Bleeding disorders

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 management of a child with a bleeding disorder.

Background

  • Haemophilia A – Factor VIII deficiency
  • Haemophilia B – Factor IX deficiency
  • von Willebrand disease – vWF deficiency / dysfunction
  • Other bleeding disorders

Presentation

• Injury or bleeding
• Fever – be wary of central line infections in haemophilia patients with a Central Venous Access Device. 

Management of a child with a bleeding disorder - ED pathway

Management of a bleed

Life Threatening
Serious
Minor
Intracranial bleed
 Muscular bleeds  Superficial bruises
Neck/throat injury
 Joint bleeds  
Abdominal bleed  Mucosal  

Life threatening and serious bleeds should be ATS category 1 or 2

Life threatening

  • Manage in resuscitation room
  • Call haematology / oncology fellow/consultant immediately
  • Give Factor without delay

Serious

  • Give Factor without delay
  • RICE = Rest, Ice, Compression, Elevation and Immobilisation
  • Analgesia – paracetamol/oxycodone – NOT Aspirin / NSAIDs
  • Ultrasound / Xrays as clinically appropriate
  • +/- fast child and contact rheumatologist on call if joint bleed.

Minor

  • Assess and treat as clinically indicated

Treatment

DO NOT DELAY ADMINISTRATION OF FACTOR

DO NOT ACCESS INFUSAPORT IF INSERTED WITHIN LAST 5 DAYS (unless under instruction of Haematologist).

Clotting Factor Replacement should be administered within 15 – 30 minutes.

Do NOT delay Factor replacement for diagnostic imaging or extensive clinical assessment

Give Factor PRIOR to intervention.

Every child known to PMH will have a treatment card which details:
  • Type of bleeding disorder.
  • Treatment product (including brand name) and if on regular replacement.
  • Presence of Factor VIII/IX inhibitor.
  • If the patient does not present with the treatment card the parents or patient should know these details.

Factors

Available from Blood Bank. Further information can be found at PCH Transfusion Medicine Protocols.

Haemophilia A:
  • Recombinant Factor VIII: Xynthas, Advate
  • Plasma-derived Factor VIII/VWF: Biostate
Haemophilia B:
  • Recombinant Factor IX: BeneFIX
von Willebrand Disease treatment (for non-responsive to DDAVP):
  • Plasma-derived Factor VIII/vWF Biostate
Patients with Inhibitors:
  • Recombinant VIIa: NovoSeven
Other Products available from pharmacy:
  • Desmopressin Acetate (DDAVP)
  • Cyklokapron (Tranexamic Acid), Antifibrinolytic Therapy

Management of Fever

  • Look for source of fever e.g. Infected central line, haematoma or joint bleed.
  • Cultures & CRP
  • Antibiotics as per ChAMP (Children’s Antimicrobial Stewardship Program) guidelines – with/without infusaport.

Bibliography

  1. Textbook of Paediatric Emergency Medicine 2nd Edition Cameron Elesevier
  2. Nelson Textbook of Pediatrics: 20th Edition Robert M. Kliegman, Bonita M.D. Stanton, Joseph St. Geme, Nina F Schor Publisher: Elsevier

Endorsed by:  Director, Emergency Department  Date:  Feb 2017


 Review date:   Feb 2021


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