Von Willebrand Disease
Disclaimer
These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline.
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Aim
To guide PCH ED staff with the assessment and management of von Willebrand disease (vWD).
Background
- Von Willebrand disease is the most common inherited bleeding disorder affecting up to 1% of the population1 It is caused by either a qualitative or functional deficiency in von Willebrand factor (vWF), leading to inadequate platelet adhesion and secondary deficiency of Factor VIII.
- 3 characteristic features are:
- Excessive mucocutaneous bleeding
- Abnormal vWD studies
- Family history of abnormal bleeding1
- vWD is classified into 3 types:
- Type 1 (common) – typically associated with mild bleeding
- Type 2(uncommon) – due to functional deficiency with variable bleeding pattern
- Type 3 (rare) – due to an almost complete absence of vWF, patients may present with bleeding symptoms similar to those with moderate to severe haemophilia.
- Von Willebrand Disease usually presents with epistaxis, easy bruising, menorrhagia, post-operative bleeding or post traumatic bleeding (including post-partum haemorrhage).1
Management2,3
All presentations to the Emergency Department should be discussed with the on-call Clinical Haematologist before any treatment is commenced.
Treatment for von Willebrand Disease can include a combination of the following:
Desmopressin (DDAVP) – increases plasma concentration of (vWF) by releasing endogenous vWF stores2:
- Refer to PCH Desmopressin Monograph for dosing and administration details.
- Used for control of minor bleeding episodes and minor surgical procedures in type 1 vWD.
- Contraindicated in patients <2yrs, seizure disorders, active cardiovascular disease, Type 2B or Type 3 vWD.
- Relative contraindication in patients < 2 years due to risk of fluid and electrolyte complications (discuss treatment options with on call Clinical Haematologist).
Plasma Derived Factor VIII and von Willebrand Factor (Biostate®)
- Patients with vWD Type 2 and Type 3 most often require factor replacement with plasma-derived Factor VIII and vWF (Biostate®) for bleeding episodes and procedures.
- Refer to PCH Transfusion Medicine Protocol Factor VIII (8) (internal WA Health only) for dosing and administration details.
- There are no recombinant vWF products yet in clinical use at PCH.
Note: Biostate® is a plasma derived product; transfusion medicine processes including consent, must be followed. Biostate® is available via Transfusion Medicine.
Tranexamic acid – anti-fibrinolytic therapy2
- Dose: 4 weeks – 18 years, oral 15–25 mg/kg/dose (maximum 1.5 g/dose) 2 or 3 times daily (round doses to the nearest portion of a tablet where appropriate).4
- Seek advice from the on-call Clinical Haematologist for dosing in renal impairment.5
- Local haemostatic measures and hormonal contraceptive use should be considered in conjunction with above measures.
- Note: persistent haematuria should not be treated with tranexamic acid.
Indications for admitting a patient with an underlying bleeding disorder3
- Suspected intracranial haemorrhage
- Persistent mucocutaneous bleed not responding to factor replacement therapy and antifibrinolytic therapy (mouth bleed, epistaxis)
- Tonsillar haemorrhage
- Persistent haematuria
- Undiagnosed abdominal pain
- Unexplained muscular or soft tissue bleed including:
- suspected psoas haemorrhage
- bleeding into hip or inguinal area
- compartment syndrome
- bleeding into neck
- tight soft tissue bleeds
Nursing
References
- Lillicrap D and James P. Von Willebrand disease: an introduction for the primary care physician, World Federation of Hemophilia Treatment of Hemophilia monograph. 2009.
- Pasi KJ, et al. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization. Haemophilia. 2004 10, 2018-231. doi: 10.1111/j.1365-2516.2004.00886.x218Ó2004
- Textbook of Paediatric Emergency Medicine 3rd Edition Cameron P, Browne GJ, Mitra B, et al (2018) Publisher: Elsevier Edition updated.
- Australian Medicines Handbook Pty Ltd. Tranexamic acid. In: AMH Children’s Dosing Companion [Internet]. Adelaide (SA): Australian Medicines Handbook Pty Ltd; 2025 [cited 2026 Apr 8]. Available from: Tranexamic acid - AMH Children's Dosing Companion
- Australian Medicines Handbook Pty Ltd. Tranexamic acid. In: Australian Medicines Handbook [Internet]. Adelaide (SA): Australian Medicines Handbook Pty Ltd; 2026 [cited 2026 Apr 8]. Available from: Tranexamic acid - Australian Medicines Handbook
| Endorsed by: |
CAHS Drugs and Therapeutics Committee |
Date: |
Apr 2026 |
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