May Was ALS Awareness Month - What Were The Outcomes?

May was ALS Awareness Month.

Again this year it featured daily vignettes of families dealing with ALS on the MDA ALS site. This year it featured a flatteringly similar campaign of vignettes of families dealing with ALS on the ALSA site.

We saw almost a thousand advocates converge (some enduring huge amounts of difficulty to get there) on Washington, DC, to talk to legislators about old and new legislative priorities related to amyotrophic lateral sclerosis and the wicked wake it leaves, especially for veterans.

What were the outcomes? What were the measurables? What value did ALS Awareness Month add?

Google gives us an interesting and inexpensive insight into news measures. Google "amyotrophic" and look at news items in May. There are a few items related to the vignettes. There are more items related to local human interest stories. There are more than enough obituaries. There are fundraisers. There are a few press releases from the bio-pharm industry with "promising" science which might impress investors.

Try the same exercise to look at the Google news archive from any other month.

Did ALS Awareness Month give us any increase in the quality or quantity of news related to ALS awareness?

Top 10 Worst Diseases?

The ALS Registry Act is needed to build on projects underway in three test cities and is supported even by this Administration. This act would help determine the causes of ALS, and more importantly help to find an effective treatment for this disease. We need this registry now! After ten years we still think that at any given time 30,000, in this country, are living with ALS. That is equal to the same old statistics that 5,000 will die annually. I would like to defend ALS but where do we draw the data from? How long are we going to allow our awareness efforts to be overshadowed by outdated recording methods? ALS is complicated as well as intense, 2-5 years life expectancies make it all the more important to monitor diagnoses as well as labeling the correct cause of death.
Millions of people each year have perished as a result of one of any number of seemingly unstoppable diseases. Here are but ten, in no particular order, that have wiped out mankind since the earliest recordings. The list, without ALS was found at "The List Universe.com".

1. Polio 10,000 Deaths since 1916
2. Influenza 36,000 Deaths per year
3. Spanish Flu Between 1918-19: 50-100 Million dead
4. Bubonic Plague 250 Million Europeans Dead (1/3 population)
5. Malaria 2.7 Million Deaths per year-2800 children per day
6. Ebola 160,000 Deaths since 2000
7. Cholera 12,000 Deaths since 1991
8. Smallpox Native Americans suffer a population drop from 12 Mil. to 235,000
9. AIDS 25 Million since 1981
10. The Black Death 75 million Deaths

"Democracies Don't Let People Die"

There is an op-ed in this morning's Wall Street Journal by Daniel Henninger entitled, "Democracies Don't Let People Die."

It speaks of what is happening (or more correctly what isn't happening) in Myanmar and China.

When I first read the title of the piece, I thought it might be about ALS in America.

Senator Tom Coburn Please Come

Senator Tom Coburn has successfully blocked the ALS Registry Legislation that passed the House by an overwhelming margin and enjoys rare bipartisan co-sponsorship by 74 of his Senate colleagues. It is hard to believe that one individual can hold this legislation hostage but it is true. It sure should scare all citizens that this can be so.

Over 500 ALS patients will attend the ALS Association National Advocacy and Public Policy Conference in Washington, D.C., next week where most of them will visit their Congressional legislations. While in Washington all of the conference attendees will band together in a candle light ceremony to remember the 5600 that died from ALS since the conference only a year ago.

Actually since there is no credible documentation of how many have ALS or die each year, the 5600 is a guesstimate that apparently has remained unchanged and unchallenged for the past decade. That is the whole purpose of the legislation, to build a credible database by which to evaluate the true prevalence of the disease and provide data that can direct research to help find the cause and a cure.

Why is a registry needed asks the good Senator Coburn? Without reliable data it is likely that direction of research and allocation of research funds will likely allow the absence of a diagnostic test, ignorance of the cause, and the failure to find meaningful therapies, much less a cure, will continue to be the status quo.

Somehow that status quo speaks ill of the will of a nation that can put astronauts in space on a regular and frequent basis. Or a nation who's pharmaceutical companies can spend billions on advertising rather than the research needed to address "orphan" diseases like ALS.

Shown above is a notice that has been posted by one of those who lost a loved one to this disease for which there are no effective therapies and no cure. It is a disease that ends up with a mind that is left intact in a body that is completely paralyzed. Maybe Senator Coburn missed these sessions in his purported medical training. No matter, because he is being invited to attend this commemorative candle light ceremony at the World War II Memorial on the Capitol Mall.

