The Journal Times, Racine, WI
By David Steinkraus

CALEDONIA - He can't muster a firm handshake; his muscles don't respond well. When Kevin Granger goes up one step into his home in western Caledonia, he needs an assist from his wife, Mary.

He has children, a modern home, a business, a summer place up north; he'll watch it slip away as his Lou Gehrig's disease progresses. If he matches the standard disease profile, he'll be dead in a couple of years, leaving his wife alone to raise the younger two of their four
children.

That's why Kevin, 44, is planning a trip to China in October where doctors will drill holes in his skull, and perhaps his spine, and inject fetal olfactory cells that he hopes will arrest the progress of the disease.

The disease Its scientific name is amyotrophic lateral sclerosis, or ALS; it became known as Lou Gehrig's disease after the Hall of Fame baseball player for the New York Yankees died from it in 1941. It is the death of the motor neurons, the nerve cells in the brain and spinal cord that control muscles. And it's fatal. Only 10 percent of the people who get ALS live for 10 or more years. Most die in three to five years after diagnosis, and death typically occurs when the nerve connections that control breathing break down.

In Kevin's case, the ailment first appeared as numbness.

"But I just thought I had a pinched nerve in my neck," he said. "I've had a pinched nerve for probably 15 years and would go to the chiropractor. My fingers would go numb, my two fingers. He would adjust my neck, and it would come back. So I thought, `Oh boy, it's getting worse, because my left hand was getting weaker.' " After a battery of tests, he thought his doctor would just hand him a prescription or a referral to a neurosurgeon. The physician told him of ALS.

"I said, `What do we do?' " Kevin said. "He said: `Nothing. You've got three to five years to live.'

"I walked out of there in tears."

It's odd where and in whom ALS appears.

"ALS is very common in veterans for some reason, and we're trying to figure out why," said Dr. Benjamin Rix Brooks, a professor of neurology at the University of Wisconsin Medical School who also practices at the veterans hospital in Madison. He is Granger's ALS physician.

"We know that athleticism is a risk factor, so Lou Gehrig, many football players. In Italy, many soccer players have it now," Brooks said. "The other thing, too, ALS is more common in Wisconsin."

In the early 1980s, he and a team of people published a paper about an ALS outbreak in Two Rivers, which they related to eating fish from a certain part of Lake Michigan. But like much about the disease, this is more elusive than illuminating.

"And so the whole issue of whether it's something in the diet, and what is it in the diet - is it mercury, is it some other toxins, all these kinds of things - still is up in the air," Brooks said. "We don't have a definitive answer. We just have a lot of exciting leads."

The state and the U.S. Centers for Disease Control are now developing a database to record information about ALS in Wisconsin in the hope of finding some pattern or clue.

Age is a risk factor for the disease, Brooks said. The older you are when you develop it, the more likely you are to die. In contrast there's Stephen Hawking, the theoretical physicist, who was diagnosed at the age of 21. He's now 62 and a grandfather.

Quest for answers Because Western medicine has few options, Kevin has looked to the East. Every few weeks, a big box arrives at his house. Inside are large plastic bags holding about 3 pounds of a Chinese herbal mixture that Mary patiently brews, over the course of about 3 hours, to produce five 16-ounce jars of liquid that looks like muddy river water. Granger drinks one jar each day.

"It tastes like chewing tobacco somebody spit out," Mary said.

That's not to say he's getting a great deal of benefit from it, Kevin said, but he thinks it keeps some of the more severe symptoms in check.

"The disease has progressed," he said. "My walking isn't very good. I can't write anymore."

Just a couple of weeks ago, Kevin said, the dog wrapped its leash around his legs as he stepped from his car. He fell and didn't have the strength to get himself up.

"It's just freaky to watch your dad fall down like that. And you can hear his head clunk against the ground," said daughter Melanie, 11. She and her 9-year-old brother, Jacob, helped Kevin get back on his feet.

"These kids have a pretty big weight on their shoulders," Mary said. "They do help out a ton.

"Really, when I'm not here they have to be me because I don't want him to go up and down the stairs if he doesn't have to, and even to get the refrigerator open is a maybe."

The inevitability of the disease gives impetus to his search for something to at least slow the ALS down. He read about the Chinese therapy, did as much background research as he could, then managed to get on the patient list through a contact in Wisconsin. It will cost $25,000 for the procedure, not to mention the cost of leaving home for a month.

"You know, the worst part about this disease - shouldn't say the worst - but one of the bad points is people will sell you any snake oil because you'll buy it. You're desperate. You'll do anything," Kevin said.

On the Internet you can find people advocating the use of "magnetic clay" which they claim draws metals and other toxins out through the pores of the skin.

"Listen to this. Listen to this e-mail," said Lee Martin, an associate professor of pathology and neuroscience at Johns Hopkins University School of Medicine in Baltimore. Omitting the person's name, he read: " `I would like to be considered for any human stem-cell trials for ALS. Reports say that it's too early to test on humans, but ALS does not allow much time. The medicine that is currently on the market is not helping ... I have nothing to lose and everything to gain.'

"That just tears me apart," Martin said.

His research involves the use of adult stem cells in mice to repair the damage of ALS. Drug and gene therapies haven't panned out yet, he said, so stem cells offer the most likely help. Adult stem cells particularly are promising because that would make it possible to use the body's own cells for healing. That evades the problem of tissue rejection, in which the body's immune attacks foreign cells.

The cells that Kevin will receive aren't true stem cells, Martin said. A true nerve stem cell could become any of the three types of specialized nerve cells, but olfactory glial cells develop into only the two types of supporting nerve cells; they don't become new neurons.

But, said Brooks, in ALS glial cells often become abnormal before problems arise in the neurons. So the hope is that replacing defective glial will prevent damage or repair it by protecting neurons and stimulating them to grow.

This work has particularly progressed in other countries, and most has been on repairing spinal cord damage. There have been some human tests in Australia, but done very quietly. The Chinese may be using fetal cells to increase the number of usable cells they can grow and perhaps to try and avoid the issue of rejection, Brooks said.

The risks With this early therapy come dangers. Scientists don't know what causes neurons to die in ALS patients. It may be a genetic defect in the neuron. But it may be that some of the supporting cells secrete chemicals that affect the neurons, and if that's the case, adding more cells will only aggravate the problem, Martin said. Experimental work now shows that motor neurons in animals and ALS patients produce special gates that, when triggered, start the neuron's self-destruct sequence, he said.

Then there's the absence of evidence about the Chinese technique.

"I have not seen any peer-reviewed, scientific literature from China on this topic. To my opinion, this is just a hearsay right now," Martin said.

But it's not a simple decision, he said.

"It's pretty easy for us to say that yeah, it's risky," Martin said. "I mean, Jesus, I don't have the disease. But I mean, these people are calling out for help, and we haven't been able to produce."

"It's not a cure," Kevin said. "It's not the answer, but if it can buy me some time ... If they come up with an answer in two or three years, I'll still be here."

After he was diagnosed with ALS, he and his wife built the summer home he had always wanted. He bought out his business partner to take full control of Kamm Glass. He goes to the office every day to deal with customers and do whatever he can to help his son-in-law manage the daily operations.

"I can't go anywhere; I owe too much money," he said jokingly.

He doesn't want to surrender.

"I don't want to say I'm handicapped," he said. "I've got my pride. I don't want to give up."

He also has two children whom he wants to raise with his wife. Given all that, he said, China looks like a good deal: "What do I have to lose?"