Haemophilia A and B

D66.7/D66.8

DESCRIPTION

Haemophilia A and haemophilia B are chronic bleeding disorders caused, respectively, by a lack of clotting factor VIII or clotting factor IX.

Sub classification of severity

Class Clotting
factor 		% of normal Signs
Mild VIII or IX 5–40% Occasional bleeds usually
after trauma or surgery.
Moderate VIII or IX 1–5% Less frequent bleeds than
severe usually post trauma/
surgery/ dental extraction.
Severe VIII or IX <1% Spontaneous joint and
muscle bleeds.

DIAGNOSTIC CRITERIA

Clinical

  • Major bleeds:
    • CNS,
    • gastrointestinal tract,
    • severe injury,
    • neck/throat (airway),
    • muscle compartment (e.g.forearm and calf),
    • advanced joint and soft tissue,
    • hip and ilio-psoas.
  • Minor bleeds:
    • early joint bleed,
    • muscle,
    • soft tissue,
    • epistaxis,
    • mouth and gum,
    • haematuria.
  • Pain/tingling in a joint suggests bleeding in a known haemophiliac.

Investigations

  • Prolonged partial thromboplastin time (PTT).
  • Normal INR.
  • Factor VIII or factor IX concentration levels < 40% of normal activity.

GENERAL AND SUPPORTIVE MEASURES

Acute bleeds into joints - Infuse IV factor concentrate first with the following adjunct therapeutic measures:

  • Apply ice packs: 5 minutes on and 10 minutes off.
  • Rest the affected joint/limb until pain free and no further bleeding.
  • No weight bearing.
  • Splint. Do not use circumferential casts.
  • Do not aspirate affected joints.

MEDICINE TREATMENT

For pain (as required):
Do not use NSAIDs and aspirin.

  • Paracetamol, oral, 15 mg/kg 6 hourly.
    If needed:

    ADD
  • Tilidine, oral, 1 mg/kg/dose (1 drop per 2.5 kg 6 hourly).

For bleeds
Emergency treatment while awaiting transfer, if indicated.

If serious bleeding in known haemophiliac, and no factor available:

  • Lyophilised Plasma (Freeze dried), IV, 20 mL/kg. Lyophilised plasma contains a minimum of 0.4 units/mL of each coagulation factor.
    OR
  • Fresh frozen plasma, IV, 20 mL/kg.

Factor VIII deficiency (with no inhibitor present)
Give until patient is pain free. Administration should be 12 hourly for major bleeds, but may be daily for minor bleeds.

Minor bleeds:

  • Factor VIII, intravenous, 20 units/kg.

Major bleeds:

  • Factor VIII, intravenous, 40 units/kg.
    Use the entire contents of the appropriate volume ampoule.

For intracranial bleeds:

  • Factor VIII, intravenous, 40 units/kg 6 hourly.
    Decrease frequency if trough factor level is > 60%, if possible.

Factor IX deficiency (with no inhibitor present)
Give daily until patient is pain free.

Minor bleeds:

  • Factor IX, intravenous, 40 units/kg.

Major bleeds:

  • Factor IX, intravenous, 60 units/kg.
    Available product is factor IX complex and also contains factors II, VII and X.

Home treatment
Home treatment of bleeds is promoted by haemophilia treatment centres. Patients or caregivers are educated on the storage, reconstitution and administration of factor and provided with a supply of factor to be kept at home for use in the event of a bleed. Factor use and bleeding episodes are monitored through the use of an appropriate chart which can be reviewed at consultations and medication collection.

For dental extraction:
Check that inhibitors are absent.
Admit for procedure and post-procedure care and observation.

Haemophilia A:

  • Factor VIII, IV, 40 units/kg, immediately before extraction.
    AND
  • Tranexamic acid, oral, 25 mg/kg/dose 6–8 hourly for 5 days.

Haemophilia B:

  • Factor IX, intravenous, 40 units/kg, immediately before extraction.

For mucous membrane bleeds

  • Tranexamic acid, oral, 25 mg/kg/dose 6–8 hourly.
  • Contra-indicated in haematuria.
  • Use with caution with factor IX complex or factor VIII inhibitor-bypassing activity and preferably only 12 hours after administration of the factor.

REFERRAL

  • All cases with suspected or established haemophilia (prolonged PTT and normal INR), for assessment, genetic counselling and planning of management to a haemophilia treatment centre.