Lou Gehrig's disease hurts his body; technology helps his brain

Andy Eddowes suffers from amyotrophic lateral sclerosis, or ALS – Lou Gehrig’s disease. Researchers have yet to fully understand the disease, which shortens the lifespan of those who have it. STEPHEN M. KATZ PHOTOS / THE VIRGINIAN-PILOT


By ELIZABETH SIMPSON, The Virginian-Pilot
© December 3, 2006



ANDY EDDOWES still has muscle enough in his face to wince at the idea of having Lou Gehrig's disease before the age of computers.

The muscle-wasting disease - known in the medical world as amyotrophic lateral sclerosis - already has worked its way through the legs, arms and hands of this 46-year-old retired Navy captain, and partway through his voice.

But as is the case with most people with the disease, his mind is marvelously intact.

Just through the lingering sensation left in his fingertips and neck muscles he can download and play his favorite music. He can e-mail Navy buddies from the air squadron he once commanded. He can read books and magazines. He can research his disease to find out what comes next, then order equipment that will help him to stay in the game when only his eyes can move.

It's a far cry from what predecessors with his disease were left with in their final stages:

Blink once for yes, twice for no.

The disease that afflicts some 30,000 Americans at any given time is a grim one, regularly mentioned in discussions of physician-assisted suicide. But a saving grace of the times is the technology that has unleashed alert minds from paralyzed bodies.

Electronics and computers have kept people engaged with the world, using everything from portable speaking devices to computers operated by eye movement.

"Not only has the technology expanded, but the prices have come down, so people can afford devices to talk with their doctors, tell their wives, 'I love you' and their children they're proud of them," says Catherine Easter, the Virginia director for the ALS Association.


Andy Eddowes uses a DynaVox communicator to talk for him. Technology has helped him be able to communicate with others and to keep his mind active.


In addition, a field called "brain-computer interfaces" is developing in which people can mentally move a computer cursor with a system that measures brain waves, or with a chip implanted within the brain.

It may sound like science fiction, but researchers are testing systems in which people paralyzed by ALS, cerebral palsy and brain injuries use thought to operate computers and move robotic arms.

The equipment Eddowes is using today would have seemed revolutionary to people a decade ago, but it will be rudimentary 10 years down the line.

Although his voice is barely understandable, he continues to have philosophical discussions with friends about politics and the war in Iraq. He's assembling a photographic family history for his 12-year-old daughter, Rachel. And he shares with a reporter the philosophy that has led him through the darkness of his disease.

"The guide I use to make my decisions is something I heard as a kid," he writes in an e-mail. "I think it was a coffee shop advertisement.

"Here it is: 'As you ramble through life, brother, whatever be your goal, keep your eye upon the doughnut, and not upon the hole.' "


If you're looking here for a character like the professor in the best-selling book "Tuesdays With Morrie," an ALS patient who shares evocative, end-of-life lessons, look elsewhere.

"It always looks darkest..., " Eddowes says through a voice-generating device, "just before it goes pitch black."

Eddowes is less emotional and introspective, more analytical and problem-solving. He's upbeat, but no Pollyanna when it comes to the disease, which he describes as "death by a thousand cuts."

Here's his computer-typed explanation of the disease: "ALS causes the nerves that control your voluntary muscles to die. And when the nerve dies, the muscle it controls also dies. Voluntary muscles include anything you can control, like your arms and legs, but also your breathing. ALS doesn't affect your brain or memory, only your muscles."

He pulls no punches with the prognosis of this disease, which researchers have yet to fully understand:

"Life expectancy for ALS patients averages three to five years from diagnosis. There's no cure."

But there is technology that can help fill in some of the holes. ALS affects people differently, and at different rates. For some, the voice goes first, but they have muscle enough to click a mouse to communicate. Others can talk but can't move their limbs. For them, voice-recognition devices can allow them to turn on lights and adjust the television volume just by talking.

The disease is a moving target. What helps someone communicate today may not tomorrow, b ut usually there are adjustments that can be made, or other devices waiting in the wings.

Not all insurance policies cover communication technology - which can cost tens of thousands of dollars - which is why groups like the ALS Association have equipment-loan closets and special grants, not just for communication devices but also for wheelchairs and equipment that helps them get out in the world.

One of the toughest aspects, though, is thinking ahead to make sure equipment is in place to substitute for the next muscle ALS is likely to steal.

It's rarely an encouraging exercise.


But it's something Eddowes - a planner by nature - cannot help but consider.

On this day in late October, he uses a combination of ways to communicate:

Computer messages he's already programmed. Keying in new responses. Snippets of information he's able to relay in the monotone his voice has settled into of late. And Ellen, his wife, who fills in the gaps.

The process is painstakingly slow at times. Nothing like the TV medical show renditions of ALS patients whose words magically appear on the screen within seconds.

But it works.

"Rather than worry about the things I couldn't do anymore, I've tried to concentrate on what I could do and on figuring out new ways to continue doing the things I enjoy."

In the dining room of their Virginia Beach home, Ellen places Eddowes' hand, dead weight but for the fingertips, on a mouse attached to a DynaVox speech-generating device mounted before him.

