My Tribute to Brainhell

What if they discovered a disease and nobody wanted it?

Four years ago this month I discovered an erie blogspot that went by the name Brainhell. As I searched the net for ALS information I was curious yet skeptical of this writer who chose a black background with a dark image of the human brian.

He was 41, and diagnosed with the fatal and incurable disorder called ALS (amyotrophic lateral sclerosis, Lou Gehrig's disease). His blog was the story of that journey, from diagnosis, to searching for a cure, to acceptance. A husband and father of two that wrote every day about his life and interacted with the group who were his online family. Mostly I was a lurker that was entrenched with this daily dairy and the way he discribed life from his place. Who was this stranger in my computer?

This intelligent young man, a very good writer, made his readers smile along this painful jounrey. He held strong opinions and made them clear to everyone and no doubt they were well thought out. He knew fame was out of reach and there was not going to be any high drama with this disease. This guy wasn't trying to be sexy, and he understood that the general public would prefer not to look his way.

Reading along with this young man's journey always made me feel very humble, for he dealt with this terrible disease with grace and his strong spirit never wavered. He had not been able to speak for quite a while and communicated via computer or message board. Some of his recent writings displayed his weakness by way of character errors while using only one finger. His last post on his blog was a message of love to his wife and his two young children.

Hemingway had it right. "If the world is not the enemy, neither is it our friend. In the end, no matter who surrounds us, we travel alone. Our friends and loved ones are there, providing an infrastructure of love and support. But courage must be drawn from within. Let the world see us as we see ourselves and have the faith to permit us to do it our way."

Brainhell died 2/2/2008 at 8:40, at home, in peace. His wife at his side.
His final words; Like Cap. James Tiberius Kirk, he said, "It was fun."

Brainhell guarded his privacy and the privacy of his family zealously. This journey we had shared with him had come to an end. But he left as a parting gift for his readers a photo of himself and some last words. I've left those for your discovery.

http://brainhell.blogspot.com/

Lou Gehrig meet Elton John

Can the great Elton John teach us a lesson?

If you happened to miss the interview with music mogul, Elton John, this morning on CBS News Sunday Morning you missed an inspiring story. John, know for his long career as a named singer and musician, was also know for his outrageous clothing, glasses and his being very gay.

His life took a major change when he met a young American boy who had AIDS. HIV/AIDS was something that John found ubiquitous in his high living gay days, but never really something that he took to heart until his friendship with Ryan White. When Ryan died from complication of AIDS, Elton made this painful event a precipitant to make a huge change in life as he went into rehab, got sober, and got serious.

As John puts it, “I wanted redemption for the fact that during the '80s, as a gay man, I didn't do enough for HIV and AIDS." And when Elton John says "we have to help," people listen. Today his foundation, now based in New York and London, is considered among the most successful AIDS charities in the world.

"We're over $150 million in given-away grants and heading towards $200 million very quickly," John said.

Amazingly, he runs his foundation with a surprisingly small overhead, of only two in the US and 12 in the UK including volunteers. He says, “That way, you know where the money's going."

What catches my eye as a person living with Lou Gehrig’s disease is the power of celebrity in bringing awareness, funds and directed action to a serious terminal health problem. More so was the recognition that maximizing the funds going to patient care and research is done by minimizing overhead by which to best use the substantial amounts raised.

It seems that the ALS community could take a good lesson from the success that Elton John has brought and continues to bring to the HIV/AIDS community both in terms of the power of celebrity and the responsibility of small overhead.

If ever there was a crying need it is the careful and objective assessment of the structure that has evolved to provide resources for people living with ALS. Can the multiplicity of national organizations with each having paid staff possibly make the maximum use of the funds needed to research, awareness, education and patient care? One cannot truly discern the amount of money now being diverted from direct patient related affairs by competing and parallel overhead organizations.

When one looks as the annual reports of the two major national ALS organizations it is not apparent or transparent on what the true costs of strictly overhead functions. Most rely on generic statements like, “over 86% of each dollar goes to patient services and research.” What is never asked and it is hard to discern is how much more could be directed to patient services if there was some major consolidation of the management of these organizations.

As one whose years where asking such a question are limited, I am sure that I reflect the curiosity of many in the ALS community.

