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.

Read the full PCH Clinical disclaimer

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 (usually autosomal dominant)
Three characteristic features are:
- Excessive mucocutaneous bleeding
- Abnormal vWD studies
- Family history of abnormal bleeding¹
vWD is classified into 3 types: Type 1 (quantitative deficiency), Type 2 (functional deficiency), Type 3 (almost complete absence of Von Willebrand Factor [vWF])
Von Willebrand Disease usually presents with epistaxis, easy bruising, menorrhagia, post-operative bleeding or post traumatic bleeding (including post-partum haemorrhage).¹

Management

All presentations to the Emergency Department should be discussed with the on-call Haematologist before any treatment is commenced.

Treatment for von Willebrand Disease can include:

Desmopressin (DDAVP)

Increases plasma concentration of (vWF) by releasing endogenous VWF stores²:

Refer to PCH Desmopressin Monograph (internal WA Health only) 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

Plasma Derived Factor VIII and von Willebrand Factor (Biostate®)

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
Patients with vWD Type 2 and Type 3 most often require factor replacement with plasma-derived factor Factor VIII and vWF (Biostate®) with bleeds and procedures.
As this is a plasma derived product – transfusion medicine processes including consent will need to be adhered to – Biostate® is available via Transfusion Medicine.

Tranexamic acid – anti-fibrinolytic therapy ²

Dose: 1 month – 18 years, oral 15–25 mg/kg (maximum 1.5 g) 2 or 3 times daily (round to the closest 250mg)
Dose adjustment should be considered for patients with eGFR < 29mL/min/1.73m²
Local haemostatic measures and hormonal contraceptive use should be considered in conjunction with above measures

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

Please refer to the Transfusion Medicine Protocols Factor VIII (internal WA Health only) for further information.

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

Endorsed by:	Drugs and Therapeutics Committee	Date:	May 2022
		Review date:	Feb 2025

This document can be made available in alternative formats on request for a person with a disability.