A Vow is a Vow, Part One

"In sickness and in health, I vow never to leave your side."

Johnny and I repeated those words to each other on Sunday afternoon, September 9th, 2007. Enjoying the moment with us were Pastor Joel, my son, Ryan, and his wife, Lisa, my daughter, Shannon, and Lisa's parents. It was a simple, yet wondrous wedding known as a Trinity ceremony. John accepted Shannon, Ryan, and myself as his own. We vowed to always be there for each other. It was a day I will never forget.

I have loved every minute of the last eleven years with Johnny. We have had great times and great trials. In February, 2005, Johnny was diagnosed with Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease - and that has been the greatest trial of all.

At first hearing the diagnosis, we were in shock. The day after that we cried, and the third day we were asking ourselves why this was happening. Johnny has always been an optimist and, by the third week, he said, "Hell, I'm 64 years old. This could have happened to me in my 30s or 40s. I've lived a good life and I am NOT going to stop living my life to the fullest!"

Over the next several months and years, he signed up for every trial and study in our area for ALS victims. It first attacked his upper body; his shoulder muscles and arms. In the first year and a half after the diagnosis it progressed slowly, but Johnny weakened dramatically in 2007. Since he has been in home care nearly all of this year, he has remained stable.

For Christmas, 2006, we planned a family reunion for Johnny and the Miller family in Hilton Head, SC. There Johnny found out that his family was proposing he move from Fort Lauderdale to Fernandina Beach, Florida into a nursing home. Johnny's response was clear, "No way am I leaving my home!" From then, we began to seriously discuss marriage and our future.

From February to April, 2007, Johnny was part of a hyperbaric oxygen treatment research study in Miami. We found out in June that his deterioration from ALS had accelerated; the experiment did nothing for him. By this time, Johnny was not able to swallow his food, the muscles of his tongue and throat having succumbed to the disease. Johnny dropped from his normal weight of 160 - 170 pounds to 114 pounds, so he ordered a feeding peg in June. He also signed up to receive an LTV900 ventilator, as his breathing had become terribly labored; the muscles around his lungs were losing their strength.

Johnny gained back all of his weight by November of last year thanks to the feeding peg, returning to his pre-ALS weight of 160 - 170 pounds. He was happy that at least one aspect of his ALS had been turned back.

To be concluded in Part 2... Read more!

Ryan, Johnny and Zeus on Father's Day, 2008

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There is Life After Vent.......

Friday, July 27, 2007

THE VENT LIFE

Who Says You're Out of the Game?


You'll find the hardware here also, but the heart of this cover story is lifestyle. To too many uninformed observers, the vent life is a contradiction in terms -- how can you have a decent life when you're plugged into the local utility? Here's rich evidence that life doesn't end after mechanical ventilation from the people who know best -- readers who use vents. They put to rest the vexatious quality-of-life issue, and prove that a vent is just a machine, not an enemy.


The Vent Life by Barry Corbet >See also Bully Pulpit >The Quality-of-Life Issue >RFs Are All Around You and Guess Where They're Showing Up? by Jerry Daniel New Mobility Talks to Vent Users by Barry Corbet Mission Possible? Converting to Noninvasive Ventilation by Jean Dobbs >Keeping the Trach >Manufacturers >Resources




THE VENT LIFE


By Barry Corbet


Once again, we look to the experts -- in this case, to people who use ventilators. From what they have told us about the vent life, we can pass on some generalizations.


The biggest single problem vent users face is the outrageous cost of their equipment and care. Many who use invasive ventilation need skilled nursing, and paying for 24 hours a day of that can be a nightmare. A few have trained friends, family and unskilled help so well they feel comfortable with a minimum of nursing care.


The care level may not be a matter of choice. Many states mandate skilled nursing for vent users. That annoys those who feel they can get by with cheaper help, and alarms those who fear their essential nursing will be cut by the current enthusiasm for cost containment.


At any level of care, by the time you throw in a couple of $10,000 vents and a power wheelchair -- and some way to transport them -- you've run up a rather astonishing bill. Consequently, most vent users must rely on Medicare, Medicaid and entitlements. An admirable few can make enough to pay their own medical expenses, but it's a fiercely heroic life they lead.


Can you learn to love your vent? Sure, said most -- it's the breath of life, and it becomes part of you. Others curse their vent as a noisy, cranky umbilical cord to a wall socket. Most gripes are not about the machine itself, but about dependency, the perception of being a burden and lack of privacy. What's the hardest thing about using a vent? It's unanimous: when it doesn't work.


Ventilators are dependable and failures are rare. Yet almost everyone we talked to has a couple of horror stories. The lines of defense, successively, are "frog-breathing" for those who can do it, a spare vent, the Ambu-Bag and calling 911. Some admit to a lingering uneasiness about it.


