Holly Mills’ grieving family continue CJD fight

Holly Mills, 26, passed away peacefully at her home in the North Yorkshire village of Thornton-le-Dale after being diagnosed with the neurological condition in 2003.

Her father, Peter, said the family is “absolutely devastated” but maintained that both he and his wife, Linda, are determined to realise a lasting legacy for their daughter.

The couple conducted a prolonged campaign to get Government approval for trials on a drug called pentosan polysulphate (PPS), which they believe was instrumental in keeping their daughter alive for so long.

Mr Mills said: “Holly survived as she did as a result of that treatment and we absolutely want to ensure research continues. Our campaign will carry on in the hope that other people will benefit and as a legacy to Holly.”

A month before Miss Mills was due to go to university, consultant neurologists told the bright, articulate 18-year-old in August 2003 that she had contracted variant CJD and had as little as a few weeks to live.

But eight years on from the devastating diagnosis, Miss Mills was able to live at home with her parents in their 17th century cottage up until she passed away on Saturday night.

Mr and Mrs Mills, who also have two sons and another daughter, maintained that her ability to maintain at least a semblance of a normal life was down to PPS. Her funeral is expected to be held in All Saints Church in Thornton-le-Dale on Wednesday next week.

Mr Mills said: “There had been no change in her and we had been out on the Saturday afternoon to Scarborough and it was a lovely autumn day. Holly had been later to bed than usual and in the early hours she passed away in her sleep, which is one comfort.”

One of Miss Mills’ brothers, Rob, 27, was in Buenos Aires in Argentina when he learnt that she had died and flew back to the UK on Monday. He was half-way through a six-month sabbatical from his job as a surveyor in London to travel around South America, New Zealand and Australia with his girlfriend, Lucy Wilde.

He said: “Holly was always my best mate when we were growing up. But when she got ill and her personality changed and she stopped talking, that’s when I felt that we had lost her.

“We had nine years to try to come to terms with what had happened to her, and now we have got to start grieving all over again. It is all really quite surreal at the moment.”

There are several forms of Creutzfeldt-Jakob disease (CJD), mainly affecting older people and leading to dementia. But in 1995 a new form, variant CJD or vCJD, was recognised, which occurred in much younger people.

It is thought that variant CJD results from exposure to BSE through affected beef or beef products. Those diagnosed with variant CJD are not expected to survive more than about 12 months.

Mr and Mrs Mills spoke about their daughter’s condition in 2005, lifting a High Court ban on her identification so they could demand research into the PPS drug and its effect on the condition.

The couple rejected the offer of 24-hour support for Miss Mills because they feared it would be too prescriptive and fought for her to be given PPS, details of which they found on the internet.

The Medical Research Council announced in 2005 that a monitoring study would analyse the effects of the drug on a small number of patients in the UK.

The results were published the following year and found that while PPS does not stop the progression of vCJD and other prion diseases, more research was needed.

The Medical Research Council announced in February this year that the world’s first accurate blood test for variant CJD had been developed.

The prototype, which is 100,000 times more sensitive than any previous attempt, could transform the diagnosis and screening of the disease.

The research team tested 190 blood samples, including 21 from people known to have vCJD. The blood test was able to detect blood spiked with a dilution of vCJD to within one part per 10 billion – 100,000 times more sensitive than any other method so far.

Prions, the infectious proteins which cause vCJD and other fatal prion diseases, can inhabit a person’s body for up to 50 years before presenting symptoms.

During this time, there is a chance a carrier of vCJD could pass on the infection, such as through a blood transfusion or even through surgical and medical instruments as prions can easily attach onto metal surfaces.

A more accurate blood test would enable earlier diagnosis and could also help identify carriers of the disease.