I wonder if he will have the courage to face those suffering from the disease and the many more who have lost loved ones. If he doesn't maybe some of these brave souls will hold a ceremony in front of his office in the Senate Office Building.

I think it is ironic knowing the Senator's one man log jam to view the warm image shown on his website.

ALS VA Registry Closed, Court Dismissed

The Department of Veterans Affairs (VA) Cooperative Studies Program was established to create a National Registry of Veterans with Amyotrophic Lateral Sclerosis (ALS). United States military veterans with ALS were identified through national VA electronic medical record databases and nationwide publicity efforts for an enrollment period of 4 1/2 years. Diagnoses were confirmed by medical record reviews. Registrants were asked to participate in a DNA bank. Follow-up telephone interviews are conducted every 6 months to track participants' health status. As of September 30, 2007, 2,400 veterans had consented to participate in the registry, 2,068 were included after medical record review, 995 were still living and actively participating, and 1,573 consented to participate in the DNA bank. 979 participants had been enrolled in the registry for at least 1 year, 497 for at least 2 years, and 205 for at least 3 years.

Fourteen studies were approved to use registry data for epidemiological, observational, and interventional protocols. This registry proved to be a successful model for identifying large numbers of patients with a relatively rare disease and enrolling them into multiple studies, including genetic protocols.
The VA concluded that if you served in the Southwest Asia Theater of Operations (Iraq, Kuwait, Saudi Arabia, the neutral zone between Iraq and Saudi Arabia, Bahrain, Qatar, the United Arab Emirates, Oman, the Gulf of Aden, the Gulf of Oman, the Persian Gulf, the Arabian Sea, the Red Sea, and the airspace above these locations) between August 2, 1990 and July 31, 1991 then ALS is presumed service connected. This means that those that apply for benefits are service connected automatically.

Just when the ALS data starts to really focus on the occurrences, would'nt you know it, the project runs out of funding and the enrollment was halted. The military areas of service where established and a set of new rules where ordained. This ALS VA Registry rule book was an opened and closed case.

What did the ALS VA Registry accomplish? Was it a genuine concern for the causes of ALS, basic benefits defined or to avoid possible future lawsuits? Was the VA aware of a similar study conducted by British researchers that reported ALS also occured in their soldiers who served thousands of miles away and that some never even saw battle? There were nearly 120,000 British and American soldiers participating in the 1990-1991 year of the first Gulf War. After the war these servicemen had two relevant nervous system disorders, the Gulf War syndrome and ALS (amyotrophic lateral sclerosis). This study published in the Nov. 26, 2002 edition of Neurology, found that like their American counterparts, many of the 50,000 British soldiers who served in operations Desert Storm and Desert Shield report a higher rate of distinct symptoms that occurred within months of their return from active duty.

http://www.citlink.net/~bhima/gulfwarsyndrom.htm

How naive of us to think the VA Registry was going to operate indefinitely but wait, we're still at war. In fact, this is the fifth year of the Iraq War. In fact, it is the 1,828th day since Mission Accomplished.
March 2007, Tom Mikolajcik, a retired Air Force general and former commander of the Charleston Air Force Base, testified before the House Committee on Veteran's Affairs. Mikolajcik was diagnosed with Lou Gehrig's disease five years ago.

He said, "We are currently exposing 100’s of thousands more service members to the elevated risk of this disease. There will be young men, women, and families celebrating a return from Iraq and Afghanistan alive, who have no idea that they may soon be facing a certain death from ALS. We will have to answer those families when they ask what the government has been doing to prepare for this onslaught. For this reason, the government is compelled to assume leadership of this issue."

"If these soldiers were dying in the field …. rather than quietly at home as a consequence of their service, we would leave no stone unturned. We would use the best existing resources and programs to make sure they had whatever they needed to survive….to ensure that no man or woman is left behind."
Recently, Dr. James Peake, secretary of Veterans Affairs, visited Charleston S.C. to meet with Tom Mikolajcik. The retired general urged Peake to grant all veterans with ALS a service-connected disability. Currently only Gulf War veterans are covered, Mikolajcik said. "My comrades in arms don't get the same benefits, and it's not fair," he said. For unknown reasons, veterans have a 60 percent higher chance of developing ALS. The retired general described the meeting as "very personable." In closing, he said "Now we just wait and pray."

These Aren't Just Any Football Cards

The ALS play clock ticks relentlessly for Hilgenberg, Duranko, Proudfoot, Brigance.

Lord, What's An Earmark?

