Dear Smart People At Microsoft,

We've been reading about your work with Steve Gleason at your Hackathon this week.

You inspire us.

We know now that you've met Steve that he inspires you.

Let me introduce you to thousands of other people living with ALS today.  They will inspire you, too.  Some are even younger than Steve, and others are much older.  Some have great support systems and others have none.  Some have means, others nothing.  All are smart. Some have had the disease for a few weeks, others have been at it for months or years.  The turnover is brutal. All lose the ability to do something every day, and those abilities never come back.  All need the help of enabling technologies.

You'll follow Steve's difficult path now that you've met him.  As you think of him and read of his remarkable work, please think about some of those others, too.

ALS runs a difficult, out-of-control path that steals abilities.  Many of us who dealt with ALS in our families found that we were constantly throwing the ball five yards behind the receiver when it came to assistive devices.  The disease runs a fast downhill course.  There is no time to learn and adjust.  You just have to do it.

I remember the days (before you were born) when the first person in the office to get a PC had to go through weeks of training to learn MS-DOS and then the applications.  Thank heavens those days are over.  Those days need to be over for enabling technologies for people with ALS, too.

Thanks so much for what you are doing.  Steve is leading a cast of thousands of diverse people with this evil disease.  You can empower and enable them all to be productive contributors and lead their lives in dignity.  You get it now.

We Don't Know What We Don't Know

Many thanks to Dr. Kevin Horton of ATSDR for his assistance with my questions in my post from July 26, "Who are the 15 Percent?"

The bottom line is that "the 15%" in the recent Registry report does not represent those people with ALS who are not located via the administrative databases.  It only speaks to the quality of what is found within those Medicare, Medicaid, VA files.

The recent Registry report does not address the completeness of the ALS Registry in finding all cases of ALS in the United States.

So how did I (and some others) get a completeness expectation?  I felt foolish yesterday that I completely missed the boat by thinking that the Registry was designed to find around 85% of people with ALS via the passive mining of administrative databases.

But, yes, there had been assertions early on in the Registry approval process that the administrative files would contain all but 15-20 percent of cases of ALS in the Unites States.  Yes, per Dr. Horton, some original pilot sites indicated that they could find more than 80 percent of PALS via administrative files. We weren't hallucinating.

Take a look --
http://web.alsa.org/site/PageServer?pagename=ALS_Registry_Background

... the CDC has created an algorithm that will enable the Agency to accurately identify approximately 80-85% of ALS cases in the United States by examining large national administrative databases ...

...In order to identify the remaining 15-20% of cases, the CDC has launched an on-line web portal that allows people with ALS to self-enroll in the Registry via a secure website...

Now I understand that today, the data in the Registry report did not swing at this completeness nail at all (that I certainly had my eye on).   They did not address how many people with ALS would potentially be outside of the administrative data sets.  Per Dr. Horton, they will do future analysis on this.

Stephen Finger has a graphic in his op-ed that speaks volumes --
http://www.huffingtonpost.com/stephen-finger/national-als-registry_b_5620631.html

His point is important, especially since the Registry report is making headlines without any important context of completeness.

We have a Registry of people with ALS who are in "the system."  They were either in administrative databases or heard and responded to the call to self-enroll.

We have no idea of how many other people with ALS are living in the United States today.

We simply don't know what we don't know... and that's important to understand.

Who Are The 15 Percent?

Note:  I goofed in my original numbers published on Saturday, July 26.  I apologize. Administrative databases contained 8472 PLUS 1789 = 10261 people alive with ALS.  The questions are still there, though.  Corrections made on Monday, July 28.  

_____

One premise of the CDC ATSDR National ALS Registry was that by passively mining data from Medicare, Medicaid, and VA files, they could identify around 85 percent of ALS cases in the United States.

People with ALS go onto Medicare much more quickly than people with other diseases.  The premise seemed reasonable.

http://www.ncbi.nlm.nih.gov/books/NBK208611/

These administrative databases cover approximately 90 million Americans, and the algorithm identifies 80 to 85 percent of all true ALS cases when applied to these databases. 