Our experts -- even those who have spent several decades on a vent -- are generally in good health. The most common complaint is needing more time on the respirator. Most feel they've learned enough over the years, and are good enough at reading their own bodies, that they can tell when they -- or the vent -- have a problem.


The MDA comes in for some criticism, this time for its lack of support for mechanical ventilation for people with Duchenne muscular dystrophy. Why, vent users ask, does the MDA feel their quality of life is so low that ventilation is a waste?


That's a point everyone agrees on -- quality of life should be defined by the person living that life, not somebody else. Not anybody else.


The Quality of Life Issue


Is a reasonable quality of life possible if you use a ventilator?


Many doctors and allied health professionals don't think so. The most common reason that MDA clinic directors cite for discouraging ventilator use is poor projected quality of life. But the health professionals' low opinion of the vent life is not shared by the people living those lives.


If your doctor, nurse or even your family and friends are telling you that your life will be terrible if you go on a ventilator, hand them a copy of these studies. And tell them to go fish.


A 1992 life satisfaction study by John Bach compared responses by 80 Duchenne muscular dystrophy ventilator users to responses by 273 MDA clinic directors. The clinic directors significantly underestimated the users' reported life satisfaction. The study concluded that patients who were perceived by physicians to have a poor quality of life were less likely to be offered assisted ventilation. In a 1992 study by Bach and Campagnolo of 395 ventilator-assisted people who'd had polio, 86 percent reported their lives were characterized by hope, value, freedom and happiness. Forty-two percent of the ventilator users were employed and 39 percent were married. Yet again, the health professionals used for controls underestimated the life satisfaction of people using ventilators and overestimated the difficulties. It was concluded that physicians should be aware of their inability to accurately gauge the life satisfaction of individuals and should not use their perception of poor quality of life as a reason to deny ventilator assistance. In a 1994 life satisfaction study by Bach and Tilton, 42 ventilator-assisted quads and 45 autonomously breathing quads were compared to a control group of health care professionals. Both groups with spinal cord injury reported higher life satisfaction ratings than were predicted by the controls. Notably, the ratings for those who used ventilators were higher in some areas than for those who didn't -- the former were dissatisfied with sexual function only, whereas the latter were dissatisfied with their jobs, health and sexual functioning. Vent users said life was friendlier, more interesting, more enjoyable, fuller and more hopeful than non-vent users. A 1992 study by Whiteneck, Charlifue and Frankel of people with spinal cord injury at least 20 years post-injury showed that those using ventilators rated their quality of life higher than those who didn't, and had a lower rate of suicide ideation. -- Barry Corbet


The above information was taken from "Independent Living and Quality of Life Among Persons Who Use Ventilators," an in-press book chapter by Margaret A. Nosek, Ph.D., and S. Ann Holmes, M.D. Both authors are ventilator users themselves.

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Invest in ALS?

"The ALS market was worth 148m in 2006" according to an investment paper.

The ALS market was worth $148m in 2006 across the seven major markets. With the only approved treatment for ALS, Sanofi-Aventis''s Rilutek (riluzole), facing generic incursion in 2012-13, future growth in this market is dependent on the success of pipeline drugs.

Scope

Scenario based analysis and forecasts of the commercial potential of the ALS market to 2016.
Analysis of
patient potential, marketing factors, commercial attractiveness and clinical attractiveness of key late-stage pipeline drugs.
Strategic recommendations for maximizing the potential of drugs in this niche orphan disease market.
Indication-specific sales forecasts of marketed and key pipeline drugs to 2016.

Highlights

A lack of new entrants, and the possibility of polytherapy, results in Sanofi-Aventis''s Rilutek (riluzole) dominating the market until patent expiry in 2012-13 in Datamonitor''s base scenario.

Orphan drug status, rapid uptake following launch, limited risk to the company''s share price if drugs fail, and possible indication expansions make long-term investment in ALS a commercially viable option.

A scenario forecast based on the clinical success of rhIGF-1 (recombinant human insulin-like growth factor I) related therapies reveals a potentially lucrative market. However, with a high attrition rate of ALS Phase III clinical trials, and mixed efficacy data in Phase II, the results of currently underway studies will be pivotal.

Reasons to Purchase

Access the lucrative potential of this underdeveloped neurodegenerative disease therapeutic market.
Benchmark key late-stage compounds against the current leading therapy Sanofi-Aventis''s Rilutek (riluzole).
Understand the opportunities in this market based on the comments of interviewed key opinion leaders.

www.companiesandmarkets.com/Summary-Market-Report/Pipeline-Insig ..

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So Much, So Fast - New Documentary on ALS

Click on the Read More! script to see the trailer and the synopsis of the film.

So Much So Fast is about the remarkable
events set in motion when Stephen
Heywood discovers he has ALS (Lou
Gehrig's disease) and his brother Jamie
becomes obsessed with finding a cure.

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Johnny's Photo Gallery - May, 2008

[Click on the Read More link to see the rest of the photos. that's Lester the famous nurse extraordinaire!]





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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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