Prophylaxis
This refers to the infusion of blood or recombinant products in anticipation of or in order to prevent bleeding. This contrasts with on-demand therapy given at the first sign of a bleed.
Prophylaxis may be:
- Primary (Long Term) Prophylaxis: Given to infants identified as being at high risk of recurrent bleeding into target joints and at risk of arthropathy. Primary prophylaxis is usually reserved for the very severe cases with factor VIII or factor IX of ≤1% of normal, as people with factor concentrations above 1% rarely develop disabling arthropathy. Regular prophylaxis is introduced after one or two severe bleeding episodes within the first 1 to 2 years of life. The aim is to minimise acute bleeding episodes. This is normally achieved with treatment given twice (factor IX) or three times (factor VIII) weekly. Factor usage corresponds to 2000-3000 IU/kg/year.
- Single Dose Prophylaxis: An injection of product may be given prior to an event (e.g. sporting) to prevent bleeding occurring in relation to that activity.
- Secondary Prophylaxis: Refers to limited term prophylaxis where there is a high requirement for on demand therapy. Regular injections over a limited time period may reduce the frequency of bleeding or re-bleeding from a target joint. Often used in the treatment of a chronic synovitis.
Who should receive prophylaxis?
Primary prophylaxis is standard treatment for infants with severe haemophilia (<1% factor) who declare themselves at high risk for the development of haemophiliac arthropathy.
When should treatment start?
In general, treatment is commenced within the first 12 to 24 months of life. It is normally commenced following the first significant spontaneous bleed (joint or soft tissue). A Port-a-Cath device may be needed to facilitate vascular access.
Short-term prophylaxis may be required for a patient with recurrent bleeding into a target joint.
Topic Code: 5236
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