NI teenager's family welcomes CJD ruling

The case was heart in the High Court

The family of a Belfast teenager, who suffers from the brain disease variant CJD, has welcomed a court ruling granting him the right to have a radical new drug treatment.

The controversial experimental procedure has not yet been tested on humans but some scientists are hopeful that the treatment could stall the progress of the disease.

In the High Court in London on Tuesday, 18-year-old Jonathan Simms, and a 15-year-old girl in England who can't be named for legal reasons, won the right to have the treatment.

The landmark High Court ruling means that Jonathan will become one of the first patients in the province to receive the drug pentosan polysulphate, which will be injected directly into his brain.

If it were the case Jonathan died out of this his death will not have been in vain

Don Simms
Father

His father Don has been campaigning for the move for the past seven months.

"The animal studies that have been carried out with this particular compound has given those animals up to 40% extension of life and who knows, with medical science as rapid as it is, what may be around the corner," he said.

"If it were the case Jonathan died out of this, his death will not have been in vain."

The case sets a legal precedent that could radically alter the way that unproven treatments are used in people with a terminal illness.

Jonathan Simms may receive the treatment

However, a hospital trust which had been lined up to carry out the treatment is still refusing to do so, even though doctors there are happy to go ahead.

The previous advice from vCJD experts in the UK was not to use pentosan - but that is now to be reviewed.

vCJD is thought to be caused by "rogue" proteins called prions, which corrupt other proteins in the brain and form clumps which slowly kill brain cells.

At present there is no available treatment that can reverse or even slow down this decline.

Pentosan, it is hoped, may be able to latch onto prions, rendering them less harmful, and perhaps allowing them to be flushed out of brain cells.

The drug molecule has to be injected directly into the brain because it is too big to pass from the bloodstream across a protective filter called the "blood brain barrier".

Click here to read BBC News Online's full interview with Don Simms

What doctors are unsure about is how much pentosan would have to be given to have an impact on vCJD in the human brain, and how much it is safe to give.

Some studies have already suggested that it could have a role in patients who have been "infected" with vCJD prions, but have not yet developed clinical signs of the disease.

Statistical experts say that although the number of people who have so far developed vCJD, probably as a result of exposure to BSE infected meat during the 1980s and early 1990s, is currently only in the hundreds, the true numbers likely to go on to develop it are unknown, as it may incubate for decades in the body.