We thought the files would only be missing around 15 percent of Americans with ALS.  Those were the people who could be counted only if they self-enrolled in the online portal.

Question 1 --

When the first Registry report came out this week, I was anxious to see how many of the 15 percent of people with ALS who are missing from these administrative databases took the initiative to self-enroll at the Registry web portal.

They found 8472 10261 cases of ALS by mining the administrative databases.  Fine.  If that's around 85 percent of ALS cases in the United States, that means that they missed (10261/.85)*.15 or 1810 cases.  There were a theoretical 1810 people living with ALS who were not in the administrative databases.

And how many of that theoretical 1810 self-enrolled at the web portal?  A remarkable 1926.  Yes, every last one of the 15 percent and then some took the time to self-enroll at the web portal.  Self-enrollments far exceeded what we expected in this "15 percent" group.

To add to the amazingness, only 17 percent of the people who were in the group that was in the administrative databases self-enrolled at the portal.  What a difference.

Something is really odd here.

Question(s) 2 --

The expectation based on small pilot studies that the Registry data mining algorithm could identify 85 percent of US ALS cases from files was clear to all of us who worked hard to get the Registry approved and well-funded.

Question 1 now has me wondering if that expectation was simply wrong.

From the recent Registry report --

The algorithm was developed initially during the pilot
projects and categorized persons as either “definite ALS,”
“possible ALS,” or “not ALS,” with a sensitivity of 87% and specificity of 85%

Now I'm wondering if the premise should have been that if a case was identified in the passive data mining that  87 percent of the time it was really ALS (and that if somebody looked like a non-ALS person in the administrative data that 85 percent of the time that person did not have ALS) ?

To add to my head-scratching, if they found MND codes (rather than the more specific ALS codes) in the administrative files, that could well account for around 15 percent of the mined records being a MND other than ALS.

Does the 15 percent refer to something strictly within those in the administrative files (and not the Americans who aren't in the files)?

Was the idea that the Registry could identify 85 percent of ALS cases in the US by passive data mining wrong?

Do we really know anything about how many people with ALS are expected to be outside of these administrative databases?


It seems like the answer to Question(s) 2 will tell me if Question 1 is a silly question asked by an advocate who didn't read the fine print closely enough.

We Need To Talk!

Yesterday we got the long-awaited first data from the CDC ATDSR ALS Registry project.

Now we need to talk.  We need to talk a lot.

This is not a time for those with the bully pulpit on ALS to be shy.  This is not a time to distract us with other projects that the Registry's budget can swoop in and do.  The Registry was always about the incidence and prevalence of ALS in the United States.  We need to speak to the numbers now.

ALSA and MDA and Communicatehealth Inc. have all received hundreds of thousands of dollars for education and outreach and communication related to the Registry project.  This is a good time for them to step up and educate and reach and communicate.

Here's the report.  It's a good starting point for a much-needed national conversation on ALS.

There's a lot to talk about.

Prevalence -- A One-Legged Data Stool

We learned how many living people with ALS that the Registry could find in that 13-month period.  It said nothing of how many people were actually newly diagnosed with ALS in that period.  It said nothing of how many people died from ALS in that period.

There are three facts that together should make every American squirm --
1. You are quite likely to GET ALS. (incidence)
2. You are quite unlikely to be ALIVE with ALS. (prevalence)
3. You are therefore quite likely to DIE from ALS. (mortality)

If they only look at the second fact, healthy Americans get quite a peachy, safe feeling.  It's the feeling that ABC News propagated last night.  Yes, "rare" makes healthy people feel safe from the threat.  Nothing could be farther from the truth.

High incidence and low prevalence are a highly toxic combination.

The Registry did not address incidence, and the report admits a huge blind spot in that regard.

Say What?

