By Jill Coley - The (Charleston) Post and CourierPosted : Tuesday Jan 22, 2008 9:57:21 EST
CHARLESTON, S.C. — Four-and-a-half years have passed since Tom Mikolajcik was diagnosed with Lou Gehrig’s disease. Seventy percent of people with amyotrophic lateral sclerosis die within five years. Time is not on his side.
Now, Mikolajcik must make decisions about how he will face the last stages of the disease before he loses the ability to do so.
The degenerative disease, which is known for killing New York Yankees first baseman Gehrig, strikes about 15 Americans daily, shutting down nerve cells responsible for movement. Limbs weaken and atrophy before paralysis spreads to the trunk of the body. Eventually, speaking, breathing and eating are affected.
Patients must decide if they want to go on a ventilator and feeding tube to hold off the inevitable a little longer.
“Today, my decision is, I will put in a feeding tube even before I need it,” Mikolajcik said. “Today, my feeling is, I want to go on a ventilator as long as I can communicate with family and friends.”
The retired Air Force general and former commander of Charleston Air Force Base is taking charge of these critical decisions by participating in a medical study testing diaphragm-pacing stimulators in ALS patients. Located below the lungs, the diaphragm is the large muscle used for respiration.
The pacing device stimulates the diaphragm with surgically implanted electrodes to maintain muscle mass. The stimulator, already used in people with spinal cord injuries, might delay the need for a ventilator by more than a year.
During the surgery, a feeding tube also will be inserted, although Mikolajcik does not yet need one. “The sooner you have the procedure, the better,” he said.
Dr. Raymond Onders, director of minimally invasive surgery at the Medical University Hospital’s Case Medical Center in Cleveland, pioneered the technology and the procedure. The late actor Christopher Reeve, who suffered from a spinal cord injury, was Onders’ second patient to receive a stimulator.
ALS is a fatal disease, but theoretically, people could live indefinitely with a tracheotomy and ventilator. But most don’t want to do that, Onders said.
Doctors can predict when ALS patients will die based on their rate of decline in respiratory function.
To measure the success of the stimulator, Onders looks for decreases in that rate.
“It’s not a cure,” he said.
Mikolajcik was successfully fitted with a stimulator last week in Cleveland. He is part of a 100-person trial in six U.S. sites. Onders previously completed a safety trial implanting the device in 16 ALS patients whose decline in breathing function slowed, delaying the need for a ventilator by more than a year.
“I want to be able to listen, watch and absorb my children and grandchildren as they grow and change,” Mikolajcik said. The mind and senses remain unaffected by the disease. But as time passes and the body shuts down, the ability to communicate diminishes.
Toward the end of the disease, some people use their eyes, looking right or left to signal “yes” or “no.” In preparation for the time he will become speechless, Mikolajcik recorded himself singing “A Bushel and A Peck” to be played when his grandchildren are placed in his lap.
In August 2003, the retired general went to the doctor with a minor complaint: He was feeling tired and not hitting his golf balls as far, he said. The doctor noticed a slight twitching in Mikolajcik’s chest called fasciculation.
The doctor told him the best-case scenario was a benign tic, and the worst case was ALS. Mikolajcik went home and Googled ALS.
“I almost fell out of my chair,” he said. The muscles in his arms shut down first, then his legs. He can move his left thumb and index finger, and if he concentrates and is well rested, he can move his left wrist and ankles. Little is known about ALS, which was discovered in 1869.
“In 70 years, there’s only one questionable drug that may extend life by three years,” Mikolajcik said. “In 70 years. Give me a break.”
For unknown reasons, veterans have a 60 percent higher chance of developing ALS. That high rate is why Mikolajcik said he feels strongly that the government has a higher responsibility to advance ALS research.
He has visited Congress three times to push for ALS research and testified before a congressional committee in July. Legislation to establish an ALS database that will warehouse information on the disease for scientists and patients has passed the House and is awaiting consideration by the Senate.
“I am blessed that I’m a Type-A personality,” Mikolajcik said. “What about those who take no for an answer?”