Creutzfeld-Jacob Disease used to take decades to kill. Sufferers tended to be over 55. But CJD is changing. Now it kills the young. Can it be a coincidence that, in the last 10 years, we've eaten 1.5 million BSE-infected animals?
On Friday morning at 11 o'clock, 20-year-old Peter Hall was buried at Chester-le-Street Parish Church cemetery, near Sunderland, Co Durham. He was killed by Creutzfeldt Jakob Disease (CJD), the human equivalent of Mad Cow Disease (BSE). First striking him at 19, just 13 months ago, it made Peter Hall the fourth British teenager to succumb to the disease to date. Two others died last year-Stephen Churchill, at 19, and an un-named 17-year-old girl. The very first teenage CJD victim, Victoria Rimmer, 16 when she was diagnosed by live biopsy late in 1994, is still in a terminal coma.
Government scientists argue that the recent increase in surveillance is the most likely reason why more cases, including teenage cases, are now being identified. But Britain's leading CJD specialist, Dr John Collinge, at St. Mary's Hospital, Paddington, disagrees. 'Given the rarity of CJD in teenagers,' he says, 'It is unlikely that such cases would have been previously missed.' In other words, we're faced with something new.
The Government still insists that there is no evidence to link BSE and CJD. Most recently, Health Secretary Stephen Dorrell said that 'there was no conceivable risk' in eating beef. But the facts tell a different story.
The evidence of a link between BSE and CJD-as exclusively reported in Night & Day last December-is mounting. For one thing, since the BSE outbreak, the whole pattern of CJD in Britain has been changing. As well as striking younger people, it also attacks a different part of the brain-the part which BSE attacks in cattle. The initial symptoms are different, too. In old-style CJD, the kind which usually struck those over the age of 55, the first sign of serious trouble was forgetfulness-very much like Alzheimer's. But today, as in these teenagers, an equally common major symptom is so-called 'ataxia'-exactly the kind of staggering and loss of balance seen in BSE-riddled cattle.
Now, too, we are seeing a sprinkling of CJD among people in their twenties and thirties: Jean Wake, 38, Michelle Bowen, 29, and Maurice Callaghan, 30, all died last year. And what of the four farmers who've died of CJD? All had worked on BSE herds, which hardly seems random. Indeed, given the worldwide incidence of CJD of one case per million population, and 20,000 British farmers at risk, only one farmer would be expected to succumb every 50 years-not four in the past three years.
None of this, it must be said, amounts to concrete proof of a link between BSE and CJD. But the Government's insistence that there is no evidence of a link is plainly untrue. That there are four known teenage CJD cases in Britain to date is bad enough. But given that any parents would be reluctant to go to the media once their child had been pronounced terminally ill, as were Derek and Frances Hall, there is a strong likelihood that there could be more British teenagers out there dying of CJD. The Halls decided to 'go public' only after the publication of Night & Day's article last year.
Calculated to protect Britain's beef trade, and first deployed in 1989, the Government's initial gambit was to insist that BSE posed only a 'remote risk' to man, when there was no evidence whatsoever to back the claim. Stuck with that half-lie thereafter, the Ministry of Agriculture actually refused to develop a postmortem abbatoir test which would determine, once and for all, the full extent of the BSE epidemic. Dr Harash Narang, the government scientist who'd formulated the test, pushed hard to get it used and thereafter was officially side-lined, demoted, smeared, and finally made redundant at the end of 1994. Working entirely off his own bat, however, Dr Narang is now perfecting a BSE urine test for cattle. As well as eradicating the disease, it would restore our beef trade at a stroke. Official policy, instead of protecting the beef trade, appears to be having the opposite effect. Just this week, fed up with the Government's ducking and diving, five German states banned all British beef imports.
Again, all on his own, Dr Narang has already perfected a urine test for CJD. It was immediately after learning of this test in Night & Day that the Halls invited Dr Narang to test their son. Twice they'd asked Peter's neurologist if he could be suffering from CJD, twice he insisted it was highly improbable in one so young. In January, Dr Narang's test confirmed the contrary.
