Haemophilia B (or Christmas disease) is caused by a deficiency of a protein
in the blood; Factor IX. Haemophilia B is less common than Haemophilia A.
The symptoms experienced by those affected are similar to those with Haemophilia
A.
The level of Factor IX in the blood determines the severity of Haemophilia
B. Factor Levels of less than 1-2% are found in people with severe haemophilia
and they experience spontaneous bleeds into joints or muscles. Moderate haemophilia
is found in people with factor levels of 2-5%, they may experience spontaneous
bleeds into joints and muscles. Or those with mild haemophilia, who have factor
levels of between 5-50%, usually only experience bleeds following trauma or
surgery.
Children with severe Haemophilia B often begin to show increased bruising
around the age of one year. It may present earlier if the child under goes
surgery, or after minor trauma, including immunisations.
The major problem in severe Haemophilia B is painful bleeding into joints,
mainly the knees, ankles and elbows. These bleeds may occur spontaneously.
If left not treated promptly the bleeds may result in permanent arthritis and disability.
Most children and adolescents with severe Haemophilia B receive preventative
treatment (prophylaxis) with an intravenous infusion of Factor IX, 2 times
a week. This is to prevent spontaneous joint and muscle bleeds.
Most persons with Factor IX deficiency requiring treatment will have a synthetic type of Factor IX called Recombiant Factor IX.
With appropriate management and factor replacement children and adults with
inherited bleeding disorders can lead active and normal lives.
This page last updated: November 2004
Review date: June 2005
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© The State of Queensland (Queensland Health) 2008.