Yahoo! News   Fri, Aug 22, 2003
Health - Reuters
First Parkinson's Gene-Therapy Patient Keeps Faith
Wed Aug 20, 3:44 PM ET

By Ransdell Pierson

NEW YORK (Reuters) - Two days after becoming the first adult to receive gene therapy for Parkinson's disease (news - web sites), Nathan Klein said he was glad to have taken "a big step forward, or maybe backward" to help himself and others with the progressive movement disorder.

The 55-year-old television producer was up and about, and in wise-cracking good spirits on Wednesday, at a press conference at New York-Presbyterian Hospital, where surgeons performed the risky five-hour brain operation on Monday.

"It feels good to get all this attention. I've never been on this end of the camera before," said Klein, who helped produce the syndicated television program "Everyday with Joan Lunden" in the early 1990s.

Klein said he felt no better or any worse than before the operation, during which Dr. Michael Kaplitt delivered gene therapy droplets to the target area of his brain through an opening in his skull.

The droplets contained countless copies of a normally occurring gene, called GAD, that doctors hope will begin producing its designated protein. The protein, in turn, is meant to produce a molecule called GABA, whose role in the brain is to calm overexcited nerve cells.

The genes are unable by themselves to enter brain cells. So each copy was stuffed into a seemingly harmless virus called the adeno-associated virus, which can penetrate human cells and drop off its gene cargo.

Parkinson's, which affects 1.5 million Americans, is a progressive disorder in which damage to nerve cells in a deep part of the brain eventually causes muscle shaking or rigidity, poor coordination and difficulty in walking.

The nerve damage disrupts production of GABA as well as a brain messenger chemical called dopamine that sends nerve signals to muscles.

"GABA normally acts as a brake to control firing of neurons, but the firing becomes extremely rapid" among people with Parkinson's, said Dr. Matthew During, a medical professor at the University of Auckland who helped conduct earlier animal trials of the gene therapy technique.

The hope is the transplanted genes will spur production of enough new GABA molecules to replace those lost to the disease.

Klein is the first of 12 patients with advanced Parkinson's disease the hospital aims to treat with the technique, which was approved by U.S. regulators. All must have had the disease for at least five years and no longer benefit from available drugs and surgical treatments.

New York-Presbyterian plans to begin treating the second patient in about a month, after assessing whether Klein is safely weathering the procedure.

In the meantime, doctors said they hope he will not encounter serious side effects from the therapy, including fever and potentially dangerous brain inflammation.

Klein, his full head of silver hair parted on either side of an eight-inch surgical scar, exuded nothing but optimism.

"I hope the gene therapy gives me a chance of getting better because right now I have trouble walking and can't play ball with my son," he said in an interview.

He said he was no longer helped by Parkinson's treatments, including a widely used pill called levodopa that is converted into dopamine when it enters the brain.

Klein said he plans to return home today to Port Washington, New York, and enjoy some good home cooking prepared by his wife, Claire.

"A cheeseburger would be fine; nothing too healthy," said Klein, whose 14-year-old twin daughter and son will help throw him a return-home party over the weekend.

Scores of gene therapy trials have been launched without success since 1990, for a wide array of diseases, including cystic fibrosis, AIDS (news - web sites) and cancer.

Two French boys with severe immune deficiency were cured with gene therapy, but they later developed leukemia -- casting a cloud over the potential of such treatments.


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