A Trial Investigating Safety and Efficacy of Treatment With BAY94-9027 in Severe Hemophilia A

Haemophilia A is an inherited disorder in which one of the proteins, Factor VIII, needed to
form blood clots is missing or not present in sufficient levels. In a person with haemophilia
A, the clotting process is slowed and the person experiences bleeds that can result in
serious problems and potential disability.

The current standard treatment for severe haemophilia A is regularly scheduled infusion of
FVIII to keep levels high enough to prevent bleeding. Due to the short half-life of FVIII,
prophylaxis may require treatment as often as every other day.

In this trial safety and efficacy of a long-acting recombinant factor VIII molecule is
evaluated in subjects with severe Hemophilia A.

120-140 patients will receive open label treatment with long-acting rFVIII either on-demand
to treat bleeds or prophylactically for 36 weeks in the main trial plus an optional extension
to continue treatment for at least 100 total exposure days (ED). Patients on prophylactic
treatment will receive study drug at dosing intervals between once and twice a week depending
on their observed bleeding. Patients will attend the treatment centre for routine blood
samples and be required to keep an electronic diary.

Male patients aged 12-65, with severe hemophilia A, previously treated with FVIII for at
least 50 exposure days may be eligible for this study.