Ride For Life: Patient donates $100K to fight PLS & ALS

Pam Wakefield sits with her husband Steve for two-and-a half hours every night while he slowly grinds away at his dinner, chewing excruciatingly slowly because he cannot control his tongue, like many of the muscles in his body.

She laces his shoes and buttons his shirtsleeves for him each morning before he goes to the office or a physical therapy session. Steve Wakefield suffers from a rare neurological disease known as Primary Lateral Sclerosis, which has been crippling motor neurons in his brain and spinal cord for seven years.

To help find a cure for PLS and Lou Gehrig’s Disease, a similar disorder, the Wakefields donated $100,000 to Emory neurologist Jonathan Glass’ research last year.

“We just had $100,000 lying around,” Pam said. “No, just kidding...Really, we just think so highly of Jonathan Glass.”

Glass will use the donation to begin testing a molecular “library” of new compounds for the ability to prevent the disease in genetically engineered mice.

“We have a lot of different ideas, none of which are proven yet,” Glass said.

The donation will also go toward testing cures for Amyotrophic Lateral Sclerosis, Lou Gehrig’s Disease’s scientific name, a more common disease that is closely related to PLS.

“In our laboratories, we are looking not only for treatments for ALS and PLS,” Glass said, “but we are also studying genetic and environmental factors that may play a role in the cause of these diseases. Steve’s support will prove valuable for all of these endeavors.”

Diagnosing the disease

Steve Wakefield first noticed something was wrong when he stumbled during a jog and then developed a limp.

“We went to 25 or 30 doctors,” Pam said. “No one knew what it was. They said it was a back injury, a virus, a lack of B-12, lyme disease.”

But, Jeffrey Rothstein, who heads the Robert Packard Center for ALS Research at Johns Hopkins in Baltimore, recognized the early signs of PLS in Steve’s tightening muscles.

Rothstein told the Wakefields PLS was a blessing compared to ALS, which is terminal and causes complete muscular collapse.

“When we left, we thought we had won the lottery,” Pam said.

The Wakefields began going to clinics but were discouraged by the long waits and lack of personal care.

Rothstein recommended they switch doctors to Glass, who had been his college roommate and is Atlanta’s leading expert on ALS.

Glass instantly put the Wakefields at ease.

“He’s just a little cheerleader,” Pam said. “He’s upbeat. He and Steve e-mail back and forth — information about the research that’s going on.”

Finding a cure

Victims of ALS and PLS remain mentally alert, while their bodies slowly deteriorate.

“You’re really nailed inside your body,” Pam said. “You’re really isolated.”

In 2000, Steve lost his ability to speak. He can make limited gestures and bark out a few words that Pam interprets. But mostly, he communicates by writing messages on an erasable Fisher Price Magna Doodle, a children’s magnetic drawing board.

Other times, Pam has to guess what he wants to say.

“Sports?” Pam asked him. He began scratching out a reply on his board.

“Are you listing the sports you do or the ones you watch?” Pam asked. “He doesn’t do many sports any more.”

Steve turned the board around to reveal a list of the sports he enjoys watching: golf, running, skiing, hiking.

Later, in his office, he pointed to a photograph taken before his illness of a road race he ran with his youngest son.

There were other pictures of the Wakefields’ former life — one taken with George W. Bush, then a gubernatorial candidate in Texas, another with President Ronald Reagan.

Steve was assistant secretary of energy under Presidents Richard Nixon and George Bush Sr. before returning to private practice as an attorney in Houston and Atlanta.

He still works in a law firm three days a week. He drives himself to work using a wheelchair in place of a driver’s seat.

When seated, Steve can use the pedals because he regains almost full use of his legs. Pam said he still drives to work because he refuses to be defeated by the disease.

“He’s just a determined man. He’s an A-plus man,” she said. “And his work is so important to him.”

Glass measures the spread of Steve’s disease every month by testing the responsiveness of his legs to pressure.

“It probably isn’t progressing,” Pam said. “But then how could we know? Every time he goes to Dr. Glass, he is always as strong as the time before.”

Steve is Glass’s longest patient.

“Most of my patients die within two years of my seeing them,” he said. “But I’ve been seeing him so long.”

Glass said every hour he spends in the laboratory is another step toward discovering a cure.

“If there is nobody out there looking for the answer, the answer will never be found,” he said. “This is why we get up every morning: because someone has to find the cure, whether we find the cure or someone else does.”