Organizations have played fast and loose with estimates for years -- a combination of a crutch and telling people what they want to hear.  We need to learn to speak to the correct numbers correctly.  ALSA and MDA have been insiders and paid contractors on this project.  They have had time to prepare for this day.  We need to rid the world of past inflated estimates of numbers of people ALIVE with ALS.  The "up to 30,000" myth needs to stop.  Once diagnosed, people die fast and there is nothing to slow ALS down.  As much as we like to think that they are living longer because of compassionate care, they don't live long.  As much as organizations like to tell us that they each support around 14,000 people with ALS in their clinic systems, that looks pretty impossible now.

Demographics -- Who Gets ALS or Who Makes It to the System?

This is a wonderful opportunity for us to step back and evaluate whether the demographic data cuts we saw in the report are really reflective of ALS or are reflective of those ethnic and socio-economic groups that make it to ALSA and MDA and Medicare and Medicaid and the VA with an ALS diagnosis.  That's an important conversation.  Do we lose people to ALS who don't look like Lou Gehrig and therefore die before they are ever diagnosed?

Don't Clam Up

After the unfortunate ABC News item last night, I was hoping that ATSDR or MDA or ALSA or someone with a bully ALS pulpit would have been on GMA this morning to talk about the numbers and the threat of a disease that you are likely to GET and unlikely to be ALIVE with.

It's also unfortunate that for the first time in years, ALSA issued a press release and did not post in on its facebook wall were it could be discussed openly.

We have spent over $40 million and have waited far too long to find just over 12,000 living people in the USA with ALS in a 13-month period.  That's worth a lot of conversation.  If you avoid that conversation, you'll miss the value in knowing the real threat of a high-incidence, low prevalence disease.

This Senator Is Different

Every year hundreds of people interested in advancing the fight against ALS call on U.S. legislators. Some officials are polite and others, not so much.  Most listen.  The message only seems to stick with those who have some past connection with ALS.  Some try to help.  Most file the ALS message in their minds along with dozens of other diseases that need more attention.

But there is one United States Senator who is different.

ALS caregiving changes a person.  "Caregiving" isn't just running errands or delivering an occasional casserole or visiting on a Sunday afternoon.  ALS caregiving involves an intense mix of physical and mental and emotional challenges.  You work a feeding tube and clean the suction machine and invent ways to communicate and deal with body functions and learn how to defy gravity and skip sleep and hold the hand of a dying loved one and watch that loved one slip away regardless of all you try to do.

And people who have been ALS caregivers have an unspoken bond.

There is one member of the Senate who shares that bond.  She is a one of our gang of ALS caregivers.

We are hardly in the league of special interest groups or fancy lobbying organizations.  We simply care and respect one another.  We don't have to talk much.  We know each other well regardless of how our lives differ in other ways. We have lived first-hand the ugly ALS menace.  We have a rage.

We are grateful that she shares the caregiver bond and that she is willing to address the nuts-and-bolts of making a difference for the next person.  Please take a look at this op-ed on the leadership of Senator Lisa Murkowski of Alaska --

http://blogs.seattletimes.com/opinionnw/2014/07/21/alaska-sen-lisa-murkowski-leads-again-on-als/#.U841VTFEMdE.blogger

This Senator is different.  Thank God.

And There Are So Many Books That We Would Love To Finish

A few weeks ago there was a list published of the books we start to read and never finish.  Thanks to e-readers, we are caught in our tracks.

In 1996 on the day that Mom was diagnosed with ALS, I went home and started the research that every family member does.  I got out the old Merck Manual and the Dorland's and didn't like what I saw.  I used the then state-of-the-art dialup modem and got online at Prodigy and Compuserve and searched. Blogs weren't invented yet, but I found a couple of message boards that served a similar purpose.  There was a woman on one who gave a continuing stream of some very helpful nuts-and-bolts information about dealing with ALS.  I knew the outlook wasn't good and that we would need all the help we could get.  I read her posts in chronological order for nearly an hour. She had a gift for being helpful.  They suddenly, there was a post that she died, surrounded by family.  No!  Out of the blue the stark reality of ALS hit me.  Bright, helpful, productive people who are able to write one day are stopped in their tracks by a disease with no mercy.  I went back and reread looking for a better ending.  There was none.  Her story was ended long before its time by ALS.