How do we get this careful and objective analysis, and how do we do a better job of accessing and allocating resources to support the people with ALS and their caregivers? I suppose if enough demand the analysis and consolidation, it may happen, but unfortunately we may not be around to see it or the holy grail of a “cure.”

It has to start sometime and that time is now. It has to start with you and you and you.

What Would Lou Gehrig Do?

I sense a basic systemic weakness in the entire national funding and resource allocation model or models now in place for amyotrophic lateral sclerosis, ALS, motor neuron disease or Lou Gehrig’s disease. For that matter the disease remains largely unknown amongst the general public.

Competition vs collaboration: The present parallel and competing organizational model engenders and promotes high overhead management structures that eat up resources that can be best diverted to patient services. In my opinion this has led to an unhealthy and spurious situation where defending the management structures has become a dominant underlying activity, rather than a continuing honest self assessment process.

Research for “The Cure”: When one looks at the overall research processes now in place and the lack of any substantial results for therapies to slow, stop or reverse progression it is clear that the present process has come up blank. Not even minor results have emerged and we are in a typical research loop of the only results being we need to do more research.

I sense that major organizations serving the ALS community have comfortably settled into a philosophy of promoting a cure for this horrible disease as the primary focus. When these organizations use a changing cast of patients in various stages of progression to unlock the wallets of the public, it makes me think of the flies on the poor little African children approach.

Cure an illusion?: A cure, particularly with the dearth of results or therapies, seems an illusion that preys on the hopelessness of PALS not dissimilar to the approach by more nefarious scammers. The reality is that unless the system is analyzed to determine why large amounts of resources are being poured into a research model that is not working. By not working, I mean that the basic premise that seems to permeate the whole research process, to use transgenic mouse models, to screen drug candidates and to uncover the disease process has now been questioned by a lab-wide study of results.

There may not be other means by which to attach the disease, causal, process and effect except some type of animal model. But it seems alarming to one whose life has been truncated, to learn that this major weakness was not appreciated before now. And that significant amount of research resources has been directed to this questionable animal model without any appreciation of the inherent weakness.

Shotgun or high caliber gun? The weakness, it would appear to this neophyte, is made all the more serious with the seriously fragmented distribution and practices by the myriad of research organizations. I might add, research organizations vying for an essentially finite funding source where we have come to find a shotgun approach rather than a cannon has come to be the acceptable approach.

Deep assessment needed?: As was stated at the outset, it seems to do the ALS community true justice, it is time for the leadership now directing the allocation and direction of resources into patient services, education, awareness, advocacy and research to step back and look deeply and honestly at the present mode of operation.

Can we accept or should we, as a community, tolerate the duplicative high resource sucking overhead structures when and if the patient is truly at the forefront of their philosophies? It seems that we cannot or should not accept the present structure if it is not serving us as efficiently as possible or it is truly not keeping patient welfare at the core of its vision.

This is not to infer there are not substantial patient services and care programs now in place on a local or regional basis. But in a finite resource situation where competition can burn up valuable allocation and utilization of the resources, we should demand a systemic review and analysis of the processes and models now in place, fragmented as they may be.

Change good or bad?: The reaction to suggesting that all is not well in the halls of higher management in the ALS community has been defensive. In fact, it has been my experience that questioning the mode of operations is considered treasonous and to be isolated rather than embraced.

In unity, strength?: How does one inspire change, real honest introspective change? It would seem that only when the community at the center of the structure speaks with one voice, can change be a possibility. Because of the transient nature of the ALS constituency, combined with the individual challenges faced by each patient, there has not been an effective coalescing of the constituents. The result is a system that feeds on itself, rather than striving for innovative and a continuing self analysis to assure the optimum allocation and use of resources.

When one looks at the top management structures of these organizations in the form of the boards of directors, and when one appreciates the close connection to ALS by many of them, and their expertise, it is difficult to correlate the present system with the crying need for self assessment and improvement.

What would Lou Gehrig do?

Another NFL Player

http://www.carrollcountytimes.com/articles/2008/02/22/sports/fsports022208.txt

OWINGS MILLS -- Baltimore Ravens director of player development O.J. Brigance is battling Lou Gehrig's disease, and will be honored with the Johnny Unitas Tops in Courage award March 11 at the annual Ed Block banquet in Baltimore. Brigance has maintained his work schedule at the Ravens' offices while dealing with his illness, a neurological disorder also called amyotrophic lateral sclerosis that damages motor neurons in the brain and spinal cord.He was a unanimous selection by the Ravens for the award as a tribute for his bravery and positive attitude in dealing with his illness since it was discovered last year.Brigance is a former Ravens linebacker and special-teams ace who won a Super Bowl with Baltimore following the 2000 season.