Remember the old Bill Cosby "Noah" routine... "Lord, what's a cubit?" Sleepy isn't terribly bright sometimes and feels as puzzled about earmarks.


The word conjures up images of pigs and pork and all of the bad smells that go with a pig farm. If you want to coin a phrase that reeks of fat and lazy animals, earmark is a perfect word to use.



Sleepy's business experience is that people who receive budget money in corporate America love it when the uses for those funds aren't terribly specific. That gives the manager much leeway in spending the funds and allows for some changes in plans that may come up during the year. When a budget has been justified by a specific purpose, that purpose can often be completely forgotten by the time the funds are spent by the manager.


Google "earmark" and you'll find explanations from the viewpoint of the OMB (part of the Executive Branch of our government): http://earmarks.omb.gov/

It looks to Sleepy like an earmark from the OMB perspective is anything from the Legislative Branch that reins in its ability to use discretion in spending the taxpayers' money.

Google "good earmarks" and you get a different perspective. Everyone agrees that the bridge to nowhere is not our Legislative Branch's finest moment, but there are cases where some specificity and accountability in having legislative intentions implemented aren't terrible things.

Perhaps when legislation is designed specifically to help sheep avoid the slaughter of ALS it isn't all that evil. It may handcuff the Executive Branch's discretion a tad, but in the big picture, is it really a smelly barrel of pork?

Medicare Is Newest "Black Hole"

In 2000 Congress met several times with an ALS patient that took on Medicare with the use of only two fingers. From the dark side of Lou Gehrig's disease, David Jayne had been granted "homebound" status by Medicare but broke the rules when he attended a football game. He was branded a Medicare criminal and his benefits recinded immediately. That's all it took for David to take on Medicare and so his solitary fight took shape. He founded, National Coalition to Amend the Homebound Restriction. This two fingered sports fan became the new Georgia Bulldog for ALS rights. David made week-long trips from his Atlanta home to Washington to lobby for legislation lifting restrictions that keep people who receive Medicare home nursing services from leaving their homes. Each trip to D.C. involved a mountain of detailed planning and scheduling by his family and friends. As with most ALS patients David couldn't speak, couldn't breathe on his own, couldn't feed himself and couldn't get on the toilet without help.
With the support of Sen. Bob Dole they pushed Congress just to take a look at the restrictions. Simple enough, you'd think? David felt that this experiment might flop when it was finally signed in 2004. He felt that Medicare simply could not handle the project. "If an adequate population sample is not achieved then Medicare will not be in favor. Congress gave Medicare the task to investigate the program in three states. Congress then authorized Medicare to enroll 15,000 in the study but their non-aggressive enrollment campaign only yielded 58 (stringent demonstration enrollment criteria kept enrollment low). Their conclusion following the two year study was written by Mathematica that stated, "Barriers to a successful experiment were difficult and policy issues remain. How lame can a federal agency get?
In my view stringing this one issue out for seven years is appalling. How much humiliation was a man near total paralysis supposed to endure. Heads should have rolled for the fact that a two year study proved no results, only that it did not work out. David had two working fingers, two eyes and with a friend, a football game could still be a blast.
Medicare has become a Black Hope that is going to suck the very existence away from ALS patients like David Jayne. President Bush has again offered dangerous policy prescriptions in his 2009 federal budget proposal that would further damage our broken health care system. This budget, if enacted, would undermine critical public health insurance programs and state budgets by cutting Medicare by $178 billion over the next five years and cutting Medicaid by $17.4 billion over five years. These heavy-handed program cuts and recycled ideas will cause further damage to our fragile health care system.
Maybe the Medicare Black Hole has a hidden message, if you got problems, "fin for yourself." For people with disabilities, finding quality attendant care can seem like a complicated maze, taking in a movie or a football game is just a few of life's simple options.
We need smarter solutions, and real reform.

To view Mathematica's full 140 page report (Black Hole) dated Oct. 2007 http://www.cms.hhs.gov/Reports/downloads/homebound.pdf

My Lands, This Is Confusing!

Sleepy is confused.

This week according to

http://www.lvrj.com/news/17591014.html ,

Coburn worked hard to add amendments to federal lands legislation, one of which would have required the Office of Manpower and Budget to complete an annual inventory of federal lands along with a tally of the costs of ownership and maintenance of each and every one. His fellow Senators rejected his amendment because of the cost and burden of our government annually registering 1.2 billion property assets and 635 million acres of land.

OK... so in Senator Doctor's mind, it's good to inventory real estate annually because it holds clues to costs of ownership but it's bad to inventory people who hold clues to a fatal (and expensive) disease.

