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March 15, 2005 – Sick woman puts best face on critical day ALS patient undergoes innovative surgery at UH

By: Diana Keough

Monday was a big day for Susan Larson. So big, in fact, she insisted her husband, Lonnie, curl her hair and apply makeup before taking her to University Hospitals of Cleveland for surgery.

"I told her nobody was going to see her, but she wouldn't let it go," says Lonnie. Susan, 49, has Lou Gehrig's disease, and on Monday doctors at UH implanted an electronic device into her diaphragm, hoping to allow her to continue breathing on her own, and thus prolong her life.

The blond, petite woman from Rogers, Ark., is the first patient with Lou Gehrig's disease (formally known as amyotrophic lateral sclerosis, or ALS) to undergo the procedure actor Christopher Reeve, who was a quadriplegic, had two years ago.

"We're hoping to not only stimulate the diaphragm muscles that are no longer working, but also see if we can maintain the muscles that are left and perhaps even improve them. It's the principle of use it or lose it," says Dr. Raymond Onders, Larson's surgeon and director of the minimally invasive surgery.

Larson was diagnosed with ALS three years ago. Before her disease, she was a tennis and golf player.

She also was a pediatric nurse specializing in autism. Lonnie, her husband of 28 years and full-time caretaker, found Onders online.

"But it was Susan's decision to pursue this surgery," Lonnie said.

Susan can no longer move her arms or legs, is fed through a port in her stomach and is unable to communicate, except by groans and moans Lonnie can somehow interpret. She is still able to breathe on her own.

Onders said the earlier in the disease the procedure is done, the better.
"We know that once ALS patients get below 50 percent lung capacity, they can live for about nine months without help from a ventilator," says Onders. Susan's lung capacity is still at 50 percent. No surgery, he said, can change the natural outcome of the disease.

Working through a long, silver, skewer-looking laparoscope, Onders placed four electrodes in Susan's diaphragm. The electrodes were attached through wires under the skin to a small external battery pack that electrically stimulates the muscle and phrenic nerves, causing the muscle to contract and air to enter the lungs.

Every year, an estimated 5,600 Americans are diagnosed with ALS, a muscle disorder caused by a nerve dysfunction. There is no cure. As the disease runs its course, most ALS patients eventually need a ventilator, which increases the risk of infection. For Susan, a ventilator also means constant noise and even further restricted activity, Lonnie says.

"Susan decided that if she needs a ventilator, she's going to let the disease take its course," says Lonnie.

The operation on Larson marks the beginning of a study by Onders, UH pulmonologist Robert Schilz and UH neurologist Bashar Katirji. They hope to enroll 10 ALS patients in this procedure. It is considered experimental, though Onders said it was approved by the Food and Drug Administration in October.

Onders has performed this surgery successfully on 15 patients with spinal cord injuries and says he always thought it might work as well in ALS patients.

Diaphragm muscles in spinal cord patients need to be jump-started; in ALS patients, the motor neurons within the muscles die. Until he went in Monday morning, Onders was unsure what he would find. He noted massive "striping" along the walls of Larson's diaphragm, indicating nonfunctioning motor neurons. (The diaphragm contains thousands of motor neurons.) Onders hopes that the electrode stimulation will allow functioning motor neurons to continue to work as the surrounding motor neurons die.

Susan will return to UH in 12 days for Onders to activate the device, but it will take two to three months before he knows if his hunch is right. But even then, he's not really sure what the outcome will be. Best-case scenario: This surgery will prolong Susan's life, Onders said.

That's what Lonnie and Susan are hoping for. Next year, there are two events she doesn't want to miss: Their 25-year-old son, Nicholas, is getting married in May, and their 17-year-old daughter, Stephanie, is graduating from high school in June.

Lonnie said of his wife: "She's a real fighter, and she's going to do anything she can to keep fighting."

To reach this Plain Dealer reporter:
dkeough@plaind.com, 216-999-4927