XTEND-Kids

  • Research type

    Research Study

  • Full title

    A Phase 3 open-label, multicenter study of the safety, efficacy and pharmacokinetics of intravenous recombinant coagulation Factor VIII Fc-von Willebrand Factor-XTEN fusion protein (rFVIIIFc-VWF-XTEN; BIVV001)in previously treated pediatric patients <12 years of age with severe hemophilia A

  • IRAS ID

    284576

  • Contact name

    Mary Mathias

  • Contact email

    Mary.Mathias@gosh.nhs.uk

  • Sponsor organisation

    Sanofi

  • Eudract number

    2020-000769-18

  • Clinicaltrials.gov Identifier

    U1111-1244-0558, WHO; 017464, IND

  • Duration of Study in the UK

    2 years, 11 months, 1 days

  • Research summary

    Haemophilia A is a hereditary X-linked bleeding disorder that occurs almost only in males and is characterised by the lack of a blood factor called Factor VIII. Severe Haemophilia A is determined by the levels of Factor VIII in the plasma; the lower the amount the more severe the haemophilia. Individuals with severe haemophilia experience frequent bleeding episodes into major joints, soft tissue and muscle either following a minor bump or spontaneously. The disease can be life threatening or lead to long-term complications. It also has significant effects on physical and mental well-being, quality of life and financial implications for the patient and their family.

    Most current therapies are prophylactic (preventing bleeds from occurring rather than treating each individual bleed). The prophylactic treatment improves long term outcomes by preventing joint bleeding, reduction in joint movement and chronic pain. Prophylactic treatment can cause treatment burden as the treatment has to be given 2-3 times a week, by injection and increases the risk of infections. These are often a reason why some patients chose to be treated only when an episode of bleeding (episode treatment) occurs however the likelihood of more bleeds in episode treatment is much higher than in patients receiving prophylactic treatment.

    BIV001 is a new class of blood clotting Factor VIII used in prophylactic treatment that prevents and controls bleeding episodes for longer. It maintains higher activity levels in the blood than currently available treatment and is only required to be administered once a week. In summary this new class of drug prevents and controls bleeding episodes for longer periods of time and potentially reducing the burden of frequent injections and in turn may improve the QoL for these patients.

  • REC name

    North West - Liverpool Central Research Ethics Committee

  • REC reference

    20/NW/0390

  • Date of REC Opinion

    17 Nov 2020

  • REC opinion

    Further Information Favourable Opinion