A multi-center, phase III, non-controlled, open-label trial to evaluate the pharmacokinetics, safety, and efficacy of BAY 94-9027 for prophylaxis and treatment of bleeding in previously treated children (age < 12 years) with severe hemophilia A.

The primary goal of the BAY94-9027 program is to develop a longer acting
recombinant FVIII (rFVIII) for use in prophylaxis across different dosing
regimens. It is expected that improved adherence to prophylaxis and
consequently better long-term outcomes could be achieved if treatment were more
convenient, and the duration of effect prolonged. A longer half-life of FVIII
would result in a reduction in the number of infusions required each week to
maintain FVIII trough levels above 1 IU/dl, fewer infusions to provide
hemostasis during surgical procedures, and would provide subjects with a
greater number of potential choices for tailoring treatment to their own
specific bleeding patterns. It is expected that a longer acting rFVIII will be
of benefit following dental, surgical, and other invasive challenges by
reducing a need for frequently repeated treatment in order to maintain adequate
hemostasis.

Main objectives: safety and efficacy of BAY94-9027 for prophylaxis and
treatment of bleeding in PTP with hemophilia A

Secondary objective: PK

The study consists of at least 6 months of prophylactic treatment (or the
amount of time required for a subject to accumulate a minimum of 50 exposure
days) with BAY 94-9027 in previously treated severe hemophilia A patients.

All subjects will receive intravenous prophylactic administration of BAY
94-9027 at least once every 7-days. Doses and dose intervals (2 times weekly,
every 5 days, or every 7 days) will be determined by the subject*s clinical
need, level of activity, and known past bleeding history and may be adjusted as
needed.

BAY 94-9027 will also be used for treatment of any breakthrough bleeding events.

After the Screening (Visit 1) and Baseline (Visit 2) visits, all subjects will
be evaluated for inhibitor development monthly for the first 3 months of
treatment, and again after 6 months or 50 exposure days.

Pharmacokinetics will be performed in at least 12 subjects in each age subgroup
(age groups 6 to < 12 years and < 6 years). Blood samples for pharmacokinetic
analyses will be collected once at pre-selected time points between pre-dose
and 72 hours post-infusion. Attempt will be made to collect PK at the baseline
visit, but if blood draw volumes or the convenience to the subject/parent will
not permit, PK may be obtained at any scheduled visit during the main study.

Infusions and bleeding events will be documented in an electronic patient diary
throughout the study.

All subjects completing the main study will be offered participation in an
optional extension study for collection of observations for at least 50
additional exposure days. Subjects will continue to be monitored for inhibitor
development every 6 months and at the end of the study.

Subjects will receive BAY 94-9027 once or a number of times per week (see
section *study design*).

Up to 10 visits, 2 times physical examination, at 3 time points in the study 2
QoL, ongoing electronic patient diary documentation, 13 blood samples

Benefit: potential to provide prophylactic protection from bleeding with a
reduced number of injections

Risk: development of inhibitory antibodies against FVIII or BAY94-9027