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Family finds hospital willing to give experimental CJD treatment

Owen Dyer, London

A teenager suffering from variant CJD, the human form of mad cow disease, is to have unprecedented treatment with a drug injected into his brain after the high court in Belfast gave the go-ahead.

Jonathan Simms, 18, from Belfast, is expected to have the treatment with pentosan polysulphate within weeks after a Northern Ireland hospital agreed that it could be carried out in its operating theatres.

His family, which is desperate for him to have the pioneering treatment in a last attempt to slow down the progress of the disease, was forced back to court after winning a ruling in the English high court the previous week. The hospital wanted the Northern Ireland high court's approval because the English ruling has no effect in the province, which is a separate legal jurisdiction.

Jonathan's family was one of two fighting for the treatment for their teenage children who have variant Creutzfeldt-Jakob disease (vCJD). They won permission in London's High Court to proceed with an experimental treatment not tried before in humans. But, although a neurosurgeon has agreed to do the procedure, the NHS trust for which he works immediately announced that it would not provide the necessary facilities.

The case was heard in camera, with the names of the neurosurgeon, the trust, and one of the patients protected by injunction. The judge, Dame Elizabeth Butler-Sloss, had power to permit the treatment but could not compel any hospital to provide it. In summing up, she sharply criticised the trust for refusing to announce its decision in advance. "It would be an unbelievably cruel blow" to the families, she said, "to have the High Court say yes and the hospital trust say no." The court case, she said, would become "an unacceptable academic exercise."

At the time of going to press, the Department of Health was looking for an alternative hospital for the other patient, an unnamed 16 year old girl. Both patients have already outlived the typical life expectancy of patients with vCJD.

The proposed treatment, pentosan polysulphate, is widely used in North America for the treatment of interstitial cystitis but is unlicensed in Britain.

Although it has never been used to treat vCJD, experiments in Japan showed that injecting the chemical into brains of rats infected with scrapie, a closely related disease, can slow the accumulation of prions. Pentosan polysulphate was rejected as a possible CJD treatment by Britain's Committee on Safety of Medicines and the CJD Therapy Advisory Group, though both organisations are now reconsidering their advice.

Expert witnesses were largely in agreement about the potential risks and benefits of the treatment, which involves inserting a catheter into the brain. A conservative dose of pentosan polysulphate will be steadily increased. The risk of brain haemorrhage is believed to be under 5% Chances of a cure are probably nil, however, and even a substantial slowing of progression will be unlikely.

(Credit: PAUL FAITH/PA) Jonathan Simms is to receive controversial treatment