Introduction.
- Haemophilia A is the commonest of the inherited disorders of blood clotting
and occurs almost exclusively in males in its active form. It affects 1:4000
of the population of Britain, although only 1:20,000 is severely affected.
Other similar disorders giving rise to a bleeding tendency are Christmas
disease (also known as Haemophilia B) and von Willebrand's disease.
Haemophilia B has very similar effects to haemophilia A, while von
Willebrand's disease usually follows a milder course.
- The conditions are due to deficiencies in the blood clotting mechanism and
are characterised by a life-long tendency to excessive bleeding. In
haemophilia A there is either a seriously reduced level (less than 5%) or
total absence of Factor VIII and in haemophilia B a similar lack of Factor
IX. The effect of this is greatly to increase the time it takes for blood to
clot, so that the affected individual may bleed seriously after minor injury or
in some cases, even after normal physical activity. The knee joint is most
frequently affected because of the damage done to the joint and its
surroundings by blood loss into the joint..
- The conditions are treated by replacement of the missing clotting factor by
intravenous injection, either at regular intervals or in emergencies caused by
bleeding episodes. Usually, affected individuals are provided with a supply
of the appropriate clotting factor and are taught to inject themselves
immediately bleeding occurs. If there is bleeding into a joint (such as the
knee) all that is usually required is for the joint to be rested for a few days
until it has settled down. Some people attend hospital for their injections.
Care Needs.
- Adults should have a clear understanding of their condition and the need to
avoid injury. They should not normally need supervision to avoid the
danger of precipitating bleeding episodes.
- Adults are taught to give themselves the clotting factor and they should not
normally require attention for this reason. If help is required because of
mental impairment or arthritis of the hands affecting manual dexterity, etc.
the need for injections is usually intermittent and infrequent. Even if
bleeding is more frequent so that help is needed more often, the time taken
to prepare and give an injection is not great and no other help should be
needed. Supervision is not likely to be required as the person can ask for
assistance when this is necessary. Watching over at night is unlikely to be
necessary.
- Repeated bleeding into joints may lead to permanent damage and the care
needs may then be similar to someone with extensive inflammatory or
degenerative arthritis.
Mobility Considerations.
Repeated bleeding into lower limb joints may lead to the development of
arthritis with a consequent effect on walking ability. For those individuals
with severe haemophilia A (less than 5% Factor VIII) or severe forms of
haemophilia B there may be a significant danger of spontaneous and serious
bleeding into joints during normal walking. This will need to be taken into
account when mobility needs are being considered.
Duration of Need.
If the risk of severe bleeding when walking has been established or severe
joint damage has occurred the need for help will be permanent. However in
some individuals knee joint replacement is an option which, when
successful, considerably improves function at the affected joint.
Further Evidence.
People with haemophilia are invariably under the care of the haematology
department of a hospital. A factual report from the hospital may help to
establish the danger of a spontaneous bleed when walking and a history of
bleeding episodes and any resulting disability.
Haemophilia and Human Immunodeficiency Virus Infection.
Before 1985 1200 people with haemophilia were infected by preparations of Factor
VIII contaminated with the HIV virus. Unfortunately a number of these people
have developed symptomatic HIV infection. Care and mobility needs may result
from the effects of haemophila and symptomatic HIV infection.
