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What is haemophilia?

Haemophilia A is caused by a deficiency in clotting factor 8, Haemophilia B by a deficiency in clotting factor 9. The deficiency is genetic and carried on the X chromosome, generally affecting only males although about 30% of female carriers may have some mild symptoms.

Depending on how much clotting factor is missing, the condition can be mild, moderate or severe. In severe haemophilia spontaneous and prolonged internal bleeding occurs, characteristically into joints and muscles. Prompt and effective treatment to stop the bleeding is required to minimise long term damage.

The Haemophilia Society was established in 1950. It is the national charity supporting people with haemophilia, Von Willebrand's Disease and other inherited bleeding disorders. The Haemophilia Society provides education and information on these conditions.

What is Von Willebrand's disease?

Von Willebrand's Disease (vWD) is a more common bleeding disorder than haemophilia and affects both males and females. Von Willebrand's Disease is caused by a deficiency in Von Willebrand's factor which has an important role in blood clotting, including stablising factor 8 and sticking platelets together to form a blood clot.

In the mild condition bruising and prolonged bleeding, especially from the nose, mouth and gut and also heavy periods are characteristic. In the rare severe form, Von Willebrand's Disease can also result in the same symptoms as severe haemophilia.

The Haemophilia Society was established in 1950. It is the national charity supporting people with haemophilia, Von Willebrand's Disease and other inherited bleeding disorders. The Haemophilia Society provides education and information on these conditions.

How is haemophilia treated?

There was no effective treatment for haemophilia until the late 1950s. Cryoprecipitate, made from human plasma, was the first effective therapy and was refined into a freeze dried concentrate in the 1970s.

The possibility of intravenous self-administration of factor concentrates to treat and prevent bleeding episodes reduced pain and disability and the need for frequent hospital visits and resulted in an improved independent life style for people with haemophilia. The manufacture of concentrates however required thousands of blood donations and prior to the introduction of heat treatment in 1985 resulted in contamination of treatment with HIV and viral hepatitis.

Recombinant treatment used today for people with haemophilia is synthetic and does not require donated blood. People with severe forms of VWD still require concentrates made from blood which are now made safer with viral inactivation measures.

What is the difference between HIV and AIDS?

HIV is a virus that replicates in one of the most important cells of the immune system; the T4 helper cell. As the virus replicates it reduces the amount of T4 cells and over many years the immune system become less able to fight infections and cancers that occur within the body.

AIDS is the syndrome that happens to people when their immune system has been severely damaged by HIV. This syndrome is diagnosed when the person has one or more opportunistic infections, which if not treated effectively, will be fatal. Pneumocystis carinii (PCP) is a serious form of pneumonia and a common AIDS opportunistic infection which, before effective HIV treatment in 1997, was the cause of many deaths.

Here is another project that interviewed people with HIV about their experiences, conducted by DIPEx.

How did people become infected?

The modern treatment for haemophilia is concentrated clotting factors 8 or 9. Thousands of blood donations are required for the manufacture of each batch of concentrated factor treatment and treatment was contaminated if any of these donations contained HIV or hepatitis viruses.

In the UK in the early 1980s 1,246 people were infected with HIV through treatment with blood products. Heat treatment was introduced in 1985 which inactivated viral infections. Today recombinant clotting factor is frequently used. This is synthetic and does not require donated blood.

What is Birchgrove?

Birchgrove is a self-help group for the people with haemophilia and HIV. Birchgrove started in 1986, taking its name from the pub in Cardiff where the group first met. It is now a national organisation.

The aim of Birchgrove was to actively support and inform those with haemophilia, HIV and Hepatitis C by organising meetings events and producing a magazine. Today Birchgrove does not organise separate events but is involved in those organised by the Macfarlane Trust and The Haemophilia Society. Back issues of the Birchgrove magazine and a history of the group can be found on their website.

Was compensation given?

Many people with haemophilia and HIV attempted to sue the government for negligence. Negligence was not accepted and compensation was not paid. In 1987 a hardship fund was established by the government and administrated by The Macfarlane Trust. The Macfarlane Trust continues to support people infected with HIV through NHS blood products today.

In 1989 all those infected received an ex-gratia payment of £20,000.

In 1990 a variety of financial settlements were awarded as recompense in an out of court settlement:

  • £60,500 for a person with partner and dependent children.
  • £32,000 for a person with partner but no children
  • £23,500 for a single person
  • £21,500 for parents of a dependent child.

A payment was also made to the partners of those with haemophilia and HIV who had been infected.

In February 2007 the Archer Inquiry was announced. It is an independent inquiry funded by private donations and not a parliamentary or government inquiry. The Scottish Health Secretary Nicola Sturgeon also announced that there would be a public inquiry in Scotland into the contamination of blood products when the Archer Inquiry was completed.

'TaintedBlood' is a group campaigning for a public inquiry and compensation for people infected with HIV and/or hepatitis C through blood products.

What is Hepatitis C?

Hepatitis C was also contained in the blood products that many people received in the early 1980s. Hepatitis C is a highly variable disease in how aggressively it develops, what symptoms result and how it responds to treatment. It has the potential to cause serious liver damage, cirrhosis and cancer. Treatment for hepatitis C involves treatment with tablets and injections which can last up to 72 weeks and is frequently associated with distressing side-effects. Response to treatment is variable but when effective can be a complete cure. When the individual also has HIV the hepatitis C is often more aggressive, causing serious liver damage and responding less well to treatment.

What is the treatment for HIV?

Antiretroviral therapy (ART) or combination therapy is treatment, usually tablets, which can prevent HIV replicating in the body. If the virus cannot replicate, it cannot destroy the immune system. The treatment must be taken every day for life. If drugs are not taken as prescribed there is a chance the virus can adapt and become resistant to the drugs making the therapy less effective. Although this treatment is very effective some side-effects can be very serious and permanent.

What does CD4 count and viral load indicate?

The CD4 (or T4) count indicates the vulnerability of the immune system. A normal CD4 count varies between 800-1200. A cell count of less than 200 indicates an immune system very vulnerable to opportunistic infections and cancers contained within the body.

Viral load measures the amount of HIV in the body. A high viral load indicates HIV is replicating rapidly and will damage the immune system. When HIV treatment is effective viral load should be 'undetectable'.

The aim of drug therapy is to prevent viral replication and so have a low viral load and to improve the immune system and so have a high CD4 cell count.