General Principles of Treatment

All patients with haemophilia, or other significant bleeding problems, should be registered with a Regional Haemophilia Centre and reviewed by a specialist haematologist on a regular basis. The frequency of review will depend on the severity of the haemophilia.

Terminology

Note: The plasma concentration of factor VIII or IX does not always correlate with the clinical severity of the disease. Some patients with factor concentrations between 1% and 5% have clinically severe disease.

FVIII: Refers to all types of Factor VIII products used to treat patients with factor VIII deficiency. This may be of recombinant or plasma derived.

FIX: Refers to all types of the Factor IX product used to treat patients with factor IX deficiency. It may be of recombinant or plasma derived. The dosing schedules are those generally recommended for plasma derived factor IX. Higher doses may be needed for patients receiving recombinant factor IX because of the lower recovery.

Pain Relief

Acute Joint Bleeding

Treat minor joint bleeds with ice application, analgesics, and resting them in a functional position.

Recovery

The factor VIII (or IX) level measured after a given dose of the specific factor can be expressed as (factor) “recovery”. This should be reviewed at least every two years but more frequently during rapid growth phases in childhood and adolescence, or where the presence of an inhibitor is suspected.

Dosing

Recording Treatment

Encourage patients to keep a record of all bleeding episodes and details of their product usage.


About this Canterbury DHB document (5234):
Document Owner:	Sean Macpherson (see Who's Who)
Last Reviewed:	November 2019
Next Review:	November 2022
Keywords:
Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer The Haematology Department Protocols and Guidelines (the Red Book) are intended as a guide for registered health professionals to communicate guidelines and share best practices with supporting health professionals within New Zealand district health boards. They are not intended to be provided to or used as a reference for patients or non-registered health professionals. All health professionals must exercise their own clinical judgement and use pertinent clinical data when treating patients. The authors and editors of this publication have checked with sources believed to be reliable in their effort to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical science, neither the authors, editors, publisher, nor any other party who has been involved in preparing or publishing this work warrants that the information contained in it is accurate or complete in every respect. They therefore have no liability (whether in tort (including negligence) or otherwise) for any loss or damage arising out of any reliance on this information. This publication has been compiled by the staff and contractors of Canterbury District Health Board (CDHB) and as such CDHB has copyright for this book. Amendments must be authorised in accordance with the documented process. No release of part of, or all of, this publication to any other party or organisation is permissible without the prior approval of the designated document owner.
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Severity	Factor VIII or IX activity
Severe	< 1%
Moderate	1 to 5%
Mild	> 5%