That the Government still refuses to recognise and utilise Dr Narang's test isn't merely a political outrage. Without fast and accurate diagnosis of CJD, of which Dr Narang alone appears currently capable, there remains no possibility of any ameliorating treatment for this hideous disease, let alone a cure. In the last five months of his life, Peter Hall was offered no treatment whatsoever. And, except for a weekly visit from his neurologist, nor was his illness closely monitored. Eloquent with anger, Derek Hall says: 'If Peter's condition was unique and devastating, as we'd been told it was, why is it that no one bothered to study what he was suffering from?'
Coming up to Christmas of 1994, Peter Hall was right as ninepence. Never ill, and with 100 per cent school attendance certificates at the back of his wardrobe to prove it, he was doing the first year of an environmental studies degree at Sunderland University and gigging in his spare time in a heavy metal rock group. (He called the group Tantalum which, as students of metallurgy will know, is a very heavy metal indeed.) Peter had long, Seventies hair but was very Nineties about it, washing it every day, and was meticulous about his appearance.
'That was the first thing we noticed,' recalls Derek Hall. 'An unusual self-neglect setting in around January 1995. Peter was looking scruffy, not eating, losing weight, becoming withdrawn and dark, and we thought, as did our GP, that it was classic student depression. But three kinds of anti- depressant tablets proved of no use whatsoever. Finally, the psychologist ruled out depression and Peter underwent a whole battery of tests-a CT scan, ECG, bone marrow, lumbar puncture, chest X-rays, an endoscopy, and pints and pints in blood tests. All showed nothing.' By May 1995, Peter's short-term memory was shot and, by early July, his loss of balance-'Like he was drunk all the time,' said his father-confined him to a wheelchair. 'By early July,' Frances Hall explained, 'he was completely apathetic-not talking except in a whisper, and even when the television was on, just sitting in his wheelchair staring at the wall.'
In August-now incapable of feeding himself, incontinent, sometimes hallucinatory-Peter was admitted to the respite ward of the local Earl's House hospital, where his mother nursed him daily. Derek Hall, who's a bricklayer, drew up plans to build an extension so they could bring Peter home. Desperate, and with no other treatment forthcoming, the Halls arranged for him to have acupuncture and herbal infusions. Amazingly, through October and November he 'plateau-ed out', according to his neurologist. Then he relapsed. In January, after Dr Narang's test confirmed that Peter had CJD, Derek Hall in formed the neurologist. 'He said, "We must keep an open mind." But when we pressed him further, all he'd say was, "Hand on heart, I can't honestly say it isn't CJD." '
Before Stephen Churchill died last May, his neurologist had had exactly the same reaction: he couldn't believe that CJD could strike someone so young. But why the disbelief? Can there be a doctor left in the country who is unaware of the increased incidence of CJD among younger people? At one point during his illness, Peter Hall was shown to a panel of 50 medical people and his symptoms described. Pronouncing his condition merely 'baffling', no one suggested CJD. The results of the postmortem will not be known for a few days yet, but the death certificate, issued last Tuesday, reads: 'A) Disease or condition directly leading to death: bronchial pneumonia.' And underneath, 'B) Other disease or condition, if any, leading to A): Chronic neurological illness.' And then, next to the word 'cause', the issuing doctor wrote '?'.
Could it be that the medical profession is running scared of CJD? Given the Government's 'no evidence' hard line, the fact that a good number of hospital doctors are on short-term contracts these days, and the collapse of Dr Narang's career after he stuck his neck out, it might not be too surprising. The CJD orthodoxy, however, is already swinging Dr Narang's way. His unique discovery of the DNA-based nemavirus-as the one true tell-tale sign of spongiform brain disease - has been confirmed by Nobel prizewinner Dr. Carleton Gajdusek. And take the recently-changed position of Dr. Stanley Prusiner, regarded by british government scientists as the oracle on CJD. Dr. American Orthodoxy himself, Prusiner had argued for 15 years that the spongiform disease agent is a non-DNA type-until last November, that is, when he conceded in a scientific paper that a DNA-based agent is implicated after all. So, how much longer do the Ministries of Health and Agriculture think they can afford to ignore what Dr Narang has to offer? Last month, a pharmaceutical company estimated that his cattle urine test would be worth 15 million on the open market. To date, in destroyed animals and lost beef sales, the BSE epidemic has already cost an estimated 2 billion. Aside from the swing in American scientific orthodoxy, in Britain there is also a groundswell-of farmers anxious to assist Narang to perfect his cattle test and many more desperate to use it, and of the surviving relatives of CJD victims who, acutely aware of the absence of treatment, of confident medical support and official candour, are offering whatever help and advice they can to other families now entering CJD's dark tunnel. A chief priority of the newly formed Northern CJD Support Group, of which the Halls and Jean Wake's family are members, is to dispel fear. 'No one explained to us,' said Derek Hall, 'that CJD is transmissible -you get it by eating something infected- but that it's not actually infectious. When tending to someone, precautions are sensible, but it's important to know that you can't actually catch CJD like a cold.' [Thus: CDJ is transmissible; infection requires intimate contact with affected material, usually through eating. The disease is not communicable. The "cold" is not a disease!- J.C]
At present, there seems to be no standard procedure for the burial of CJD victims. On advice from Newcastle General Hospital, no viewing of Peter's body was allowed and the coffin was kept closed. In January, however, when Maurice Callaghan was buried in Belfast, special precautions included a closed coffin, a specially limed grave dug to a depth of nine feet, not the usual six, and the gravediggers issued with protective clothing and surgical gloves. The Callaghan family have no idea who ordered the precautions.
