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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.
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.
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.
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.
The Lindsay Tribunal was the body established by the government to investigate why serious infection from blood products arose 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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Last Reviewed: 23rd August 2001
|Clio36 Posted: 19/06/2009 17:54|
HI All, I'm new to this forum but hope someone can advise me. I have an 8 yr old daughter with level 2 Von Wilebrands Disease. Does anyone have a son or daiughter/relative with the same? She is currently going through a reoccurance of nose bleeds which I can only imagine is part and parcel of the disease. We are hoping to get her referred to a haemotologist soon for an check up as its been been 5 years since her last one! Any feedback or similiar stories would be greatly appreciated! Thanks!
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