"Where do you need your hand?" she asks, adjusting it just so on the mouse. "Is that too high?"

She pulls it down slightly. Eddowes clicks on a panel, and an electronic voice says:

"I first noticed ALS symptoms in the fall of 2002, but I didn't know what they were."

The first symptom, he remembers, was a thickening feeling in his throat. In the spring of 2003, he felt weakness in his left hand, followed by random spasms up and down his left arm and side. "Like Christmas tree lights," he says.

He was transferred from Italy - where he commanded an electronic reconnaissance squadron - to Hampton Roads so he could be near doctors at Portsmouth Naval Medical Center. He was about to undergo surgery on his spine when an orthopedist first recommended he see a neurologist in November 2003.

The symptoms were so clear by then, the diagnosis was easy. He took medical retirement from the Navy in April 2005, retiring as a captain after 22 years of service.

A year and a half ago he began using a speech-generating device that can be mounted to his wheelchair. By clicking on panels, he can call his dog, Quinto, say, "Good morning," turn on the TV, adjust the volume and talk with others about his care.

When he lost the strength in his arms needed to turn pages of a book, he went online and found a $3,500 device that turns pages. His wife told him that for $3,500, she'd turn the pages for him. So he found a Web site called www.bookshare.org, where he could read books for $75 a year. He's read everything from Harry Potter novels - a passion he shares with his daughter - to Jimmy Carter's latest book on conflict in the Middle East.

Carlos Urroz is there to help connect the wires to the world. He is an assistive technology manager with the ALS Association and visits more than 300 people with ALS in Virginia, Maryland and Washington. The computer engineer works in conjunction with speech pathologists, occupational therapists and rehabilitation specialists.

Some people prefer low-tech, such as alphabet boards, to spell out words. At the high-tech end are "eye gaze" computers that people can use by looking at the screen to type out letters.

There are all manner of variations in moving a cursor: Some use their cheek or chin or eyebrow, some a "puff and sip" method on a tube. Even if they lose the ability to talk or walk, they can do their banking online. They can get involved in advocacy work. They can read books, magazines, keep up with sports scores and e-mail friends.

"It's like turning on a light sometimes," Urroz says.

"Sometimes they were depressed because they weren't interacting with people, they were just looking out the window. The computer becomes their life because it gives them a way to access the world."

It also allows them to weigh in on medical decisions. When their throat muscles become so weak they can't swallow, do they want a feeding tube? As their breathing becomes labored, are they willing to go on a respirator? Once they're on a respirator, is there a point - say when they can no longer communicate at all - when they want to be removed from life support?

It's that point - unable to interact with the world - that health experts refer to as a "locked in state."

A study by German scientists released in the Psychophysiology journal in November found that using computers to engage people with ALS before they reach that point can slow the progression of the disease.

Urroz tries to match people's abilities and interests and comfort levels with devices to keep them engaged, whether it be through a simple gesture or sophisticated electronics.

"Any kind of communication keeps the mind active."


Eddowes has created a haven of sorts in his computer room.

He can play Handel's "Messiah" or Steely Dan or one of hundreds of other songs he's downloaded onto his computer.

A tiny patch in the corner of one of the lenses of his glasses is calibrated to an infrared light on the computer that allows him to move a cursor, type messages and surf the Internet.

A word-recognition program means he only has to write a few letters of a word and five words pop up. He picks the right one and moves on.

He e-mails friends. He writes letters to raise money for ALS research and organizes trips to Washington to lobby legislators. He downloads photographs and designs scrapbook pages of a family trip out West that he, Ellen and his daughter made while he could still walk.

"I've always enjoyed figuring things out," he writes. "ALS has simply provided me with a whole new realm to attack.... I don't view new technology as a surrendering of capability, but as way to maintain capability."

Urroz is already on Eddowes's schedule to return sometime later this month to figure out what devices he needs next because clicking a mouse with his fingertip has become harder.

Maybe a sip-and-puff device, or using his cheek or his lip to move the cursor for the day when he can no longer move his head. They'll also talk about technology he can use in a lying down position to prepare for that stage.

There's no easy way out of this disease.

"I don't try to pretend that ALS won't get me in the end. I just refuse to give up anything before I absolutely have to. As the flag on the back of my wheelchair says, Don't Give Up The Ship. I've had to give up a great many things so far. But I still have enough that I can do to fill my days and bring some happiness."

One thing about ALS, he says: It gives a person time to think, time to track the doughnut even as the hole gets bigger.

He's in this for the long haul. He already has a feeding tube in place for when he can no longer swallow, and he'll let a ventilator breathe for him "when the time comes."

But not a moment sooner.

"ALS has changed the way I live," he writes. "I need help doing things like dressing and eating now, but it hasn't changed who I am. And it can't change anything that's already happened in my life.

"As I see it, ALS only has power over me if I decide to give that power away.

"And that's something I'll never do."


Reach Elizabeth Simpson at (757) 446-2635 or elizabeth.simpson@pilotonline.com

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