THE LUCKIEST MAN: LIVING WITH LOU GEHRIG'S DISEASE
As ALS disease progresses, one O'Hara family makes adjustments
December 6, 2013 4:47 PM
Pam Panchak/The Pittsburgh Press
Neil Alexander of O'Hara watches as his wife sets up a diaphragm pacing system he is using in response to the advancing symptoms of ALS.
Pam Panchak/The Pittsburgh Press
Neil Alexander of O'Hara shows the diaphragm pacing system he is using in response to the advancing symptoms of ALS.
By Kaitlynn Riely / The Pittsburgh Press
One in an occasional series.
The new device that Neil Alexander will keep by his side is neither sleek nor stylish. It looks, joked his wife, Suzanne, “like a Sony Walkman from the 1980s.”
But the O’Hara couple, says Mrs. Alexander, are “thrilled” to have it.
The tan-colored boxy device operates the diaphragm pacing system that Mr. Alexander had surgically installed last week at Case Medical Center in Cleveland. The Alexanders think the technology, made famous in 2003 when "Superman" actor Christopher Reeve used it and recently approved by the FDA for use in ALS patients, will prolong the 48-year-old’s ability to breathe independently.
It is the latest step in the Alexanders’ journey with ALS. And it is, said Mrs. Alexander, the “first of many invasive measures to prolong his independence.”
Nearly two-and-a-half years ago, Mr. Alexander, a father of two young children and then director of corporate services for Downtown-based financial management firm Hefren-Tillotson Inc., was diagnosed with ALS, also known as amyotrophic lateral sclerosis or Lou Gehrig’s disease. ALS affects the motor neurons, usually leading to paralysis and difficulty breathing and swallowing. The average patient dies two to five years after diagnosis.
The Alexanders decided to be proactive in their approach to the disease, with the goal of extending Mr. Alexander’s independence and life as long as possible. They formed LiveLikeLou.org, a donor-advised fund of the Pittsburgh Foundation, to raise awareness of the disease, fund research and support patient care.
Since March 2012, the organization has raised $625,000, with funds going toward initiatives including a $100,000 grant for medical research at UPMC and a college scholarship program for children of people diagnosed with ALS.
And they have made changes in their lives, some big, some small. Mr. Alexander switched to an electric toothbrush as the muscles in his hands began to atrophy. Last year, the Alexanders built an addition to their home so their bedroom and bathroom would be accessible once Mr. Alexander started using a wheelchair. He started using one earlier this fall, when the disease started making walking more difficult and causing him to fall. He now uses the wheelchair for most trips outside his home.
So far, his voice and his ability to swallow and eat have remained largely unaffected by his ALS. But in recent months, Mr. Alexander, a swimmer, started feeling shortness of breath and tightness in his chest.
“When it’s breathing, especially, that’s when you go, ‘uh oh,’” Mr. Alexander said.
David Lacomis, co-director of the Center for ALS Research at UPMC, said Mr. Alexander’s capacity to move air in and out of his lungs has declined by about 45 percent since he diagnosed him in June 2011.
“It’s not terrible, but it’s a substantial decline,” he said.
The Alexanders had planned to pursue the diaphragm pacing system, an option approved for ALS patients by the FDA in September 2011. At least five other Pittsburgh-area ALS patients have had the surgery here or in Cleveland, said Dr. Lacomis.
Last month, the Alexanders traveled to Cleveland to meet with Raymond Onders, professor of surgery at Case Western Reserve University School of Medicine and the doctor who developed the diaphragm pacing system. It was originally used in patients who had respiratory problems due to spinal cord issues and in 2003, Dr. Onders implanted the device in Reeve, the actor who required a ventilator to breathe after he was paralyzed in a horse-riding accident.
After the publicity that Reeve’s surgery received, Dr. Onders started getting the question: if the device could help wean patients with spinal cord injuries off the ventilator, could it delay the need for ALS patients to get on a ventilator?
Dr. Onders said his research shows that it can, by about 18 months. The pacing system works by stimulating the diaphragm muscle, which supports the breathing process. Electrodes are surgically implanted in the diaphragm and then attached through the skin to an external battery-powered generator, which sends electrical pulses to stimulate the diaphragm.
In the past 14 years, about 900 people, most of them with ALS, have received the device, and on the Monday before Thanksgiving, Mr. Alexander traveled to Cleveland to became one of them.
He knew, from his own symptoms and his doctors in Pittsburgh, that his breathing was declining, but when Dr. Onders saw him, he said the state of his diaphragm indicated Mr. Alexander’s ALS was at “a much later stage,” with the right side of his diaphragm nearly unable to be stimulated. The discrepancy suggested that Mr. Alexander had been using accessory muscles in his chest to compensate for the weakening of his diaphragm, Dr. Onders said.
Mr. Alexander is now using the diaphragm pacing system, with the goal, Dr. Onders said, of maintaining the left side of his left diaphragm. Eventually, Mr. Alexander plans to use the system constantly, but for now he is turning it on for just a few hours a day to let his body get used to what he says feels like an “electrical flutter” in his chest.
He is still recovering from the surgery, hopeful that his new device will slow the rate of deterioration.
“We wouldn’t know unless we cloned Neil if the different things that we do are helping him,” Mrs. Alexander said. “We think it’s prolonging his independent breathing, and the only way to know that for sure is if you made a duplicate of him and watched the progression of the disease over time.”
The future, they said, will likely bring additional changes as the Alexanders seek more measures to prolong Mr. Alexander’s life and its quality, such as a feeding tube and a catheter for urinating.
Among these changes, there are some things, however, that haven’t changed for the Alexanders and their children, 11-year-old Abby and 9-year-old Patrick.
The Alexanders plan to make their annual Christmas Eve morning trip to the Strip District to shop for their traditional family seafood feast. Mr. Alexander, his diaphragm pacing system installed, is determined to make the journey in his wheelchair for the first time, his wife by his side.
“Hopefully, the seas will part,” Mrs. Alexander said.
Kaitlynn Riely: firstname.lastname@example.org or 412-263-1707.
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