Holly Mills was a lively teenager about to start university. But with her whole life ahead of her, she suddenly found herself in the grip of tragedy.
Within the space of just a few months, the gregarious 18-year-old had become so severely brain damaged that she was unable to move or communicate.
Eight years on, Holly has to be fed through a tube into her stomach and shows no emotion or awareness of her tragic predicament. Her days are spent in heartbreaking, silent immobility.
Holly is one of only three people still alive after developing the full-blown symptoms of Creutzfeldt–Jakob disease (also known as new variant CJD, or vCJD) — the human form of mad cow disease.
Holly’s parents Peter and Linda, both 61, are devoting themselves to their daughter’s full-time care and to a programme of daily mental stimulation, in the belief they are keeping her brain alive until a treatment emerges that will help her.
While they and their three older children live in hope of a cure for Holly, there is growing concern that another CJD outbreak may be imminent.
New evidence collected by the Health Protection Agency (HPA) suggests that one in 4,000 people who were eating meat before 1996 is probably carrying CJD (after that date, cattle infected with mad cow disease were, theoretically, removed from the food chain).
That could mean that as many as 15,000 people nationwide could be affected. While this is in line with a previous survey, the latest findings suggest CJD might be more prevalent in older people.
The findings — which received little publicity when published a few weeks ago — are mid-way results of a programme testing 30,000 samples of tonsil and appendix tissue removed during routine operations across the country.
The aim was to look for evidence of ‘silent’ or symptom-free infection, though scientists admit the method used in the tests cannot identify CJD with total accuracy.
Until recently, a definite diagnosis of the disease in people who have the symptoms could be made only after death, because brain tissue analysis was required.
However, John Collinge, a professor of neurology and a leading expert at the Government’s CJD research unit at University College, London, has developed a blood test which can check if the disease is present by detecting evidence of the so-called prions or infectious proteins known to cause the disease.
He believes the number of people infected could be as high as one in 1,000 and says the CJD situation is ‘very worrying indeed’.
So far, there’s little official interest in investing the several million pounds needed to turn the blood test into the high-speed screening tool needed to bring it into routine use. Critics say this is because the Government fears what it might find.
Mad cow disease, or bovine spongiform encephalopathy (BSE), first emerged in Britain in 1986 as a result of ‘cannibalism’, when beef offal was fed to cattle, which are natural grass-eaters.
The proteins, called prions, that cause BSE were found in large quantities in the brains, spinal cords and spleens of cattle (although they were also subsequently discovered in meat tissue, too). When animal carcasses were ground down to form feed stuff for other cattle, prions were passed on.
They then colonised the brains of the cattle which ate them, and were passed to humans via cheap, mechanically-recovered meat — such as processed sinews and offal that were used at the time in school dinners and baby food.
In humans, the prions triggered the development of a new form of fatal human dementia called new variant CJD, which was first identified in 1996. These prions are entirely new infectious agents, completely different from viruses, bacteria or parasites.
Basically, they are faulty versions of healthy proteins in brain and nervous tissue that then induce their neighbours to become faulty. As a result, the brain cannot function: all signals are disrupted or shut down completely, leading to almost certain death.
