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 Emergency Department disclaimer.

Aim

To guide PCH ED staff with the assessment and management of von Willebrand disease.

Background

  • Von Willebrand disease is a common autosomal dominant disorder, with three subtypes
  • Von Willebrand Disease usually presents with easy bruising, mucosal bleeding, post-operative bleeding or post traumatic bleeding 
    • Menorrhagia and post-partum haemorrhage are common presentations in females.

Management

Treatment for von Willebrand Disease is usually with:

  • Patients with Type 1 account for 60-80% and is a partial quantitative deficiency with mild to moderate episodes
  • DDAVP (Desmopressin) 0.3 microgram/kg diluted in 30mls of 0.9% saline and given IV over 20 minutes increases plasma levels 3-5 times from baseline.
  • Patients with type-2 disorders (functional deficiency) and the rare type-3 disorder (homozygous disorder) will require factor replacement therapy with plasma-derived factor VIII (Biostate).
    • Recombinant factor VIII does not contain von Willebrand factor.
    • Supplies of plasma-derived factor VIII (Biostate) are kept in the fridge in Transfusion Medicine (Haematology laboratory).  
  • All presentations to the Emergency Department should be discussed with the on-call Haematologist before any treatment is instituted.

Indications for admitting a patient with an underlying bleeding disorder

  • Suspected intracranial haemorrhage
  • Persistent mouth bleeding not responding to factor replacement therapy and antifibrinolytic therapy
  • Persistent haematuria
  • Severe persistent epistaxis
  • Undiagnosed abdominal pain
  • Suspected psoas haemorrhage
  • Bleeding into hip or inguinal area
  • Compartmental syndrome such as forearm bleeding
  • Bleeding into neck
  • Tonsillar haemorrhage
  • Tight soft tissue bleeds.

Nursing

Bibliography

  1. External Review: Catherine Cole - Paediatric and Adolescent Oncologist/Haematologist – July 2015
  2. Sadler et al 2000 Accessed Up to date
  3. Textbook of Emergency Medicine 2nd edition E Doyle  271
  4. Mannucci  PM Treatment of Von Willebrand’s disease NEJM 12 2004 351 (7) 683-694

Endorsed by:  Director, Emergency Department  Date:  Aug 2017


 Review date:   Aug 2020


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


Related guideline