Haemophilia


  • Statewide logo

    This guideline has been adapted for statewide use with the support of the Victorian Paediatric Clinical Network

  • See also

    Find a haemophilia treatment centre 
    National Haemophilia Guidelines
    Haemophilia Foundation Australia 
    Von Willebrand disease

    Key Points

    1. Assessment and investigation should not delay factor replacement.
    2. If there is ongoing bleeding after factor replacement, presume that the patient has developed a factor inhibitor and treat accordingly.
    3. All children with haemophilia and bleeding should be discussed with a haemophilia treatment centre.
    4. A haemophilia treatment plan should be made, in consultation with a Haematologist, before a haemophiliac undergoes a procedure (E.g. lumbar puncture).

    Background

    • Haemophilia is an X-linked bleeding disorder affecting 1 in 6,000-10,000 males and less than 1 in 300,000 females.
    • Haemophilia A is clotting Factor VIII (8) deficiency.
    • Haemophilia B is clotting Factor IX (9) deficiency.

    Assessment

    History

    • Elicit a detailed description of each type and mechanism of injury. Internal and joint injuries are often missed.
    • Determine the type of haemophilia (Factor VIII or IX); the child's treatment plan, blood product replacement plan and if there are Factor VIII/IX inhibitors.
    • Check if the child is on a home prophylaxis program and when the most recent dose of factor(s) was administered.

    Examination

    • Assess the site and extent of bleeding
    • Assess the impact on function.
    • Major or suspected bleeding in the head, neck, chest, gastrointestinal tract and abdomen and/or pelvis should be treated with clotting factor immediately, before a full assessment is complete. 

    Assessment of severity

    Severity

    Concentration of
          Cloting Factor (%)

    Typical Bleeding Picture

    Severe 

    <1 

    Frequent bleed episodes are common; predominantly into joints & muscles.  Bleeding can occur    spontaneously or after a minor injury.

    Moderate 

    1-5

    Can bleed after a minor injury.  May have bleeding into joints.  Severe bleeds occur with moderate trauma, surgery & invasive procedures.

    Mild

    >5-40

    Bleeding occurs with major traumas such as surgery & invasive procedures.

    Management

    Investigations

    • Clotting factor replacement should not be delayed by investigation.
    • Imaging of the site of suspected bleeding is dependent on the mechanism of injury (trauma).
    • Routine coagulation studies are not required.

    Treatment

    • Most bleeds will require factor replacement with the exception of minor soft tissue injuries and bruising that does not impact on function or mobility. 
    • Prompt clotting factor replacement reduces the pain and long-term consequences of bleeding.
    • Invasive procedures such as arterial puncture and lumbar puncture must only be performed after clotting factor replacement.
    • Do not give IM injections.

    Clotting Factor Replacement

    Do not delay clotting factor replacement in severe or life-threatening bleeding.

    Recombinant clotting factor concentrates are the product of choice. Occasionally children will be treated with plasma-derived concentrates for management of factor VIII/IX inhibitors.

    • Doses should be rounded up to use whole vials. E.g. 20 kg child requiring 30 units/kg factor VIII, give 750 units. 
    • The presence of a new inhibitor should be suspected in any child who fails to respond clinically to adequate factor replacement, particularly if the child has been previously responsive.
    • If the child has factor inhibitors (proven or suspected), a special product is required to ’bypass’ the inhibitor to initiate clotting. i.e. A bypassing agent
    • As clotting factor concentrates are not routinely available in all hospitals, children in regional or rural areas should have advanced care plans and ready access to blood products.

    Note that there are two recombinant Factor VIII (FVIII) products available. Whilst it is recommended that children maintain treatment with their established brand of FVIII product, in an emergency situation administration of any brand of recombinant FVIII is acceptable.

    See Haemophilia Treatments
    for more information on including the dosing and administration of factor products.
    Please note that clotting factor concentrates are not routinely available in all hospitals.

    General Measures - Joint and Muscle Bleeds

    For muscle and joint bleeds P.R.I.C.E will limit bleeding and reduce pain. Initiate on arrival.   

    • P = Protection (immobilise the affected area in a position of comfort e.g. splint/slings/crutches)
    • R = Rest
    • I = Ice (Apply a cold pack to reduce bleeding and pain)
    • C =Compression bandage (gentle)
    • E = Elevation

    Venous Access

    • Children with haemophilia will require frequent venous access, this can be a major fear and stressor, so minimisation of unsuccessful or distressing venepuncture is essential
    • Use distraction and relaxation techniques and consider nitrous oxide sedation. Ask parents about their preferred method for comforting and distracting their child.
    • Children with haemophilia are at risk of venepuncture related bleeding. Treat veins with care, apply pressure for at least 3 minutes post venepuncture.
    • In general, IV cannulas are not left insitu on discharge of Haemophiliac patient unless discussed with specialist.

    Analgesia

    • Do not use products containing aspirin or NSAIDS (e.g. ibuprofen, diclofenac) as they may worsen bleeding.
    • Paracetamol may be sufficient. Opiates can be used for severe pain.
    • Splinting and immobilisation is an effective adjunct for reducing pain.

    Consider consultation with local paediatric team when

    All children with haemophilia with bleeding episodes should be discussed with the haemophilia treatment centre.

    Consider transfer when

    All children with

    • Suspected intracranial haemorrhage
    • Bleeding into neck/throat
    • Forearm/calf bleed at risk of compartment syndrome
    • Bleeding into hip or inguinal area (due to risk of iliopsoas haemorrhage)
    • Undiagnosed abdominal pain
    • Persistent haematuria
    • Bleeds causing severe pain
    • Children requiring care beyond the level of comfort of the local hospital or treating medical team

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

    Consider discharge when

    • No active bleeding
    • Appropriate follow-up is arranged
    • Patients are provided with a Haemophilia Centre Treatment Card (for their management plan)

    Last updated October 2019

  • Reference List

    1. Guidelines for the management of haemophilia in Australia (2016). https://www.blood.gov.au/system/files/HaemophiliaGuidelines-interactive-updated-260317v2.pdf
    2. NBA (2006). Evidence-based clinical practice guidelines for the use of recombinant and plasma-derived FVIII and FIX products, National Blood Authority (NBA), Canberra, Australia. http://www.blood.gov.au/system/files/documents/fviii-fix-guidelines.pdf
    3. WFH Guidelines for the management of hemophilia (2nd edition), World Federation of Hemophilia (WFH), Canada. http://www1.wfh.org/publications/files/pdf-1472.pdf