Jean Angells' Fascinating Story

Here's a link to the story behind the Angell family's battle with ALS.

EMBRACING THE NEW NORMAL:
Jean Angell Speaks from Her Eye-Response Computer

Packard Center board member Jean Angell has lived with ALS for 10 years, the last six on a vent. What follows is the complete interview on which the story about her on the back page of the Winter 2008 ALS Alert is based. Read more!

Retired General Plans Attack on Lou Gehrig's Disease

Hat tip to the Times and Democrat for this imspiring article.

CHARLESTON -- 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, or ALS, 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 will also 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 breathing function decline 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 the government has a higher responsibility to advance ALS research.

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?"

Read more!

Reflections on a Job-esque Week

From Jim Jordan's website - November 4, 2007

I went to see one of my best friends in the hospital this week. He's struggling with Lou Gehrig's disease (ALS). Two weeks ago he passed out and fell in the same hospital while they were doing work on his wife's broken foot. Their travails have been horrible lately. On the way in I called his wife to see if they needed anything. It turns out that she needed someone to be there to receive my friend from his therapy session because her car had just been broad-sided by a delivery truck.

While I waited for him to be wheeled down I looked around for something to read and saw their Bible sitting by the window. It didn't take me but a second to decide what I wanted to review, I picked the book of Job. You know the story, Job was wealthy and happy, blessed in every way, until Satan made a deal with God that Job would be tested. Everything goes wrong in Job's life. His children are killed in a windstorm and he loses his livestock. Job's wife and friends give their two cents on what God was trying to tell Job by these terrible events.

There were two new things that stood out even though I'd read this story numerous times. The first thing I noticed was reading God's response to all the ponderings ("words without knowledge") of the humans in chapters 38 and 39. He uses nature to make His case. It becomes plainly clear that the idea that Job and friends would even try to "figure out" the Creator's purpose is absurd. In chapter 42, Job confesses his sin. This is the way The Message puts it:



Job answered God: "I'm convinced: You can do anything and everything.
Nothing and no one can upset your plans.
You asked, 'Who is this muddying the water,
ignorantly confusing the issue, second-guessing my purposes?'
I admit it. I was the one. I babbled on about things far beyond me,
made small talk about wonders way over my head.
You told me, 'Listen, and let me do the talking.
Let me ask the questions. You give the answers.'
I admit I once lived by rumors of you;
now I have it all firsthand—from my own eyes and ears!
I'm sorry—forgive me. I'll never do that again, I promise!
I'll never again live on crusts of hearsay, crumbs of rumor."



Job didn't know what he was talking about. And neither do we. There are times when God annoys me, honestly. But that is not a hindrance to my undying love for Him. Job was right to ask "Why me?" And many times I see the stupid things that happen to me and ponder the incredible turn of bad luck that my friend has had. Both my friend and I have grown closer to God in recent years and yet our fortunes have been ransacked by a superhuman assault during the same time. Thanks be to God? Do we blame Him? Do we accuse Him?

The second nugget I had overlooked in the book of Job was the name "Satan" in Hebrew. It means "accuser" or "adversary". It is a logical distinction for the prince of darkness but it speaks volumes to us. Do we desire to be the accuser, the adversary of God? Will our discontent, as rational as it is, become fertile ground from which we oppose our God? The answer is "no"!

A few days after my friend fell in the hallway at the hospital, passing out and falling dead weight onto the tile floor, his left eye was still bulging out of the socket, green, red, and black, I went to visit. I arrived just as the chaplain showed up to the room. Seeing he had a visitor she said she was praying for him and would come back later.

My friend sat crumpled in his wheelchair, swollen eye and broken shoulder, withered arms at his side, he leaned forward slightly and spoke softly. "God...is...good." He nodded his head for emphasis and then repeated the statement.

What a testimony! It's easy to blame God, to become His adversary because of the crappy hand you've been dealt. By all our human standards His timing is awful and His blessing of our fidelity is often nil. But there is nothing more rewarding than simply being at peace with your Creator. He is good. He is awesome. He is perfect. Did we not always know that?

Heavenly Father, You are almighty and all-knowing, therefore I abhor myself and repent in dust and ashes. Though you lead me to the cross, breaking down each of my muscles and limbs, even though you slay me, yet will I trust in you. For you are all there is, all there ever was, and all that will ever be. I stand in awe. I can do no other than worship you forever.

Give my friend strength in his journey and bless his wonderful and devoted wife with your peace that goes beyond understanding. Indeed "beyond understanding" is where you are taking us. In Jesus' name I pray, amen. Read more!

"She's Dying. His drug could save her"

From The Hook.

Mary Jane Gentry is going to die, and the UVA Health Sciences
Center, which has saved countless lives, has pulled away the experimental drug
that might save her.
"It was a ray of hope," she says, "and then they
stopped it."
When she was diagnosed 18 months ago with an aggressive case of
"Lou Gehrig's disease," Gentry knew it was a death sentence. Doctors told her
she had less than three years to live.
Desperate, Gentry-- herself a nurse
at UVA-- agreed to participate in a novel drug study. After eight weeks, she was
thrilled by a sign that the disease not only seemed to have slowed, but might
actually be reversing. She could suddenly move her left hand, which had been
useless for several months.
And her experience wasn't isolated: nearly half
of the study patients reported noticeable improvements in their condition while
none reported side effects.
So why did UVA halt the study?

Click on the link for the full story. Read more!