Sleepy doesn't understand the logic.

The One Willing To Speak Up In This Conversation...

...is the one whose disease is stealing her ability to talk.

http://www.tulsaworld.com/news/article.aspx?articleID=20080412_1_A13_hShew08807

ALS patient fighting back by: JIM MYERS World Washington Bureau4/12/2008 12:00 AM
She wants to know why Sen. Tom Coburn is blocking a bill to create a national patient registry. WASHINGTON -- Sherry Owens clearly is frustrated. The Sulphur businesswoman, wife and mother of two grown sons is suffering from Amyotrophic Lateral Sclerosis (ALS), better known as Lou Gehrig's disease. With her muscles collapsing, Owens, 53, must use a wheelchair, but that's not what she wants to focus on these days. Owens' frustration is caused by some of the responses she has been receiving from U.S. Sen. Tom Coburn's office to her questions about why the Oklahoma Republican is blocking a bill to create a national ALS patient registry. "It is like talking to a brick wall when you call up there," she said. Owens also took issue with what she saw as sarcasm from one of the senator's aides as he explained how Coburn came up with the scores of holds he has put on various bills. "That really set me off," she said, adding that the response played a part in her decision to take her concerns public. "I am so sick and tired of hearing those answers." Coburn would not comment. Senate Majority Leader Harry Reid, D-Nev., who introduced the bill to create an ALS patient registry, says it will improve understanding of the debilitating disease and possibly boost chances for a cure. Reid's version of the bill authorized $25 million for the first year with additional funds to be added later; an amended version changed the funding to authorize $43 million over the next several years. Co-sponsors range from liberal Democrats to conservative Republicans. Reid spokesman Jim Manley said Coburn "is blocking a bipartisan, broadly supported bill that has 72 co-sponsors and would easily pass the Senate. It is a particular shame that Coburn is dragging his feet on this by creating a tyranny of one, considering ALS patients have so little time." Patrick Wildman, an official with the Washington, D.C., office of the ALS Association, said no one with that group could comment on the bill or its status. Owens said she has nothing to lose by going public with her frustrations. She points out that ALS has been well-known for decades because of Lou Gehrig; she considers a registry only as a starting point. "They are not doing enough for a disease like ALS," she said, conceding that the number of ALS sufferers is small compared with people with AIDS or cancer. But Owens said such comparisons become meaningless when ALS hits home. She questioned why Coburn, who often cites his experiences as a physician, would take the position he has taken on a bill dealing with a disease. Owens' problems had begun several years earlier, but she was diagnosed with ALS two years ago. ALS is a progressive neu rodegenerative disease that affects nerve cells in the brain and the spinal cord. ALS patients in the later stages of the disease may become totally paralyzed, and the disease eventually leads to their deaths. A registry "is not going to save my life," Owens said, but she added that at this point, any improvements in how people with ALS now live would be welcome. "I don't want to have this conversation with somebody else 10 years from now because nothing was done," she said. "My God, it has been years. Most of us don't have a lot of time to waste."

This Could Be More Fun Than Starbuck's

If you require a feeding tube, I'm sure the good physician/Senator will be glad to assist. Notice that RSVP is required. Sooners, I hope you will enjoy some java and interesting conversation when you're in DC.

Curts Pitch 4 ALS Goes Global!

Curt Schilling is on the DL but that does'nt stop his ALS Advocacy.

The Boston Red Sox's travel to Japan for exhibition games and Curt took time to visit ALS patients and their families at the Tokyo Dome. There are approximately 8,000 known ALS patients living in Japan. It is thought however that there are 75% of that number living in Japan that are coping with ALS undiagnosed. The ALS chapter was started in 1986 and works in relative obscurity, which is unfortunate. Curt's plan is to change that in the 2008 baseball season.
Curts Pitch 4 ALS is a program started 17 years ago. Basically Shonda and Curt donate $100 dollars per strikeout, and $1000 per win to the local ALS Chapter(s). What started as a program to benefit the Philadelphia ALS chapter run by their friend and tireless advocate Ellyn Phyllips has now grown to encompass the Arizona and Boston Chapters of ALS.
Curt has recruited pitcher Daisuke Matsuzakas and is hopeful that he can generate a massive amount of awareness for ALS in Japan. So far the Japanese players are not involved with ALS, and the ties are just too strong between the game and the disease to not try and make that happen.

Me thinks that nominating Curt Schilling to run for public office would be a good thing but this Ambassador is very simply over qualified.