Up to 4 per cent of the blood in the UK's blood banks could be contaminated by the lethal brain condition vCJD (variant Creutzfeldt-Jakob disease), the human version of mad cow disease. UK agencies apparently know the identities of some of the 'silent carriers' of the disease, but are still accepting blood from them, which is being given to healthy patients.
One carrier was allowed to regularly give blood for 20 years and it's now known that at least three people who were given his blood have died. According to the Creutzfeldt-Jakob Disease Surveillance Unit in the UK, a government agency based at Edinburgh University, four cases of vCJD have arisen from contaminated blood.
The true extent of the problem isn't known, but no country will accept blood from anyone who has lived in the UK since 1980. In fact, Britons who tried to give blood following the 9/11 terrorist attack on New York were turned away from hospitals.
Specialists maintain that a process called 'leucodepletion' or 'leucoreduction'-removal of the immune system's white blood cells from the blood-'cleans' the blood of infections such as vCJD. But Christine Lord, whose 24-year-old son died from the disease in 2009, claims it is effective in only 30 per cent of cases.
She is campaigning for the introduction of a simple blood test that can quickly determine whether someone is carrying vCJD. Her campaign has the support of former health minister Frank Dobson, who served in the Blair government until 1999 and who has sanctioned the spending of lb100 million a year to clean up all stocks of blood.
The new test was developed by Professor John Collinge and others at the Medical Research Council's Prion Unit, and Collinge has been using the test on patients in his own clinic. In a small trial of 190 blood samples, the test detected 15 of the 21 samples that were infected with vCJD and there were no false positives-the test didn't pick up signs of infection that weren't actually there.
What is vCJD?
BSE, or mad cow disease, is caused by an infectious agent called a 'prion', an abnormal protein that accumulates and eventually leads to nervous tissue damage in the brain and spinal cord. The human form of the condition, vCJD, can lie dormant for up to 20 years, and early symptoms relate to the nervous system, including depression and loss of coordination.
Dementia occurs later in the course of the disease, but it's only in its last stages that brain abnormalities can be detected on MRI (magnetic resonance imaging) scans. The disease is invariably fatal, and death usually occurs around 13 months after the first symptoms appear.
How much, how many
Collinge and his researchers want the test to be properly evaluated in a large-scale trial "so we will know how many people are likely to be infected with vCJD", says Dobson. And this, believes Christine Lord, is the very reason the government doesn't want to roll out the test.
Since her son Andrew died, Christine has been investigating the mad cow disease scandal, which occurred within a 10-year window when contaminated meat was allowed into the human food chain. Although the number of deaths from vCJD stands at 176 and peaked in 2000-when 28 deaths were reported-many thousands more are 'silent carriers' of the prion infectious agents, a type of protein that causes vCJD.
The prions can incubate for 15 to 20 years-some experts reckon they can lie dormant for up to 50 years-without the carrier showing any symptoms. Estimates of the numbers of silent carriers have varied wildly from 3.4 million to just 15,000, but Collinge and the Health Protection Agency said in 2012 that "60,000 UK citizens may be silently carrying vCJD". If true, that equates to around one in 1,000 Britons being infected.
As 4 per cent of the general population regularly gives blood, it can be assumed that a similar proportion of people infected with vCJD, as a typical cross-section of the population, also give blood. If so, between 2 per cent and 4 per cent of the blood in UK blood banks could be infected, depending on the effectiveness of leucoreduction.
Christine, a freelance journalist, has become convinced there is a massive cover-up at the heart of the mad cow or BSE (bovine spongiform encephalopathy) scandal, with government officials allowing contaminated meat into the human food chain. She says she has received threatening phone calls, including the ultimatum to take down her campaigning website, while government agents who have tried to help her have suddenly been gagged by the Official Secrets Act.
"I'm not worried. What more can anyone do to me? I've lost my son, and he asked me just before he died to find out why this had been allowed to happen to him," she says.
In her investigations since Andy died, she has discovered that:
-Since 2007, every blood transfusion bag in the UK has carried a disclaimer that the blood may be contaminated with BSE.
-The first animal-to-human infection was recorded in 1963, although it may well have been happening for many years before that.
-BSE is not restricted to meat products, as contaminated meat has also been used in the manufacture of baby food, pharmaceuticals and vaccines, and school meals.
The birth of BSE
As Christine discovered, humans have been infected with vCJD from eating contaminated meat for many years. The first recorded incident was in 1963 after a tribesman from Papua New Guinea died from a disease called 'kuru', a form of CJD, which he contracted after eating contaminated monkey brains.
In 1974 a patient was infected with CJD from a corneal graft, and two epilepsy patients were infected two years later through infected electrodes. However, the first cases in hospital may have occurred much earlier: two cases were recorded in the 1950s following the use of contaminated neurosurgical instruments.
Mad cow disease was centred in the UK, with the first cases observed in cattle in 1984. Although cows are essentially herbivores, they were being fed the brains and spines of lambs infected with scrapie, their version of mad cow disease. Once the disease was discovered, around 183,000 infected cows on UK farms were slaughtered, but for years government ministers denied that any infected meat had entered the food chain. The then agriculture minister John Gummer was famously photographed in 1990 eating a hamburger with his four-year-old daughter just six months after the government had banned beef offal for human consumption.
Ten years later, the Phillips enquiry into the BSE outbreak revealed a cover-up among civil servants, ministers and government agents, all of whom had repeatedly underplayed the real threat from BSE and persistently failed to tell the truth to consumers, Lord Phillips concluded. Complacency plus a desire to both avoid a national panic and protect the meat industry combined to produce a response from government that was too little, too late.
The young die first
Most of the 176 fatalities from vCJD have been among the under-25s. Experts reckon that everyone over the age of 15 in the UK has eaten at least 50 BSE-infected meals, probably from school meals or low-quality meat used in burgers, sausages and hotdogs.
Christine suspects that Andrew was infected from eating school meals, although she can't be certain as contaminated meat was also used in infant meals and in the manufacture of childhood vaccinations and in vegetable soups. Even strict vegetarians and Hindus, whose religion forbids them from eating beef, are among those who have died from vCJD.
The prions that cause vCJD have also been found on dental and surgical instruments; they cannot be killed by standard hospital sterilization procedures. Three mothers in the terminal stages of vCJD have given birth by caesarean section and the birth units could still be contaminated, Christine says.
Several women in Australia died from vCJD after contracting it from hormone injections, and hundreds of haemophiliacs have been told they are at special risk because some of their blood-clotting medicines were sourced from blood donors who later developed symptoms of vCJD.
Andrew started to show signs of vCJD infection in 2007 and he died two years later, by which time he was quadriplegic. Although his death in 2009 was recorded among the three that were officially recognized as the result of vCJD infection, Christine is convinced many more deaths have occurred that have not been properly attributed. She still meets parents who are convinced their child died from vCJD, but whose death was never classified as such; over the past two years, zero deaths have been attributed to vCJD infection.
Figures from other countries also suggest that vCJD isn't going away. In 2011, two children died from vCJD in Paris, and France has now become the second most-affected nation after the UK, with 27 confirmed cases so far.
Christine doesn't want her son to have died in vain: in addition to the introduction of the blood test to identify silent carriers, she wants those involved in the original cover-up to be brought to justice.
But if the true picture is anywhere near as grim as she paints it, plenty of people will ensure that neither of her demands is met.
To read more about Christine's campaign and her forthcoming book Who Killed My Son?, about her son's struggle with the disease and her fight for justice, go to www.justice4andy.com.