Johnny by the Sea

Johnny, Debbie, Ryan and Shannon out on the pool deck. Ryan and Shannon flew in for a few days from Tampa once again. Wednesday afternoon, April 9th, 2008. [Note that the date for this post is to ensure that it stays at the top of the blog] Read more!

Johnny Getting A Hair Makeover

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Johnny Poolside and Surfside

The pool deck is Johnny's favorite hangout. The neighbors all greet him and mingle. When that gets boring he enjoys watching the waves....
Photo is from Saturday, May 4th, 2008. Read more!

"A Latter Day Lou Gehrig Survives and He Demands to Be Heard"

This New York Times article of the 15 year struggle by Chris Pendergrast with ALS is a breath of fresh air. Full story below:

For a man so active, a blur of energy climbing mountains, running fishing-boat charters, fixing cars and moving, always moving, it began as weird glitches in the gears.

There were twitches in the eyes and hands, spasms and cramps in muscles he didn’t know he had. Then it got worse. Chris Pendergast, then a 44-year-old elementary school teacher from Miller Place on Long Island, who could hike forever, would be walking across the lawn and then fall on his face as if some microscopic shift in terrain were too much for him. There were tests and more tests, and when he received the diagnosis on Columbus Day, Oct. 13, 1993, he had an obvious question.

“Well,” he asked the doctor who had just told him he had amyotrophic lateral sclerosis, commonly known as Lou Gehrig’s disease, “what’s the treatment?”

There wasn’t any response, so he repeated the question, figuring he hadn’t been heard. But it didn’t take him long to figure out that the silence was his answer. There was no treatment, there was no cure, and as he soon learned, he would almost certainly be dead in three to five years.

Even Mr. Pendergast has no idea why he’s alive today.

Luck of the draw? His own manic, stubborn will? Divine intervention? Take your pick. But if sometimes sickness leads you into the light as well as into the dark, who could ever invent the journey that Mr. Pendergast has taken since he was given his prognosis?

Last week marked an anniversary forever etched in Yankees lore — May 2, 1939, when Gehrig, his strength ebbing by the day, took himself out of the lineup after 2,130 consecutive games. He announced his retirement on June 21, after receiving the diagnosis of A.L.S. He gave his speech proclaiming himself “the luckiest man on the face of the earth” on July 4. He died on June 2, 1941.

Mr. Pendergast will mark an unlikely anniversary of his own on Sunday. Eleven years ago he came up with an idea that horrified family and friends, who soon realized that Mr. Pendergast was so stubborn he was actually going to do it. He set out from Yankee Stadium on a circuitous, 350-mile, intricately mapped, 16-day route that would take his motorized wheelchair to Capitol Hill to call attention to the need for more A.L.S. research and immediate access to Medicare and Social Security benefits.

He did that ride for two more years. Since 2001 he’s made an annual wheelchair ride between Montauk and Manhattan. This year he plans to make the trip again, with perhaps 10 other people with A.L.S., who will do parts of the ride with him over the next seven days. Every year someone says that maybe it’s time to stop. Every year he says, no, it’s not. His foundation, Ride for Life, has raised $3 million for research and patient services.

Sitting in his wheelchair at Stony Brook University Medical Center, speaking through an amplified microphone, Mr. Pendergast has no illusions about the Darwinian worlds he’s competing in — one for personal survival, the other for attention in the bazaar of marches and runs and walks for one cure or another. Most of the efforts, he said, are organized and supported by the people who have survived a disease. But 90 percent of people with A.L.S. are gone in five years. Almost no one lives as long as he has. So, he figures, if there’s a divine logic to his ability to survive, maybe this is it.

“They say the squeaky wheel gets the grease, but since there’s no surviving constituency for A.L.S., there’s no squeaky wheel,” he says. “The patients die quickly and die in the most difficult environment, and the families just close the book and walk away. There’s no survivor to rally around, no celebrations, there’s just heartache. The disease lingers generation after generation, and every five years another poster disease comes along that gets the attention and massive research money, while this one keeps killing and killing, 6,000 people every year.”

He adds: “The answer isn’t to take money from your group. I don’t think different disease communities should be scrapping for pieces of the pie. I would like to have a bigger pie.”

Sometimes all the money in the world doesn’t buy a miracle. After all, before there was Chris Pendergast, there was Lou Gehrig, whose name turned A.L.S. from an unpronounceable medical oddity to a household name. And there is still no cure and precious little progress.

But sometimes miracles do happen, whether it’s his 15 years with the disease, those 11 years of improbable voyages, seeing his first grandson born a year ago, the way he’s transferred all his stubborn manic energy to this stubborn, manic cause.

“Thinking back to 1993, given the choice between being cured then or being where I am now, I don’t know,” he said. “I think I might choose where I am now, because of the joy and peace it has given me.”

The amplified voice was quiet for a moment.

“That being said, I’ll take the cure now.”

E-mail: peappl@nytimes.com Read more!

Lou Gehrig on Lou Gehrig's Disease

"The past few weeks you've been reading about a bad break. Today, I consider myself the luckiest man on the face of the earth. That I might have been given a bad break, but I've got an awful lot to live for. Thank you."

- Lou Gehrig, July 4, 1939

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Physical Therapy Time

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In The Arms of the Angel - Sarah MacLachlan and Josh Groban

[This is from last year.]

We were alone in the hospital room but he didn't acknowledge that I was there. A few moments later I looked up from my laptop and John was staring right at me, not that vacuous stare but what looked like a "Hey, what's up?" stare. I quickly put the laptop on the night table and went to his side.

"Hi there!" I wanted to asked him how he felt but I decided that would be a stupid question to ask of a man with severe ALS trying to recover from a heart attack who hasn't been able to talk in a month. I learned my lesson many months ago when I flippantly said "Get well soon!" He laughed at me then and said, "Get well? Really? No one with ALS gets well, Jimmy."

So I went on to say that I've been praying for his alertness and strength to return. I soon realized that he hadn't been looking at me at all. His eyes that I thought were looking right at me were still looking...where I had been, only they were more fixed now than before. And I wondered what he was looking at. Nothing? Something paranormal?

I remembered my grandfather saying that my late grandmother was standing at the end of his bed summoning him. My paternal grandfather, who never had a shred of Alzheimer's or other senility told us matter-of-factly, "She's asking me 'What are you waiting for?'"

I have no idea what my friend is looking at. But I do know that he is in some twilight zone between the realms of the known and the unknown. Science, with all its bells and whistles, can only look at a man in his state, physically recovering, yet mental capabilities unknowable, and say, "He's in the arms of the angel."

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Not More Physical Therapy!

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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?"

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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!