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Clinical Practice Guidelines

Haemophilia Treatments

  • See also

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    Ìý

    Ìý
    Von Willebrand disease

    Clotting factor replacement

    Some haemophilia treatment products are stored in hospital pharmacies. Others are stored in hospital or state blood banks

    Consider the availability of a product before prescribing. More information

    Haemophilia factor replacement products

    Product type

    Product name (Brand)

    Vial sizes
    (units)

    Comment

    Recombinant FVIII(8)

    Ìý Ìý Ìý

    Xyntha®

    250, 500, 1000, 2000, 3000


    Standard half life

    Advate®

    250, 500, 1000, 1500, 2000, 3000
    Eloctate®

    250, 500, 1000, 2000, 3000


    Extended half life

    Adynovate®

    250, 500, 1000, 1500, 2000, 3000Ìý

    Recombinant FIX(9)

    Ìý

    Benefix®

    250, 500, 1000, 2000, 3000

    Standard half lifeÌý
    Alprolix®

    Ìý250, 500, 1000, 2000, 3000

    		 Extended half lifeÌý

    Plasma derived FVIII(8) & von Willebrand factor

    Biostate®

    250, 500, 1000 (units of FVIII)

    Recombinant factor VIIa bypassing agent

    Novoseven®RT

    1 mg,Ìý 2 mg, 5 mg

    Use in children with factor VIII or factor IX inhibitors

    Factor VIII Inhibitor bypassing agent (plasma derived)

    FEIBA-NF®

    500, 1000, 2500

    Use for children with factor VIII inhibitors

    Not used in children receiving Emicizumab (Hemlibra)

    Instructions on how to prepare & administer Clotting factor concentrates can be found here

    Recommended initial dosage of factor VIII (8) & factor IX (9) replacements for injuries


    Type of bleed

    Haemophilia A

    Haemophilia B

    Comment

    Use recombinant FVIII (8)

    (units/kg)

    Use recombinant FIX (9) (units/kg)

    < 12 years of age

    > 12 years of age

    Joint

    Day 1: 40
    Day 2: 40

    Day 1:100

    May need follow up dose Day3: 50

    Day 1: 60

    May need follow up dose Day3: 30

    May need further doses depending on clinical response

    Muscle (minor)

    30

    100

    60

    Only superficial muscles are affected and there is no neurovascular compromise

    Muscle (major)

    50

    150

    100

    When deep muscles are affected, there are concerns of neurovascular compromise and/or suspected significant blood loss

    Calf and forearm bleeds mayÌýlead to compartment syndrome and be limb threatening.Ìý Consider consulting a surgeon

    Involvement of the iliopsoas muscle may be associated with significant blood loss

    Oral mucosa and dental

    30

    100

    60

    Administer with anti-fibrinolytic treatment

    Epistaxis (active)

    30

    100

    60

    Apply firm local pressure.Ìý
    Administer with anti-fibrinolytic therapy to preventÌýrecurrence

    Gastrointestinal

    50

    125

    75

    Gastroenterology review is required; aÌýsite of bleeding is usually foundÌýÌýÌý
    Consider adjunct anti-fibrinolytic treatment

    Genitourinary

    50

    125

    75

    Evaluate for all causes.ÌýA site of bleeding is rarely found.ÌýDo not give anti-fibrinolytic treatment

    CNS/head

    75

    175-200

    125

    Always treat with factorÌýreplacementÌýprior to imaging
    Neurosurgical review is required

    Trauma or surgery

    50-75

    175-200

    125

    Consultation withÌýa haematologist is essential

    Non-factor treatment: Emicizumab (Hemlibra)

    • Emicizumab (Hemlibra) is a bi-specific monoclonal antibody directed toward factor IXa and factor X, and acts to mimic the function of missing factor VIII
    • Creasts an equivalent factor VIII level of approximately 10% or more
    • Effective in children with haemophilia A, with or without factor VIII inhibitor
    • Administered via subcutaneous injection
    • Not intended to treat acute bleeding episodes
    • Breakthrough bleeding is treated with factor VIII (or bypassing agents in a child with factor VIII inhibitors)
    • Emicizumab (Hemlibra) affects coagulation testing (APTT, factor VIII). Standard coagulation testing will not be accurate and should not be used to monitor APTT, factor VIII or VIII inhibitor

    Antifibrinolytic treatment

    • Reduces breakdown of blood clots and is effective for treating and preventing the recurrence of mouth bleeds and epistaxis in all severities of haemophilia
    • Contraindicated for treatment of haematuria
    • Can be used in children on Emicizumab prophylaxis

    Dose

    Tranexamic acid (500 mg tablet): oral, 25 mg/kg/dose (max 1.5 g/dose) three times daily, for 5-7 days

    Haemophilia inhibitor bypass agents

    Special considerations for the treatment of children with inhibitors to factor VIII or IX

    • In haemophilia, inhibitors refer to IgG antibodies that neutralise clotting factors
    • Management of children with inhibitors is complex and the haemophilia treatment centre should be consulted
    • Treatment and prevention of bleeding in children with inhibitors is managed with bypassing agents eg Novoseven®RT, FEIBA®
    • Different bypassing agents should never be administered concurrently due to the risk of thrombosis eg Novoseven®RT, FEIBA®
    • Children with factor VIII inhibitors, and on Emicizumab prophylaxis, should not receive Activated prothrombin complex concentrate (FEIBA®) due to reports of thrombotic microagniopathy and thromboembolismÌý
    • Many children with inhibitors to factor IX have severe allergic reactions, including anaphylaxis, to clotting factor replacement. These reactions can be the first symptom of inhibitor development

    Recommended dosage of Haemophilia inhibitor bypass agents

    Inhibitor treatment productÌýtype

    Inhibitor treatment product name

    Indication

    Dosage
    (IV boluses)

    Recombinant activated factor VII (7)

    Novoseven®RT

    Haemophilia A or B with inhibitorÌý

    In minor bleeding:Ìý Ìý Ìý Ìý Ìý Ìý Ìý Ìý Ìý Ìý Ìý Ìý

    90 microgram/kgÌýevery 2 hours until haemostasis achieved, then wean dose frequency

    Dosing may need to be modified in children on Emicizumab (Hemlibra) prophylaxis

    Desmopressin (DDAVP) use in mild haemophilia A

    • DDAVP releases stored factor VIII and von Willebrand factor into the circulation
    • It is used in children with mild haemophilia A where there is a documented record of safe and satisfactory response to a Desmopressin challenge
    • It is 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)Ìý
    • Fluid restriction (approximately 2/3 of daily maintenance fluid requirement) is recommended in the 24 hour period following Desmopressin administration
      • Dose: 0.3 microgram/kg (max 20 microgram) stat
      • Administration: via intravenous infusion or subcutaneous injection
      • 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

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    Last updated April 2023