Paediatric Acute care Guidelines PMH Emergency Department

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 Bleeds

Paediatric Bleeding Disorder Pathway 

Life Threatening

Serious

Minor

Intracranial bleed

Neck/Throat injury

Abdominal bleed

Muscular bleeds

Joint bleeds

Mucosal

Superficial bruises

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 PMH Transfusion Medicine Protocols

Haemophilia A:
Recombinant Factor VIII: Xynthas, Advate
Plasma-derived Factor VIII/VWF: Biostate

Haemophilia B:
Recombinant Factor IX: BeneFIX

vion 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 eg. Infected central line, haematoma or joint bleed

Cultures & CRP

Antibiotics as per Champ guidelines – with/without infusaport

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