Also see guideline
Management of a Child with a Bleeding Disorder Background
Most children with severe Haemophilia are treated with prophylactic factor replacement starting between age 1 – 2 years. This is given intravenously, one to three times weekly for severe Haemophilia A, twice weekly for severe haemophilia B. Recombinant factor (Advate or Xyntha – factor VIII; Benefix – factor IX) is the treatment of choice.
Supplies of recombinant factor VIII and factor IX are kept in the fridge in Transfusion Medicine (Haematology laboratory).
All presentations to the Emergency Department should be discussed with the on-call Haematology/Oncology Fellow or Haematologist. Please refer to
Haematology Transfusion Medicine Protocols for further information. Mild Haemophilia is not usually treated with prophylactic factor replacement but can still be a cause of severe bleeding problems and may require urgent factor replacement therapy or DDAVP.
Haemophilia A: Factor VIII deficiency
This is a sex-linked condition due to factor VIII deficiency. Severe cases have <2% factor VIII, moderate cases 2-5% and mild >5%.
Generally started in severe cases over 12 months of age
The main reason for prophylactic treatment is the prevention of haemarthroses, and this becomes more likely as the child starts walking, usually around 12 months of age.
Despite prophylactic treatment, occasionally these patients may present with spontaneous joint bleeding and may require additional factor therapy.
Approximately 10% of patients with Haemophilia A may develop inhibitors to treatment – usually this occurs in the first ten treatments
Treatment of patients with inhibitors is difficult and may require bypassing agents such as factor VIIa.
Mucosal bleeding is a common problem in young children and can be treated with antifibrinolytic therapy using Tranexamic Acid.
The dose is 15-20mg/kg/dose given TDS orally. Tranexamic Acid comes as 500 mg tablets. For children:
< 25 kg give half a tablet TDS
25-35 kg give one tablet TDS
35-50kg give one and a half tablets TDS
> 50kg give two tables TDS
Advice about specifics of treatment should be sought from a Clinical Haematologist
Treatment should be regarding as
emergency and a dose of factor VIII 50 Units/kg IV given urgently For minor knocks to the head, such as contact with furniture or doors, factor treatment does not need to be administered
Admit to hospital if there is any suspicion of concussion or persistent headache or vomiting
Haemophilia B (Christmas Disease): Factor IX deficiency
This is a sex-linked condition with factor IX deficiency. The presentation is identical to haemophilia A with similar factor levels.
The treatment of choice is recombinant Factor IX (Benefix)
Most patients with severe factor deficiency are treated with prophylactic infusions, given twice weekly
Inhibitors to factor IX are less common but can present with allergic manifestations
The same principles apply as for haemophilia A – give factor IX 50 units/kg IV urgently.
External Review: Catherine Cole (Paediatric and Adolescent Oncologist/Haematologist) – July 2015
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Author / Reviewer
Kids Health WA Guidelines Team
Dr Meredith Borland HoD, PMH Emergency Department
30 July, 2015
30 July, 2015