The condition of a Belfast man with variant Creutzfeldt-Jakob disease (vCJD) has stabilised after undergoing two years of experimental treatment for the degenerative brain condition.
Jonathan Simms (20) was diagnosed with vCJD three years ago. His family went to the High Court in December 2002 to seek permission to start treatment with an experimental drug called pentosan polysulphate (PPS). The drug must be given directly into the brain as it cannot pass from the bloodstream into brain tissue.
Mr Don Simms, Jonathan's father, told the Daily Telegraph that a medical evaluation had concluded that "Jonathan Simms is no longer terminally ill".
Dr Stephen Dealler, consultant in microbiology at Lancaster Royal Infirmary, said the young man's condition had become stable.
"Everyone was shocked at how well he responded. We had expected him to die in April but at a meeting to discuss his progress several months ago people realised that he had not gone downhill." Dr Dealler said Mr Simms was the first of 12 patients in Britain to receive the treatment and has shown the most progress.
Pentosan polysulphate is derived from beechwood and has been used in the past to treat cystitis. It has anti-coagulant and anti-inflammatory properties. However, because of its chemical structure it is unable to cross the unique barrier that separates brain tissue from blood vessels. It is, therefore, administered directly into the ventricles (deep chambers) of the brain via a catheter inserted by neurosurgeons. It is a potentially risky treatment, with haemorrhage a concern.
Dr Richard Knight, consultant neurologist at the UK National CJD Surveillance Unit said: "PPS may have some effects on the disease mechanisms of CJD but there is no evidence that it reverses or even halts neuronal death and the associated neurological dysfunction." He said there was no proven therapy for vCJD in clinically ill humans.
A different drug, quinacrine, is being assessed in a clinical trial at the National Prion Clinic in London. It too has toxic effects, especially on the liver, but it can be taken orally. Meanwhile, the young man with the first indigenous case of vCJD in the State is in a serious condition in a Dublin hospital. It is understood he is being considered for treatment with quinacrine or PPS.