Paediatric Acute care Guidelines PMH Emergency Department

Also see guideline Management of a Child with a Bleeding Disorder 

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.

Treatment for von Willebrand Disease is usually with:

  • DDAVP (Desmopressin) 0.3 microgram/kg diluted in 0.9% saline and given IV over 20 minutes for the common type-1 patients.
  • 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
  • Please refer to the Haematology Transfusion Medicine Protocols for further information 

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

References

External Review: Catherine Cole (Paediatric and Adolescent Oncologist/Haematologist) – July 2015

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