Haemophilia A/Factor VIII Deficiency (No Inhibitors)

Prophylaxis

Treatment on-demand

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ICD-10 code: D66

PROPHYLAXIS

Prophylaxis (primary and secondary) should be considered for patients with severe haemophilia A (<1% factor activity) who can access the health facility twice weekly for infusions, or have indwelling venous catheters and are candidates for home-based care.

Primary prophylaxis:	Prophylaxis started in the absence of documented joint disease/damage.
Secondary prophylaxis:	Prophylaxis initiated after joint damage has occurred

• Factor VIII, intravenous 25 units/kg, twice weekly.

Proposed rounded dosing for paediatric patients
The clotting factor should be rounded to the nearest full vial to avoid wastage.

Age in years	Average weight (kgs) & IU required per dose	Rounded dose (IU)	Available products
0-1 years	8.0 kgs, 200 IU	300	1 x 300IU
1-2 years	11 kgs, 275 IU	300	1 x 300IU
2-3 years	13.5 kgs, 337.5 IU	500	1 x 500IU
3-4 years	15.5 kgs, 387.5 IU	500	1 x 500IU
4-5 years	17.5 kgs, 437.5 IU	500	1 x 500IU
5-6 years	19.5 kgs, 487.5 IU	500	1 x 500IU
6-7 years	22 kgs, 550 IU	600	2 x 300IU
7-8 years	24 kgs, 600 IU	600	2 x 300IU
8-9 years	27 kgs, 675 IU	800	1 x 300IU plus 1 x 500IU
9-10 years	30 kgs, 750 IU	800	1 x 300IU plus 1 x 500IU
10-11 years	34 kgs, 850 IU	1000	2 x 500IU
11-12 years	38 kgs, 950 IU	1000	2 x 500IU
>12 (adults)	50 kgs, 1250 IU	1300	2 x 500IU plus 1 x 300IU
>12 (adults)	60 kgs, 1500 IU	1500	3 x 500IU
>12 (adults)	70 kgs, 1750 IU	1800	3 x 500IU plus 1 x 300IU

TREATMENT ON-DEMAND

Minor bleeds:

Bleeds into the muscle or soft tissue, mouth or gums, epistaxis, painless haematuria and early joint bleeds.

• Factor VIII, intravenous, 20 – 40 units/kg
  • If there is evidence of ongoing bleeding after 12 hours, consult with a local haemophilia treatment centre.

Major bleeds:

Advanced muscle or joint bleeds result from severe injury or bleeds that affect the central nervous system, gastrointestinal system, neck or throat, hip or iliopsoas muscle, or forearm compartment.

• Factor VIII, intravenous, 40 - 50 units/kg.
  • Use all the contents of the appropriate volume ampoule.
  • All of these patients need hospitalisation.
  • Discuss all patients promptly with the local Haemophilia Treatment Centre.

LoE: III³

Intracranial bleeds

• Factor VIII, intravenous, 40 – 50 units/kg 6 hourly.

Decrease frequency if the trough factor level is >60%, if possible.

Click here to access the pdf.

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Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_ Updated February 2025

Chapter 3: Blood and Blood Forming Organs, 3.6 Haemophilia A and B, 3.6.1 Haemophilia A/Factor VIII Deficiency (No Inhibitors)