High-tech brain surgery keeps girls alive

Mystery disorder ... Leanna, left, and Bethany Mills suffer from a rare movement disorder but have been saved by electrodes implanted in their brains. Left: Broken electrode, an X-ray of Leanna's head.

NICHOLAS MILLS has been battling for more than a decade to keep alive his two daughters, who are plagued with an extremely rare and debilitating movement disorder for which there is no known cause or cure.

Mr Mills and his wife Michelle, from Newcastle, were delighted when their daughter Bethany, 12, got out of her wheelchair and began to speak normally following complex $100,000 brain surgery in France four years ago.

"Bethany couldn't get off the floor, couldn't raise her head and was on so many medications, but two weeks after the operation she pushed me out of hospital in a wheelchair, it was amazing," Mr Mills said. Her sister Leanna had the same treatment, in which electrodes are placed deep into the brain and connected to batteries implanted under the skin, but the 14-year-old urgently needs another round of life-saving treatment after an electrode failed.

Deep brain stimulation is available in Australia for adults but not for children, necessitating rounds of fund-raising by the local communities to supplement funding from the Federal Government's Medical Treatment Overseas Program. But Mr Mills says Leanna must be on a plane to the Montpellier University Hospital Centre by the end of the week or she may not survive. "And that doesn't give us enough time to sell raffle tickets," he said.

He quit his job in the coal industry when the second and third of his four daughters suddenly and mysteriously became ill as two- and four-year-olds in 1998. Their symptoms, a mix of cerebral palsy and Parkinson's disease symptoms such as tremor, twisting or spasmodic movements and stiff limbs, were so unique that Mr Mills was investigated by child welfare authorities on suspicion of poisoning them.

The condition, called primary dystonia, was eventually diagnosed by experts at London's Great Ormond Street Hospital for Children, but an exhaustive battery of tests here and overseas have yet to pinpoint a genetic cause. Their two sisters, Katey, 16 and Olivia, 10, are perfectly well.

"It's been a constant battle. Katey is 16 going on 100 because she's been a carer for her sisters and I can't work so money's so tight, but we won't give up," Mr Mills said.

A pediatric neurologist at John Hunter Children's Hospital, Robert Smith, has been treating the girls for 12 years, and says it is possible they are the only two people in the world to have their particular range of symptoms.

Bethany's batteries will have to be changed every two years but for now she can run, attends school with the help of a carer and is progressing in writing. But Leanna, who originally had a milder form of the dystonia. But she did not respond as well as her sister to the surgery, and has severely deteriorated since the lead connecting an electrode to a battery broke a few weeks ago [see x-ray].