´óÏóÊÓƵ

See also

Ìý


Haemophilia

Key points

1. Assessment and investigation should not delay treatment such as factor replacement
2. All children with von Willebrand disease (VWD) and bleeding should be discussed with the paediatric haematology team
3. A treatment plan should be made, in consultation with a haematologist, before performing any procedure

Background

Von Willebrand disease is the most common inherited bleeding disorder

• Caused by a deficiency (either quantitative or functional) in von Willebrand Factor (VWF)
• Bleeding is primarily due to impaired platelet adhesion. Factor VIII levels may also be reduced due to shortened half-life from reduced or dysfunctional VWF
• Affects males and females equally
• Characterised by easy bruising, bleeding from mucous membranes (particularly epistaxis, oral mucosa, menorrhagia) and post-op bleeding

The three main phenotypes of VWD

Assessment

History

• Elicit a detailed description of site and extent of bleeding
• Determine type and severity of VWD (if known)
• Check if any type of treatment has been given at home eg tranexamic acid

Examination

• Assess site and extent of bleeding
• Assess the impact on function in the setting of injury
• Major haemorrhage, head injury or any suspected internal bleeding should be treated with clotting factor immediately, before a full assessment is complete

Management

Investigations

• Treatment such as clotting factor replacement should not be delayed by investigations
• Consider FBE and ferritin in children with a history of recurrent mucosal bleeding, as anaemia and iron deficiency are common
• Imaging may be indicated based on history and examination findings
• Routine coagulation studies are not required

Treatment

Give clotting factor replacement immediately if severe or life-threatening bleeding

Antifibrinolytic treatment eg tranexamic acid

• Effective for treating, and preventing the recurrence of, mouth bleeds and epistaxis in all severities of VWD
• May be given alone or as adjunct therapy to desmopressin or factor concentrate
• Reduces breakdown of blood clots
• Often helpful for management of heavy menstrual bleeding either as a primary treatment or in combination with hormonal management
• Contraindicated for treatment of haematuria
• Dose: Tranexamic acid (500 mg tablet) oral, 25 mg/kg/dose (max 1.5 g/dose) three times daily, for 5-10 days (duration dependent on severity of injury)

Desmopressin (DDAVP)

• Used in children with mild to moderate Type 1 VWD where there is a documented record of safe and satisfactory response to a desmopressin challenge
• Occasionally effective in Type 2 VWD, never effective in Type 3 VWD
• Not adequate as a single agent to achieve haemostasis in major bleeding
• Generally, it is not used in children <3 years old or children with seizure disorders (can cause hyponatraemia and seizures)
• Desmopressin releases stored factor VIII and von Willebrand factor into the circulation
• Fluid restriction (approximately 2/3 of daily maintenance fluid requirement) is recommended for 24 hours following desmopressin administration
• Dose: 0.3 microgram/kg (max 20 micrograms) stat intravenous infusion or subcutaneous administration
• Intravenous infusion: dilute to a final volume of 50 mL with Sodium Chloride 0.9% and infuse over at least 30 minutes
• Subcutaneous injection: apply pressure to the site for 1 to 2 minutes post injection

Von Willebrand Factor/Factor VIII Plasma Concentrate (Biostate®)

• May be required in Type 1 VWD if severe bleeding or unresponsive to DDAVP
• Used to treat bleeding in people with Type 2 and Type 3 VWD
• A human plasma-derived product
• Biostate® contains FVIII and VWF in a ratio of 1:2.4
• Recombinant FVIII products do not contain von Willebrand factor
• May be stored in hospital pharmacies or in hospital or state blood banks

Recommended dosage of Biostate®

Note that the vial will state both Factor VIII and VWF units, prescribe according to Factor VIII units (eg Biostate® 2000 Factor VIII units). Round dosage to the closest vial size.

Instructions on how to prepare and administer clotting factor concentrates can be found here.

Analgesia

• Ensure adequate analgesia
• Do not use products containing aspirin or NSAIDs (eg ibuprofen, naproxen, diclofenac) as they may worsen bleeding

Consider consultation with local paediatric/haematology team when

Any child with VWD with suspected or identified bleeding episodes

Consider transfer when

Any child with VWD and the following

• suspected intracranial haemorrhage
• bleeding into neck or throat
• forearm or calf bleed at risk of compartment syndrome
• bleeding into hip or inguinal area (due to risk of iliopsoas haemorrhage)
• undiagnosed abdominal pain
• persistent haematuria
• bleeding causing severe pain
• ongoing mucosal bleeding

For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

Consider discharge when

• No active bleeding
• Appropriate follow up is arranged
• Children are provided with a management plan eg Haemophilia Treatment Centre Card

Parent information

Last Updated April 2026