Haemophilia

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

Read the full PCH Emergency Department disclaimer.

Aim 

To guide staff with the assessment and management of haemophilia.

Background 

  • Most children with severe haemophilia receive prophylactic factor replacement starting between ages 1 - 2 years. This is given intravenously, one to three times weekly for severe Haemophilia A or 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 Haematologist before any treatment is instituted
  • Please refer to Haematology Transfusion Medicine Protocols (WA Health internal access only) 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.

Management

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%.

Prophylactic treatment

  • 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, these patients may occasionally present with spontaneous joint bleeding and require additional factor therapy
  • Approximately 10% of patients with Haemophilia A may develop inhibitors to treatment – this usually 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.

Head injury

  • Treatment should be regarded as an emergency.
  • Give factor VIII 50 Units/kg IV 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.

Head injury

  • The same principles apply as for haemophilia A – give factor IX 50 units/kg IV urgently.

Bibliography

  1. AMH Children’s Dosing Companion (2015) Australian Medicines Handbook Pty Ltd
  2. Textbook of Paediatric Emergency Medicine 2nd Edition Cameron Elesevier 2012
  3. Nelson Textbook of Pediatrics: 20th Edition Robert M. Kliegman, Bonita M.D. Stanton, Joseph St. Geme, Nina F Schor Publisher: Elsevier 
  4. Textbook of Pediatric Emergency Medicine, Fleisher, Gary R. Ludwig, Stephen.  6th Edition. 2010
Reviewer/Team: Meredith Borland (ED Director), Dennis Chow (ED Consultant), Deirdre Speldewinde (ED Consultant), Gabrielle Anstey (ED CNS), Craig Hasler (ED CNM)
Last reviewed: Apr 2018


Review date: Apr 2021
Endorsed by:

Director, Emergency Department
Date:  Apr 2018


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