Since then, the Callaghans have achieved something unprecedented-the grant of an inquest on the grounds, as his brother Gerry explains, 'that Maurice's death from CJD, given he was only 30, may not have been by natural causes. We discovered, too, that in law, an inquest must be granted if there's- and I quote-"a continuance of circumstances which comprise a threat to public safety". Which, to us, certainly seemed to apply to the possible dangers which BSE poses to humans.' The inquest is scheduled for September.
The Northern CJD Support Group is also determined to bring CJD out into the open, not least by lobbying to have it classified a notifiable disease, which would ensure that every suspected case of CJD was centrally reported and collated. They also want the Government to publish not just the annual numbers of CJD deaths but also a breakdown of all victims by age, the major symptoms, and the specifc area of brain attacked. 'Then we'll have a clearer idea of whats happening to us' said Derek Hall.
That same year, Narang, a clinical virologist for the Public Health Service Laboratories (PHSL), devised a random slaughterhouse test for diagnosing sub-clinical BSE in cattle that hadn't yet shown symptoms of the disease but which were going-as they still do-straight into the national diet. As a first step to eradicating BSE, the test would have proved how widespread the disease really was. John MacGregor, then Minister of Agriculture, refused to use it. But Narang wouldn't be put off. He kept pushing the test, and working to formulate still better ones. This made him a political liability: beef was already safe, the Government insisted. Since then, Narang's once brilliant career, based on much original work on spongiform disease over 25 years, has been all but destroyed.
This disgraceful saga may yet have the best possible ending. For it appears that Narang now has the very means to rid Britain of BSE, which has afflicted 52 per cent of dairy herds so far. [Remember: milk from contaminated and uncontaminated cattle is mixed- J.C] Having already formulated a simple urine test for diagnosing CJD, he is just now perfecting a similar test for BSE. "Just test their urine, you see," he says, "and you could clean every herd of those animals harbouring the disease." His crucial test might well have been in use already had he not, a year ago, been made redundant by the PHSL, and denied essential laboratory facilities ever since.
But first, the evidence. When Ministers and officials insist, as they do daily at the moment, that there is 'no evidence' linking BSE with CJD, they're wrong on two counts. What they mean is that there's no concrete proof-and that's true. As every one knows by now, since BSE was discovered in 1985, CJD has been on the increase -27 cases in 1985 rising to 55 cases in the last recorded year, 1994. There are three basic types of CJD and before the BSE outbreak the vast majority of cases were of Types One and Two. But since 1985, there has been an increase in the third, very rare type of CJD-the so-called ataxic or Kuru type. In its major characteristics, it closely resembles BSE. If BSE had nothing to do with CJD, all the newer cases would have been far more likely to be of Types One and Two. Another indicator of a link is that, since the BSE outbreak, the pattern of CJD has been changing in four key ways: the age it strikes people, the major symptoms, its tell-tale pathology-that is, the area of the brain it attacks-and its shortened incubation time.
* Age spread: before BSE, the vast majority of CJD cases were confined to people aged 55 and over. Back then, there was not one recorded British case of CJD among teenagers, and only three cases in the rest of the world. Now, there are three British teenage cases as against just four others world-wide.
