How to Treat Haemophilia?


Things to note:
  • Haemophilia register
  • Ideally, patients should attend a specialised haemophilia centre with a dedicated multi-disciplinary health care team
  • Medic alert bracelet
  • Dental care (see below for management of tooth extraction)
  • Avoid contact sport
Acute bleeds into joints
  • Apply ice packs
  • Bed rest
  • Rest the affected joint/limb until pain free and no further bleeding
  • No weight bearing.
    Splint (no circumferential casting)

Medical Treatment:
For mild to moderate pain:
  • Paracetamol, oral, 1 g 4-6 hourly when required to a maximum of 4 doses per 24 hours.
If needed:
ADD
  • Tramadol, oral, 50mg, 6 hourly.
For severe pain:
  • Morphine, IV, 10 mg 4 hourly.
Exercise great caution when taking blood specimens.
Taking blood from femoral veins is absolutely contra-indicated.
Avoid IM injections. Avoid aspirin and NSAIDS.
HAEMOPHILIA WITH NO INHIBITORS
  • The dose of the factor VIII and IX is individualised as it is dependent on body mass, severity of the condition, and the nature and site of the bleeding.
Dental extraction
Check that inhibitors are absent.
In haemophilia A:
  • Lyophilised factor VIII concentrate, IV, 40 units/kg immediately before extraction.
In haemophilia B:
  • Lyophilised factor IX concentrate, IV, 40 units/kg immediately before extraction.
  • Tranexamic acid, 250 mg disolved in 10 mL of water.
    • Rinse mouth for 2 minutes 6 hourly.
Mucous membrane bleeds
  • Tranexamic acid, oral, 1 g 6 hourly.
    • Contraindicated in haematuria or in patients with thrombotic tendencies.
In mild von Willebrands disease or established responders of mild factor VIII deficiency:
  • Desmopressin, IV, 0.3 mcg/kg in at least 30 mL sodium chloride 0.9% administered over 30 minutes.
Emergency treatment while awaiting transfer, if indicated
If serious bleeding with known haemophilia, and no factor VIII available:
  • Fresh frozen plasma, IV, 10-20 mL/kg
HAEMOPHILIA WITH INHIBITORS
  • Refer for assessment and planning with a haematologist
  • Factor VIII inhibitor-bypassing activity (FEIBA) - under haematologist supervision only
VON WILLEBRANDS DISEASE
Mild bleeding
E.g. epistaxis and menorrhagia.
Antifibrinolytics, e.g:
  • Tranexamic acid, oral, 1 g 6 hourly
  • Recurrent menorrhagia can also be treated effectively with oral contraceptives
More severe mucous membrane bleeding
For mild von Willebrands Disease, which occurs in 80% of patients:
  • Desmopressin, IV, 0.3 mcg/kg in at least 30 mL sodium chloride 0.9% administered over 30 minutes.
Note:
  • Desmopressin is not effective in type 3 and the majority of type 2 von Willebrands disease
  • Intermediate-purity factor VIII concentrates, which contain both von Willebrand factor and factor VIII, may be used for patients with very low von Willebrand factor levels
  • During surgery or after major trauma, patients should receive:
    • Cryoprecipitate, IV, 1 unit/10 kg 12 hourly.
OR
  • Lyophilised factor VIII concentrate, IV, 30-50 units/kg/dose given every 12 hours
  • Continue for 48-72 hours to ensure optimal haemostasis
  • For major surgical procedures, use for 7-10 days
  • Antifibrinolytic agents may be used in combination with desmospressin or von Willebrand factor containing concentrates (cryoprecipitate or factor VIII) to treat bleeding episodes


When to refer:
  • All cases with suspected haemophilia (prolonged PTT and normal INR) to a haemophilia treatment centre, for assessment, genetic counselling and planning of management
  • Patients with proven antibodies against factor VIII
  • For further replacement, complex situations and complications in consultation with a haematologist.