South African Haemophilia Foundation (SAHF) Medical and Scientific Advisory Council (MASAC) Treatment Guidelines - Treatment of Bleeding Episodes

Treatment of Bleeding Episodes

Always refer to the Haemophilia Treatment Centre Physician’s Instructions

Please refer to the Section on: Products Available in South Africa
Please Note: Patients with Inhibitors require Special Treatment

Factor VIII replacement for Haemophilia A, no inhibitor

Dose depends on bleeding severity
  • Minor bleed: 20 – 40 IU/kg
  • Major bleed: 40 – 50 IU/kg
Expected response
  • 1 IU/kg = 2% rise in Factor VIII level
Half life Factor VIII
  • 8-12 hr

For serious bleeding Factor VIII assay may be required to monitor the response to the infusion.

If there is no response to appropriate replacement therapy, test for inhibitors.

Please note:

The Haemophilia Treatment Centre physician chooses the most suitable product for each patient. Please follow these recommendations.
Plasma-derived Factor VIII is treated with heat or solvent/detergent to inactivate viruses.
Round off dose to the nearest vial; do not discard excess Factor VIII but rather infuse it.
Repeat doses may be required depending upon the severity of bleeding: always needed for major bleeds every 12 - 24 hours.

Please note:

The Haemophilia Treatment Centre physician chooses the most suitable product for each patient. Please follow these recommendations.
Plasma-derived Factor IX concentrates are treated with solvent/detergent to inactivate viruses.
Factor IX Complex [Prothrombin complex concentrate (PCC)] also contain Factors II, VII and X (can reverse the effects of warfarin).
NB: thrombosis or disseminated intravascular coagulation may occur with frequent or large doses of PCC.

Factor IX replacement for Haemophilia B, no inhibitor

Dose depends on bleeding severity
  • Minor bleed: 20 – 40 IU/kg
  • Major bleed: 60 – 80 IU/kg
Expected response
  • 1 IU/kg = 1% rise in Factor IX level
Half life Factor IX
  • 16-24 hr

For serious bleeding Factor IX assay may be required to monitor the response to the infusion.

If there is no response to appropriate replacement therapy, test for inhibitors.

After Joint or Muscle Bleeds
Rehabilitation Exercises

After Joint or Muscle Bleeds

Rehabilitation after a bleed is essential to maintain strength and range of motion.
When to start rehabilitation exercises?
  • As soon as the pain is gone.
What exercises?
  1. Static exercise.
  2. 3 days after resolution of the bleed: free active exercises where the only resistance is gravity.
  3. 10 days after the resolution of the bleed: weight bearing exercises to build up muscle strength and bulk.
Hepatitis
Transfusion acquired infection

Hepatitis

Any person with Haemophilia and related bleeding disorders may have transfusion acquired infection.
  • Test annually for HAV, HBV, HCV.
  • Antibody/antigen negative patients should be immunised, and response assessed.
  • Active infection should be excluded in positive patients.
  • Patients with chronic active hepatitis should be referred to a Hepatologist for management.
NB: Patients with hepatic dysfunction may have other Factor deficiencies (test PT or INR) or a low platelet count.