_____

One would think that the NFL Players' Association might be interested to see if they're getting more than their share of ALS!

http://alsspreadtheword.blogspot.com/2008/02/superbowl-sunday-food-for-thought.html

Twisted Fate

In 1938, Franklin D. Roosevelt was in the White House and the seldom photographed president was leading the country from a wheelchair. The March of Dimes he helped create was an effort that began with a radio appeal, asking everyone in the nation to contribute a dime to fight polio. In only a few days his plea had received $230,000. Roosevelt served four terms as president, led the country through some of its greatest crises and doing so while disabled.

Lou Gehrig had taken himself out of the lineup, putting the Yankees first and his failing limbs on the bench. Just before Lou Gehrig's farewell speech, Dr. Habein's of the Mayo Clinic thought that the New York fans needed a "Press Release" to explain the sluggers diagnoses. In an attempt to ease the pain of their loss, the release told Yankee fans that the Iron Horse had Infantile Paralysis better known as Polio. Lou and Eleanor knew the truth about ALS (amyotrophic lateral sclerosis) but went along with the story that gave the Yankee legend this hopeless fifty-fifty chance. When the Mayo Clinic decided to hide the real horrors of ALS, it may have been the set-back we deal with yet today. As Lou Gehrig's body failed and became more and more paralyzed he would keep the lie alive by acting out this fabrication of hope.

All those dimes were too late for Lou Gehrig and after his death the tragedy of ALS was explained to the public, at least to those who were still listening. The ALS connection was made and because of his death, Lou Gehrig became a legend for a whole different reason.

Polio was eradicated with a vaccine because a country rallied and gave dimes to aid in the research. FDR is still the face on a Dime while Lou Gehrig's disease sadly remains the same.

Lights, Cameras, Congressional Action!

This week the House Oversight Committee was the focus of a huge media circus. The he-said-he-said bickering of Roger Clemens and his ex-trainer became a matter of Congressional scrutiny. This is understandable since there are so many young people who emulate those our society reveres on the pedestals of sports. Put a ripped sports star and some tabloid material in front of a committee with lots of lights and cameras, and we have an interested Congress!

A few months ago some not-so-famous people interested in a couple of other Yankees tried to get the ears of some of these same Congresspeople. Their Yankees were Lou Gehrig and Catfish Hunter – two talents who tower over Roger Clemens in many ways yet met cruel demises through no fault of their own. The not-so-famous people who asked for some time with their Congresspeople included many with ALS – Lou Gehrig’s Disease. They included the widows and widowers and orphans left behind by this disease. They weren’t as famous and buff as Roger Clemens in his expensive suit. Many were in wheelchairs and needed great deals of assistance just to get in and out of the doors in the Congressional office buildings. Some didn’t get the warm reception that Roger got the other day, and certainly the media paparazzi weren't capturing their case.

Some Congresspeople listened and some didn’t.

There are tools at http://capwiz.com/alsa/home/ to see how your Congresspeople and Senators listen to public health issues involving our two Yankees. If only they were as engaged in Lou Gehrig’s Disease as they are in what kinds of shots went into Roger Clemens’ buttocks.

The March

It's been a little over the past three years since I officially joined the ranks of the PALS. (That stands for People with ALS.) It has been a revealing journey, that's for sure. The steady drum beat of those that drop off the active list gets to one's deepest soul. My world has been expanded by the internet to include an increasing family of PALS. We share our fears, some of the hard lessons learned, the frustration with the decades without a single drug to significantly extend life or slow progression. Above all we come to share the march of those losing the battle.
I suppose it must be similar for many disease communities, but, for me being a PALS has been tough. My courage, tolerance, stamina have not been my long suits. Not a good mix of deficiencies when faced with not only your own terminal illness but that of so many fine people that have come into my life, my fellow PALS, their families, and the wonderful dedicated people working with the local ALS Association and MDA organizations.

