Haemophilia Treatments

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 here

Product type	Product name (Brand)	Vial sizes (units)	Comment
Recombinant FVIII(8)	Xyntha^®	250, 500, 1000, 2000, 3000	Standard half life
	Advate^®	250, 500, 1000, 1500, 2000, 3000	Standard half life
	Eloctate^®	250, 500, 1000, 2000, 3000	Extended half life
	Adynovate^®	250, 500, 1000, 1500, 2000, 3000	Extended half life
Recombinant FIX(9)	Benefix^®	250, 500, 1000, 2000, 3000	Standard half life
Recombinant FIX(9)	Alprolix^®	250, 500, 1000, 2000, 3000	Extended half life
Plasma derived FVIII(8) & von Willebrand factor	Biostate^®	250, 500, 1000 (units of FVIII)
Recombinant factor VIIa bypassing agent	Novoseven^®RT	1 mg, 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)

Type of bleed	Haemophilia A	Haemophilia B		Comment
	Use recombinant FVIII (8) (units/kg)	Use recombinant FIX (9) (units/kg)
	Use recombinant FVIII (8) (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 may lead to compartment syndrome and be limb threatening. 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. Administer with anti-fibrinolytic therapy to prevent recurrence
Gastrointestinal	50	125	75	Gastroenterology review is required; a site of bleeding is usually found Consider adjunct anti-fibrinolytic treatment
Genitourinary	50	125	75	Evaluate for all causes. A site of bleeding is rarely found. Do not give anti-fibrinolytic treatment
CNS/head	75	175-200	125	Always treat with factor replacement prior to imaging Neurosurgical review is required
Trauma or surgery	50-75	175-200	125	Consultation with a haematologist is essential

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

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

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

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 thromboembolism
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

Inhibitor treatment product type	Inhibitor treatment product name	Indication	Dosage (IV boluses)
Recombinant activated factor VII (7)	Novoseven^®RT	Haemophilia A or B with inhibitor	In minor bleeding: 90 microgram/kg every 2 hours until haemostasis achieved, then wean dose frequency Dosing may need to be modified in children on Emicizumab (Hemlibra) prophylaxis

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)
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