Haemophilia A is a hereditary disorder, caused by a genetic mutation that leads to deficient or defective factor VIII, a protein in the blood that is important for clotting. It first came into public prominence as the “royal disease” when a number of descendants of Queen Victoria, starting with her eighth child-Prince Leopold-were diagnosed as haemophiliacs.
There is no lasting cure for haemophilia. Haemophilia is usually treated by regular and lifelong preventative replacement with factor VIII concentrate, administered as an intravenous infusion. Some patients may only receive treatment in response to actual episodes of bleeding, which is known as demand therapy.
Factor VIII concentrate was introduced in 1966. Initially all factor VIII in medical use was derived from human blood plasma. Human plasma is still used as a source of factor VIII. Plasma from screened donors is pooled together and then subjected to viral inactivation procedures. However, in the past, blood was not screened for viral infections and viral inactivation, such as heat treatment, was not available. Batches of freeze-dried factor VIII concentrates were obtained from large pools of tens of thousands of blood donors, many of whom were at “high risk” for the transmission of various viral infections.
In 1973, the British government started purchasing blood products from the US because of a shortage of supplies. In contrast with the system of voluntary blood donations in the UK, American blood products were frequently sourced from paid donors in the prison systems of states such as Arkansas and Louisiana. Four major pharmaceutical companies, Rhone-Poulenc Rorer, Baxter International, Alpha Therapeutic and Bayer, were the major players in the blood trade at the time. It has since been reported that drug addicts, sex workers and alcoholics were frequently used as blood donors.
In July 1982, the Centers for Disease Control in Atlanta, Georgia, reported three cases of AIDS in haemophiliacs. The routine testing of all donated blood for HIV began in 1985.
However, while AIDS was being recognised as a new illness and facts about HIV transmission were emerging, a major tragedy was unfolding in the UK. In the late 1970s and early 1980s, around 4, 689 British haemophiliacs were infected with hepatitis C and 1,243 were infected with HIV. Some of those affected acquired both hepatitis C and HIV. Of the HIV-positive individuals, less than 250 are alive, as of 2019.
Haemophilia treatment has become safer with a reduced reliance on blood products, along with safer manufacturing procedures for human plasma-derived factor VIII . Recombinant factor VIII was first obtained through genetic engineering procedures using DNA technology in the 1990s and has largely replaced the use of human plasma.
But what about the victims of the transfusion of infected blood? There has been much delay in acknowledging the problem and in adequately compensating the victims. Lord Archer chaired an independent privately-funded inquiry into Contaminated Blood and Blood Products during 2007-2009 which apportioned blame to the US suppliers. In 2008, the Scottish government set up a public inquiry which was chaired by a judge, Lord Penrose. The report in 2015 claimed that the incidents of contamination between 1970 and 1991 were mostly unavoidable. The only recommendation was to offer hepatitis C testing to all Scottish recipients of blood transfusion before 1991.
On July 11 2017, Prime Minister Theresa May announced a public inquiry in England. The Infected Blood Inquiry, chaired by Sir Brian Langstaff, a former High Court judge, opened on April 30 2019 following preliminary hearings in September 2018. The campaign group Tainted Blood is coordinating efforts on behalf of victims and relatives, for whom haemophilia awareness ribbons in black (for death), red (for HIV) and yellow (for hepatitis C) have become a visible emblem of their fight for justice. The inquiry is ongoing, and its conclusions are awaited in due course. Also in April 2019, Theresa May pledged £29 million in support payments for the victims of the scandal.
The scandal of contaminated blood product usage in the UK has all the hallmarks of a poorly handled public health problem. A protracted period of deception and denial has finally, albeit belatedly, led to the beginnings of a thorough investigation, although all of this has come far too late for many of the victims.
Ashis Banerjee (ex-NHS)