I have lost many new friends, PALS who have the courage, tolerance and stamina that I admire and is missing in my own life. This week I lost a friend who I never met. I read his marvelous narratives of living with ALS. His skill with words and expressions were stand outs, ALS or whatever he put pen to paper or finger to keyboard to memorialize. He had another skill and tool that was so powerful, that of being a professional videographer. He used this avenue to share his journey and his philosophy of his life with ALS. He gave a face of this disease to the thousands of readers of his newspaper columns and viewers of his videos. In both cases he opened his deepest thoughts and shared them with the public and those of us in his boat with this disease, where his words reflected our own thoughts and fears.

I will miss Leo Greene for his contributions to all of us. He was no saint, his son put that in context in his comment on Leo's last article, but few of us are. He did have the courage and rare talent to give a life, blood and guts to ALS that I did not find but in very rare instances. Leo and I had a great dialog via email and his last response, a long one, a bright one, was dated the day of his death.

I hate this march, the continuous loss of friends, friends who have far more to contribute than many. So, I say my little personal thanks to this talented man and I wish him nothing but peace on the journey that he now is on.

Count Von Count, Help!

The internet is amazing. Blogger The McKay Chronicles popped up in a Google alert...

Even as I write this, it's hard to believe that it's been over two months since Martha died. You know, before she got sick, I knew virtually nothing about Amyotrophic Lateral Sclerosis. I did not know anyone who had the disease. The only thing I knew about it was that it was more commonly known as Lou Gehrig's disease, and it was ultimately fatal. Now, we've lost Martha, we have heard of five or six other people in Pulaski county with ALS, and I know of another man who was just diagnosed with the disease yesterday, also in Pulaski county. That's a little bizarre. It certainly makes you think it might have some environmental influences or causes. Anyway, not to put a macabre spin on this little posting, but it popped into my head, and since I'm doing the typing, it made it to the Internet for perpituity.

Later Google found this from ALScounts

Pulaski County is about midway between Chicago and Indianapolis. The old railroad tracks run through its small towns and it's highly agricultural. Roughly 14,000 people live in the county. That's only about .0000467 of the U.S. population of around 300,000,000. If we suppose that the 30,000 Americans with ALS premise is correct, Pulaski County's share of PALS is only 1.4 at any moment in time. If there are 3 PALS in Pulaski County right now, that is over double what one might expect. If there are 6 PALS in Pulaski County right now, that seems astronomical.Of course, if we had a good registry of patients, the numbers could be verified and our health officials could stay on top of any real or perceived clusters. In the meantime The McKay Chronicles did us all a favor by capturing an observation on the internet.

It seems obvious to me that a simple headcount of all cases of ALS would help verify or dispel perceived clusters. In the meantime, please use the power of the internet to spread the word.

Maybe it ain't all bad news...

This blog from Candy Minx came to my attention today and it is illuminating for all sorts of reasons. One, it surely pours some cold water all over the pointy heads of those in the US who pooh pooh universal health care and only spout a litany of evil. Two, it might even cause a few of us to consider that you don't have to live down here to be at the head of line when it comes to science and medicine.

Our friends in Canada where universal health care is a way of life, and has been for a long time, have accumulated a rather impressive lists of accomplishments from this nefarious system.

Maybe the Canadians need to take a page from our illustrious system and have their own FDA so their terminally ill folks can be protected from experimental drugs or kept from trials with flippant exclusive criteria. Some of us with ALS feel that we are protected to death.

Do I recall that the recent China lead toy import onslaught affected those to the north?

Heck if there was was CDA, maybe they could ban pharmaceuticals from the US and thereby keep their prices floating as high as ours. I mean why should we be the only ones where the choice between being able to purchase meds or eating is de rigeur?

Oh, let me pry my tongue from being firmly stuck in my cheek. Actually, these days my tongue doesn't do much that I tell it to. I am told it may be ALS. Could that be?

Just in case the link to Candy's Agnostic Minx blog didn't show, here it is:

http://gnosticminx.blogspot.com/2008/02/i-wonder-why-there-conspiracy-theories.html

Breaking News!

Breaking News! That TV tactic that plays to our fears and has spread now to the science field, playing on our ALS hopes. In the early 90's we had breaking news of a drug called Myotrophin. This new hope would turn victim to European clinical trials and the FDA freezing their lottery program. Leon Charash, head of the MDA's medical advisory committee, said the Myotrophin injections produced a skin inflammation that might alert patients and bias the trial.

