A Study With OPTIVATE® in People With Von Willebrand Disease

Inclusion Criteria:

1. Have given written informed consent.

2. Be aged 12 years or older.

3. Have severe VWD (VWF:RCo <20%) of known type. Severity will be confirmed by a currentVWF:RCo result of <20%.

4. Be known or expected to require a concentrate for management of VWD.

5. Must have had at least one bleed in the last 12 months which required treatment with aFVIII and VWF concentrate.

6. Have a known lack of, or poor response to, DDAVP.

7. Have a prothrombin time (PT) of not more than 3 seconds above the upper limit of thereference range.

8. At the Baseline Visit (Visit 1), patients must have had at least 5 days since theirlast infusion of replacement factor concentrate or DDAVP.

9. Female patients of child-bearing potential must have a negative result on a humanchorionic gonadotropin-based pregnancy test. If a female patient is or becomessexually active, she must practice contraception by using a method of provenreliability for the duration of the study. Female patients must not be lactating.

Exclusion Criteria:

1. Have a history of inhibitor development to VWF or FVIII or a positive result atscreening.

2. Actively bleeding (Note: the patient can enter the study once the bleed iscontrolled).

3. Presence of major systemic illnesses: renal disease, liver disease, or neurological orpsychiatric disease which would compromise the outcome of the study in the opinion ofthe investigator.

4. Known or suspected hypersensitivity to investigational medicinal product (IMP) or itsexcipients.

5. Have a recent history of alcohol or drug abuse.

6. Administration of a new chemical entity within the 4 months preceding enrolment.

7. Participation in any other clinical study in which investigational or marketed drugswere employed in the 30 days preceding enrolment into this study, with the exceptionof the BPL clinical study Protocol 8VWF03.

8. In the opinion of the investigator, the patient is unlikely to comply with the studyprotocol.