Haemophilia is a group of inherited blood disorders
that affect the bloods ability to form clots. Protein factors in white
blood cells clot and form scabs on cuts, so without them a could bleed to death
from a relatively minor cut. People who have haemophilia require transfusions
of blood plasma and blood products in order to maintain a suitable level of
blood clotting ability.
There are two main types of haemophilia: type A
and type B. Type A is the most common, and is caused by a lack of the plasma
protein factor VIII, which aids blood clotting. It is an inherited disorder
carried through the female line but affects men at the rate of one in every
10,000. Half of the daughters of a female carrier will carry the defective gene,
and all of the female children of a male haemophiliac will be carriers.
Type B haemophilia is also sometimes called Christmas
disease. It is seven times rarer than type A haemophilia and is caused by a
lack of a different plasma protein, factor IX. It also is an inherited disorder,
and is carried in the female line in the same manner as Type A haemophilia.
are the symptoms of haemophilia?
Haemophilia is a spectrum complaint, meaning that
the symptoms can vary significantly from person to person in their severity.
The most significant symptom of either type of haemophilia is bleeding, which
may go unnoticed in early childhood if it is mild.
As the child grows older, bleeding becomes more
manifest, especially if the child undergoes surgery. Internal bleeding may occur
anywhere in the body, and in the joints. Nosebleeds, bruising, spontaneous bleeding,
haemorrhaging and excessive bleeding from cuts or during tooth extraction are
all signs of haemophilia.
can haemophilia be treated?
Unfortunately, there is no cure for haemophilia.
The only way to prevent haemophilia would be genetically to screen potential
female carriers of the defective chromosome.
The disorder can be held in check and treated,
however. The defective clotting factor, whether factor VIII or factor IX, can
be replaced by an infusion of factor concentrates extracted from donated blood.
People with haemophilia can be given instructions on how to administer blood
factor products at home, but infusions are often done in hospital.
With regular infusions, haemophiliacs can lead
relatively normal healthy lives. It is recommended that diagnosed haemophiliacs
receive vaccinations against hepatitis.
In the recent past, contaminated blood products
were given to haemophiliac patients in Ireland, leading to many deaths. The
Lindsay Tribunal is currently investigating this scandal. Blood products in
Ireland are now carefully screened for any possible infection.
did Irish haemophiliacs come to be infected by blood products in the past?
In the 1970s and 80s the Irish blood transfusion
service often had to import certain blood products due to a shortage of supply
in Ireland. Some of these blood products were infected with HIV and hepatitis
C, and were given to Irish haemophiliacs who went on to develop these diseases,
causing serious illness and death.
Today, blood and blood products are carefully and
vigorously screened for any possibility of infection. This is a procedure known
as haemovigilance. However, in the past, screening was not as advanced as it
is today, and there appear to have been crucial lapses of vigilance on the part
of the Blood Transfusion Service Board that are currently the subject of a tribunal.
was the Lindsay Tribunal?
The Lindsay Tribunal was the body established
by the government to investigate why serious infection from blood products
in Ireland. It first sat in September 1999 and started hearings in May 2000.
These hearing ended in November 2001.
The tribunal was chaired by Judge Alison Lindsay
- her report was finally published in September 2002. She concluded that
blood products should be of the highest standard and safety and that this
is the situation now. Her report said that the bulk of the evidence heard
at the tribunal was historic in nature and that 'great changes' have taken
place in personnel, facilities and procedures since.
Judge Lindsay decided not to refer her report
to the Director of Public Prosecutions or to make any recommendation on the
need for another tribunal to examine the role of American pharmaceutical
companies - moves recommended by the victims. At the time of the publication
of the report, 78 haemophiliacs had died from the contamination scandal,
with hundreds more infected.
A separate compensation tribunal was initiated
in March 1996. As of mid-2000 the State had paid out nearly €2.5 million
to over 1,300 people who were infected, and their relatives.
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