108
20 to <404×108 to <8×108
40 to 608×108 to <1.2×109
60 to <2001.2×109 to <4×109

PFU = plaque forming units.
a The maximum dose in children below 3 years of age is 1 mL (2×109 PFU)

If a dose is missed, beremagene geperpavec should be administered as soon as possible, and weekly dosing should be resumed thereafter.

Dosage considerations

Wounds should be gently cleaned prior to cutaneous administration using a product that does not contain a virucidal agent. Medicinal products and ointments at the wound area should be removed and the wound should be cleansed prior to beremagene geperpavec administration to ensure no reduction in its activity.

Active ingredient

Beremagene geperpavec

Beremagene geperpavec is a gene therapy based on an engineered, replication-defective herpes simplex virus 1 (HSV-1) encoded with COL7A1 gene, addressing the underlying genetic cause of dystrophic epidermolysis bullosa. The HSV-1 vector belongs to the human herpes virus (HHV) family of double-stranded DNA viruses. Upon cutaneous application to the wounds, beremagene geperpavec can transduce both keratinocytes and fibroblasts.

Read more about Beremagene geperpavec

Related medicines

Develop a tailored medication plan for your case, considering factors such as age, gender, and health history

Ask the Reasoner

Liability Disclaimer : RxReasoner has utilized reasonable care in providing content and services that are accurate, complete and up to date. However, RxReasoner does not accept any responsibility or liability about it. The content and services of RxReasoner are for informational purposes only and they are not intended to be a substitute for the knowledge, expertise, skill, and judgment of physicians, pharmacists, nurses, or other healthcare professionals involved in patient care. RxReasoner offers no medical advice. Users are responsible for the use of the provided content. A shown indication or treatment should not be construed to indicate that the medication is safe, appropriate, or effective in any given patient or under any particular circumstances. The absence of an indication or treatment should not roule out the existence of other appropriate medications. Always seek the advice of a physician or other qualified health provider with any questions you may have regarding a medical condition or medicament. RxReasoner is not liable for any damages allegedly sustained arising out of the use of its content and services.