Introduction

What is Haemophilia?

The word haemophilia originates from the Greek term haemo or hamia, for blood and philia which means friendship, or affinity. It is a genetic disorder that carries through the X-chromosome and transfers through the females, but almost always manifests itself in males. Haemophilia was discovered by Dr. John Conrad Otto in 1803, when he discovered a: “hemorrhagic disposition existing in certain families.”

There are various types of the disorder, from haemophilia A or 'classical' haemophilia where sufferers are deficient in the clotting agent Factor VIII. Haemophilia B or 'Christmas disease' effects people who are deficient in Factor IX. Haemophilia C is a milder form of the disorder and affects 1 in 100,000, and does not lead to bleeding into the joints as with A and B.

Diagram of haemophilia

Image courtesy of US National Institute of Health

A further type of blood disorder associated with haemophilia is von Willebrand disorder, named after Erik Adolf von Willebrand, a Finnish paediatrician who first published his knowledge of the disease in 1926. Von Willebrand’s is less severe than haemophilia A and B, but more severe than C. It manifests itself in women as well as men, so menstruation and childbirth can result in a higher loss of blood.

According to the UK Haemophilia Society website, there is contention over whether or not women can have haemophilia, “the levels of many women who carry haemophilia are low enough that they would be considered to have haemophilia if they were a man. For that reason we tend to say that while the textbooks might say that only men can have haemophilia we believe that if a women wants to describe herself as having haemophilia then she does.”

What is contaminated blood?

The term contaminated blood describes a series of events throughout the 1970s and 1980s that resulted in thousands of haemophiliacs around the world being infected with various diseases ranging from different forms of hepatitis, to HIV to vCJD, the human form of “mad cow disease”.

Famous haemophiliacs

Queen Victoria famously

carried haemophilia but was never showed symptoms. Her offspring famously passed haemophilia onto various Royals across Europe.

Tsarovich Alexei Nikolaevich suffered from haemophilia B and was treated by the mystic, 'mad monk' Grigori Rasputin.

Mother Teresa is rumoured to have suffered from mild haemophilia

Richard Burton discovered he was a mild haemophiliac after kicking a television

In the 1970s, as American pharmaceutical corporations gathered blood from donors across the country, they were not monitored nor were there the currently accepted regulations in place to make sure that they were not collecting diseased blood. Thus some high profile drugs companies sourced the blood from prisoners, homosexuals and heroin addicts - high risk groups for transmittable diseases such as HIV and hepatitis B and C - in exchange for money. They then pooled the blood, making it into products that were distributed throughout the world.

The Arkansas Prison Blood Scandal, an American prison medical administrator John Byus claims that the FDA regulations of blood only spanned “recommendations” but that when accusations that their prison blood farming caused the epidemic, “nothing has been proven”.

In the 1970s and 1980s when there were blood shortages in the UK that were needed in order to use blood plasma to make the emerging haemophilia treatments Factor VIII and Factor IX, the NHS imported this commercial, unscreened American blood.

Thus over 4,800 British haemophiliacs were given these various diseases.

In 2006, an independent inquiry was set up by Lord Archer of Sandwell, and in 2009 it concluded that the NHS and Department of Health made serious mistakes when importing American blood products and treating American patients.

Lord Archer recommended that the government review its compensation programme to victims of contaminated blood, and that it re-evaluate its evaluation that the Irish government was flawed when it compensated its contaminated blood haemophiliacs.

Introduction

What is Haemophilia?

What is contaminated blood?

Famous haemophiliacs

WEEKLY NEWS

Cameron backs Skipton Fund review

Contaminated blood film to denounce Factor VIII

Protest comes as FDA imposes blood fine

Government backs down on judicial review