See also

Find a haemophilia treatment centre 
National Haemophilia Guidelines
Haemophilia Foundation Australia 
Von Willebrand disease

Key Points

1. Assessment and investigation should not delay factor replacement
2. If there is ongoing bleeding after adequate factor replacement, consider that the patient has developed a factor inhibitor and treat accordingly
3. All children with haemophilia and bleeding should be discussed with a haemophilia treatment centre 
4. A haemophilia treatment plan should be made, in consultation with a Haematologist, before performing any procedure (eg lumbar puncture) 

Background

• Haemophilia is an X-linked bleeding disorder affecting 1 in 6,000–10,000 males and less than 1 in 300,000 females 
• Haemophilia A is clotting Factor VIII (8) deficiency 
• Haemophilia B is clotting Factor IX (9) deficiency

Assessment

History

• Elicit a detailed description of each type and mechanism of injury. Internal and joint injuries are often missed
• Determine the type of haemophilia (Factor VIII or IX); the child's clotting factor treatment plan and if there are Factor VIII/IX inhibitors
• Check if the child is on a home prophylaxis program and when the most recent treatment was administered

Examination

• Assess the site and extent of bleeding
• Assess the impact on function
• Major or suspected bleeding in the head, neck, chest, gastrointestinal tract and abdomen and/or pelvis should be treated with clotting factor immediately, before a full assessment is complete 

Assessment of severity

Management

Investigations

• Clotting factor replacement should not be delayed by investigations
• Imaging of the site of suspected bleeding is dependent on the mechanism of injury (trauma)
• Routine coagulation studies are not required
  Note: Emicizumab (Hemlibra) affects coagulation testing. Standard coagulation testing will not be accurate and should not be used to monitor APTT, factor VIII or factor VIII inhibitor
• Consider FBE and ferritin in children with a history of recurrent mucosal bleeding, as iron deficiency is common

Treatment

• Most bleeds will require factor replacement with the exception of minor soft tissue injuries and bruising that does not impact on function or mobility
• Prompt clotting factor replacement reduces the pain and long-term consequences of bleeding
• Invasive procedures such as arterial puncture and lumbar puncture must only be performed after clotting factor replacement
• Do not give IM injections

Clotting Factor Replacement

Do not delay clotting factor replacement in severe or life-threatening bleeding

Recombinant clotting factor concentrates are the product of choice. Occasionally children will be treated with plasma-derived concentrates for management of factor VIII/IX inhibitors

• Doses should be rounded up to use whole vials, eg 20 kg child requiring 30 units/kg factor VIII, give 750 units
• The presence of a new inhibitor should be suspected in any child who fails to respond clinically to adequate factor replacement, particularly if the child has been previously responsive
• If the child has factor inhibitors (proven or suspected), a special product is required to ’bypass’ the inhibitor to initiate clotting ie a bypassing agent
• As clotting factor concentrates are not routinely available in all hospitals, children in regional or rural areas should have management plans and ready access to blood products

Note: there are several recombinant Factor VIII (FVIII) products available. Whilst it is recommended that children maintain treatment with their established brand of FVIII product, in an emergency situation administration of any brand of recombinant FVIII is acceptable

See Haemophilia treatments for more information, including the dosing and administration of factor products
Please note that clotting factor concentrates are not routinely available in all hospitals

General measures: joint and muscle bleeds

For muscle and joint bleeds P.R.I.C.E will limit bleeding and reduce pain. Initiate on arrival   

• P = Protection (immobilise the affected area in a position of comfort eg splint/slings/crutches)
• R = Rest
• I = Ice (apply a cold pack to reduce bleeding and pain)
• C = Compression bandage (gentle)
• E = Elevation

Venous Access

• Children with haemophilia will require frequent venous access, this can be a major fear and stressor, so minimisation of unsuccessful or distressing venepuncture is essential
• Use distraction and relaxation techniques and consider nitrous oxide sedation. Ask parents about their preferred method for comforting and distracting their child
• Children with haemophilia are at risk of venepuncture related bleeding. Treat veins with care, apply pressure for at least 3 minutes post venepuncture
• In general, IV cannulas are not left insitu on discharge unless discussed with specialist 

Analgesia

• Do not use products containing aspirin or NSAIDS (eg ibuprofen, diclofenac) as they may worsen bleeding
• Paracetamol may be sufficient. Opiates can be used for severe pain
• Splinting and immobilisation is an effective adjunct for reducing pain

Consider consultation with local paediatric team when

Assessing any child with haemophilia with bleeding episodes.

Note: consult with the haemophilia treatment centre

Consider transfer when

• Child requiring care beyond the level of comfort of the local hospital or treating medical team
• Assessing any child with any of:
  • suspected intracranial haemorrhage
  • bleeding into neck/throat
  • forearm/calf bleed at risk of compartment syndrome
  • bleeding into hip or inguinal area (due to risk of iliopsoas haemorrhage)
  • undiagnosed abdominal pain
  • persistent haematuria
  • bleeding causing severe pain

For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

Consider discharge when

• No active bleeding
• Appropriate follow-up is arranged
• Children are provided with a management plan eg Haemophilia Centre Treatment Card

Last updated May 2023