The Government's 'no evidence' orthodoxy argues that the recent increase in surveillance is the most likely reason why more cases, including teenage cases, are now being identified. But Dr John Collinge, who is the leading orthodox voice-he heads up the Prion (spongiform disease) Group at St Mary's Hospital, Paddington-holds the contrary view: "Given the rarity of CJD in teenagers," he says, "it's unlikely that such cases would have been previously missed." Today, too, there's more of a broad sprinkling of CJD cases down through the under-55s. Just last month, in a Manchester hospital, a 29-year-old woman in the terminal stage of CJD had her baby born by Caesarean section three weeks before she died. The child appears well, but only Narang has the practical means to test whether or not vertical transmission of CJD, mother to child, has occurred.
* Symptoms: the second big change in CJD is in its clinical symptoms. Before the BSE outbreak, typical symptoms resembled those of Alzheimer's disease-most notably forgetfulness and impaired, out-of character behaviour. Today, according to specialists who have worked on post-1985 CJD cases, an increasingly common symptom is ataxia-the kind of shuddering, loss of balance and drunken gait you see in BSE stricken cattle.
Ataxic shuddering and unsteadiness are also the major symptoms of Kuru, a type of CJD once common in a New Guinea tribe, the Fore, who ritually ate their dead. (In dialect, kuru means 'shaking disease'. Since the Fore stopped their cannibalistic practices in the late Fifties the disease has died out.) Another characteristic of Kuru, in common with post-1985 ataxic CJD, is that it would strike all age-groups. In children, Kuru's incubation period could be as short as four years.
The orthodox wisdom on this factor is that the children probably got infected via cuts or scratches-intravenously, that is, rather than by eating diseased flesh. But Narang long ago spotted another intravenous route: "Children's open gums when they lose their milk teeth". By way of testing his theory: "I fed infective material to mice, grinding away at the teeth of some of them first. They all became ill far more quickly." [Mice traumatised in this way would likely succumb to any infection more rapidly, this is unlike natural childhood open gums.- J.C]
* Pathology: thirdly, marked changes have also been observed in CJD pathology. Before the outbreak, old-style Alzheimer's-like CJD would attack the brain chiefly in the cerebral cortex and sometimes the cerebellum. With the rare strain of CJD, just as with BSE, the disease attacks the central grey matter and the cerebellum.
To date, Night & Day has learned of six such atypical cases in adults. Since our discovery was random-almost accidental- there are bound to be more. Stephen Churchill, 19 when he died last May, might well be another such case. As described in The Lancet of October 28, his symptoms were actually a mixture of Alzheimer's-like and ataxic ones. But as post-mortem analysis revealed, the disease was of the ataxic/Kuru type which had chiefly attacked the central grey matter and cerebellum.
* Incubation time: given the recent increase in CJD cases, incubation time also fits a new pattern. It's been 10 years since the BSE outbreak, and Kuru's mean incubation time is between 10 and 15 years in adults, less in children. BSE's incubation time is also characteristically short: laboratory animals inoculated with BSE take roughly half the time to die as those inoculated with scrapie (spongiform disease in sheep).
All evidence, yes, but by no means conclusive evidence that, for example, BSE caused Stephen Churchill's death. Before 1990, spongiform disease was unknown in cats but, last year, Dr Moira Bruce, of the Neuropathogenesis Unit in Edinburgh, solved that mystery. To do so, however, she first had to inoculate series of mice with infective cat brain material, and then wait to discover what strain of disease they would die of. It was BSE. The series took three years to complete.
A similar process will have to be carried out to determine if BSE killed Stephen Churchill. Currently, brain material from two farmers who died of CJD is being tested by Dr Bruce in this manner. But the same work in relation to Stephen Churchill has yet to begin. In great part because the families concerned want straight answers, Narang has been given brain samples from three recent CJD victims, one of them Stephen Churchill.
So here we have it-not only a strain of CJD which closely resembles BSE. What's turned up in humans could well be BSE. For it's now accepted that BSE existed at a low level in cattle well before the 1985 outbreak. It therefore follows that those rare cases- past and present-could all be BSE. But only a three-year test series using mice could tell us for sure.
ON AVERAGE, WE'VE EATEN 80 MEALS OF CONTAMINATED BEEF EACH