Risking All For A Cure

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When Jonathan Simms was 17, doctors thought he had 14 months to live. The Belfast teen was exhibiting the first symptoms of variant Creutzfeldt-Jakob disease (VCJD), the human version of mad cow disease, and there could only be one prognosis. But three years later, his condition is no longer considered terminal. Thanks to a controversial new therapy, he may become the first known survivor of a disease that has killed 147 Britons since 1995.

After the diagnosis, Simms' father Don read about the use of an anticoagulant called pentosan polysulphate (PPS) to delay the onset of scrapie, a disease which produces similar brain lesions in sheep as VCJD does in humans. The drug was not licensed for human use in Britain — and doctors were un-willing to test it on Simms until Don secured the High Court's permission in late 2002. Within months Simms' condition stabilized, then improved. Five other victims are now being treated with PPS, with similar results. Doctors believe PPS shuts down the "rogue" prions blamed for the disease. Experts disagree on future numbers of VCJD cases; some say there's an epidemic still to come. If so, the PPS treatment offers hope that a diagnosis is no longer a death sentence.