The latest is the Lithium study coming from Italy and is laying claim that it slows ALS progression. To date one Internet forum has had over 300 comments on the subject, every possible angle being examined by people coping with ALS.

Mixing Lithium with Rilutek? One claim indicates that this combination can increase the effectiveness of Rilutek. Could this confirm that Rilutek has never lived up to being the only FDA approved drug slowing progression? Lithium salts seems to be very exciting but what are the guide lines right now? How do you take it, when, how much and what are the side effects. The current results are interesting but it has to go further to larger trials and that equals more costly time.

Breaking News, this study has been reported to the National Academy of Sciences. The NAS was signed into being by President Lincoln on March 3, 1863. After years of modifications it is now known as the National Academies. Take a look at this site and you will understand that Lithium may be the next needle lost in the hay.

Patients coping with ALS deserve a lot more than just Breaking News. ALS needs one research group expediting this type of discovery and never let it rest.


http://alslithium.atspace.com/

Where Do The Fines Go?

Last week I read that a Superbowl participant was fined $10,000 for wearing an unauthorized hat at media day. That reminded me that Bill Belichick dropped a ton of money in spygate fines a few months ago. Indiana Pacers have been huge contributors to the NBA's fine fund recently. NASCAR finally decided that their huge fines fund would go to charities as is the custom with other professional sports.

Does anybody know where these huge amounts of money really go?

Does anybody know how to get the fight against ALS in line for some of these charitable windfalls?

ALS has been ironically cruel to so many sports figures, it seems like a fitting use for this money. We always read about the $10,000 knuckle rap given to a wealthy athlete or a $500,000 knuckle rap given to a sports franchise, but do we ever actually learn which charities get the money?

Super Tuesday

For those who might be in a position to schmooze with some candidates today, it might be a good chance to ask them (preferably with the national network cameras running) about their positions on the ALS Registry Act S 1382.

Here's the current cosponsorship status of those left in the running for President -

Clinton - Yes
McCain - No
Obama - No

Cosponsors and Non-Cosponsors from All States

FDA - Crisis or Research Drought?

There were some Congressional hearings last week regarding the state of the FDA.

The New York Times calls the FDA an agency in crisis, lacking funds and talent.
http://www.nytimes.com/2008/02/03/opinion/03sun1.html?em&ex=1202274000&en=a7ef53c3b05c12ac&ei=5070

USAToday gives us the perspective that the lack of new therapies being approved by the FDA is caused by a drought in drug research.
http://www.usatoday.com/money/industries/health/2008-02-03-fda-drugs_N.htm?loc=interstitialskip

This is reminiscent of the Great Taste vs. Less Filling ads that we used to see on Superbowl Sunday. Both answers are right. It's simply a matter of perspective when it comes to beer. When it comes to ALS, it's simply a matter of life and death.

A person with amyotrophic lateral sclerosis has no effective therapies... even therapies that are risky long-shots but might cure or stop the disease. Nothing. Nada. Zip. Zero. Nil. Zilch. Talk about outrageous!

Eleanor Gehrig used to pick greens in Central Park to supplement Lou's diet because there was a theory in 1940 that vitamin E may help ALS. Today people with ALS often take vitamin E supplements because they hear a 2008 theory on the internet that vitamin E may help ALS.

Surely the FDA and our pharmaceutical corporations can deliver something better than vitamin E theories after all these years. Please. It's not just the other guy's problem.

Rubik's ALS Puzzle

Headline:
An experimental drug may help some patients with ALS - live longer.

Of course the keyword here is, 'May'. Scientists have found Sod1, Rac1, Nox2, Ros and who knows what else. So it goes on and on, little four letter parts of a puzzle that are not complete.

