Canterbury DHB

Context

General Principles of Treatment

In This Section

Specialist Management

Terminology

Pain Relief

Acute Joint Bleeding

Recovery

Recording Treatment

Vaccinations

Specialist Management

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

Severity

Factor VIII or IX activity

Severe

<1%

Moderate

1-5%

Mild

>5%

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 BeneFIX because of the lower recovery.

Pain Relief

Pain relief must be adequate, especially for large joint and muscle bleeds.

Acute Joint Bleeding

Minor joint bleeds should be treated with ice application, analgesics and rested 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.

Recovery

Dosing

Recording Treatment

Patients should be encouraged to keep a record of all bleeding episodes and details of their product usage.

Records of product use will be kept at each treatment centre.

About this Canterbury DHB document (5234):

Document Owner:

Mark Smith (see Who's Who)

Issue Date:

August 2016

Next Review:

August 2019

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 5234