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

Textbook of Paediatric Emergency Medicine 2nd Edition Cameron Elesevier
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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