Twelve years later another set back; (Rilutek FDA approved 1995)
The research failed to replicate several studies in the SOD1 mouse model that have led to clinical trials of drugs that showed promise for treating ALS. Their results showed the compounds minocycline, creatine, ritonavir, celecoxib, sodium phenyl butyrate, ceftriaxone, WHI-P131, thalidomide, and rilutek had no survival benefit at their reported routes and doses. The therapeutic effect of the FDA-approved drug rilutek is known to be marginal, providing on average only two months extended survival in ALS patients. Source, Rehab

Dea Ja Vue. That revolving door of ALS patients caught between Life and Death. ALS is more complicated than a Rubik’s cube which is many sided, with multiple connections and various colors. It appears that maybe color blind researchers are toying with the cube all alone. Yes, it has four side, yes, it's not easy but are you going to tell us, it can't be won. Of course there are the causes, therapies, bio markers, genomics, existing drugs, patient needs, palliative care as well as all avenues of research. Each day someone releases a new hunch or a press release that reads, we continue to be confused by all these colors. Who is in charge of ALS research today? No one! Who oversees and is accountable for existing medical research activities for ALS? No one! What is the strategy for solving this Rubik’s ALS Puzzle? To many strategic plans and competing to lay claim that their theory meets approval for further discussion and/or funding!

May I suggest the NIH and any research group applying for grant money call on the one with a proven theory. Lars Petrus became Rubik's speed champion at age 22. At age 47, Lars holds the speed record at 13.60 seconds and is now employed by Google. What could Google possibly see in a puzzle champion? Think about that!

ALS & Military, Job-Related Difficulty?

This morning I read in several newspapers about the horrible increasing suicide rate among our military.
From the AP story --


"We have been perturbed by the rise despite all of our efforts," said Col.Elspeth Ritchie, psychiatry consultant to the Army surgeon general. ... Ritchiesaid Thursday, as she did last year, that officials are finding that failedpersonal relationships are the main motive for the suicides, followed by legaland financial problems as well as job-related difficulties."I also have a daily Google news search for the word amyotrophic. Google usually finds a couple of fundraisers, a little research news, and a lot of obituaries. Today it found, of all things, a Pravda story on the rise in U.S. military suicides.

From Pravda-English ;


"Most likely, soldiers commit suicide over their inability to cope with symptomsof the so-called Gulf War Syndrome. ... Brain cancer deaths, amyotrophic lateralsclerosis (commonly known as Lou Gehrig's disease) and fibromyalgia are nowrecognized by the Defense and Veterans Affairs departments as potentiallyconnected to service during the Gulf War."



I'm not about to rely on Pravda as my most trusted news source, but it sure makes one wonder why the ALS connection to Gulf War service isn't mentioned in the American story. Perhaps it's just assumed to be included under "job-related difficulties."

Doctors, Might Be ALS Even If They're Old...

This week the U.S. Department of Health and Human Services announced the appointment of Dale Bredesen, M.D., to the National Advisory Council on Aging. Dr. Bredesen is not only an expert in issues of aging, but he also has done considerable research on amyotrophic lateral sclerosis. His dual expertise is a welcome addition in a group that can influence healthcare for the elderly.

Elderly patients with ALS are often misdiagnosed as having the more obvious maladies of aging such as strokes and arthritis. If the ALS diagnosis is made late in the game, the patient has one foot in the grave before learning about ways to help deal with the difficult, short path ahead. If the ALS diagnosis is missed completely, the patient often dies without ever knowing what the problem really was. How many families have buried Grandpa who never got better from his "stroke" or have had a funeral for Grandma who "just wouldn't eat because she kept choking on her food?" How many elderly deaths have attributed to something other than the real culprit -- ALS? How many doctors examine their elderly patients closely enough to recognize ALS when they see it?

ALS occurs in the elderly. It's not just a disease for Lou Gehrig-aged people. In fact, mortality data show that that more females than males die from ALS in the older population. Talk about a crummy glass ceiling to break!

Superbowl Sunday Food For Thought

There has been a lot of coverage amidst the Superbowl media circus about the retired NFL players who face the crippling side-effects of their careers. They are trying to shine some light on their problem since the NFL and the NFLPA have not.

There have been so many athletes after Lou Gehrig who have faced ALS, including many football players. A fairly young NFL star of the Seahawks, Glenn Montgomery, died from it. Wally Hilgenberg of the Minnesota Vikings has it now. Notre Dame All-American and Bronco All-Pro Pete Duranko is dealing with ALS, too. CFL star Tony Proudfoot was recently diagnosed.

There are theories about the triggers. Head trauma? Stress? Turf chemicals?

What if NFL players' ALS is job-related? Would that not add some significant fuel to the retired players' fire?


MSNBC 2/3/08