Over the years blogs have arisen and it's not unusual for people with ALS to blog their journeys. Some are warm and hopeful, others are edgy and dark. Some are pleasant and cooperative, others are outraged and demanding change. Some are all about ALS, others speak little of the disease itself.  They all are important for understanding some courageous people who are facing an outrageous disease.

The big problem today is the same I experienced that night in 1996.  The blogs all end before the author is finished.  ALS stops it all.  Tomorrow the next person starts a new blog.

Fortunately the internet preserves some of these valuable writings long after the blogger has died.  The endings are often abrupt.  The work is not finished.  That's ALS.

There are many blogs from living people with ALS and you can find those with a Google search.

Here are some oldies that are part of a valuable archive of how much really hasn't changed in the world of ALS.  They will give you painful insight into the people and the disease, and we should all look for the ideas that will finally fix the fight against ALS. Some started before the writers even heard of ALS. All were ended by the writers' deaths.  You'll get the best understanding if you read them from oldest to newest entries.  I've tried to point you to the older posts.

http://barbarabrenner.net/?paged=8

http://scjohnson63.tumblr.com/page/6

http://als1dog2kids1wife.tumblr.com/page/2

http://brainhell.blogspot.com/2003_12_01_archive.html

http://www.lifewithals.com/Blog.html

http://carlamuses.blogspot.com/search?updated-min=2006-01-01T00:00:00-08:00&updated-max=2007-01-01T00:00:00-08:00&max-results=5

http://www.melissa-erickson.blogspot.com/search?updated-min=2008-01-01T00:00:00-08:00&updated-max=2009-01-01T00:00:00-08:00&max-results=12

http://www.iambreathing.com/plattitude?page=6

http://kensjourney.com/index.html

http://alsboy.wordpress.com/2008/08/

http://twohlson.com/journal/

People With ALS And Their Caregivers Need A Place At THE Table, Not Just A Table

It's too easy to break out another table and say that people with ALS have a place at the table.  A separate table is not THE table.

People with ALS and their caregivers need to be at the same tables with researchers and clinicians.  They need to talk directly.  

I was fortunate to go to a college where faculty and administrators sat with students in the same cafeteria at the same tables.  That was a good thing for all.  A few years later I worked at a large corporation where the man whose name was on the company skipped the management dining room and joined employees at the tables in the regular cafeteria.  That was a good thing for all.

ALS meetings, be they scientific or policy or clinical, need to include people with ALS and their caregivers.  And "include" doesn't mean a separate session or a separate table.  When meetings exclude the very people with the disease, it speaks volumes about one big blind spot in the fight against ALS.

We can and must do better.  People with ALS and their caregivers must be welcome at every ALS meeting, both in person and virtually.  They must be at the table, both in person and virtually,  whenever direction is given to the fight.  They are the ultimate experts regardless of what scientific and organizational egos think. 

Some Words Blind Us

The fight against ALS has never been a well-oiled machine.  Everyone knows that it would be more effective if people worked together better.

We love the word "partnership."  That gives us visions of people and organizations finally working together.

The word "collaboration" makes us so happy.  At last people are sharing to help everyone advance the science.

Then we find out, often much later or by accident,  that a "partnership" came with a big money contract.  And next we discover that people in a "collaboration" were paid for the arrangement.

Those magic words that give us visions of people doing the right things for the right reasons have blinded us to pertinent business arrangements.

There's nothing wrong with a mutually beneficial business deal, but people need to know who is being paid by whom to do what in the fight against ALS.  We donors and advocates hear the magic words and assume things that may well not be true.  Scientists and not-for-profit employees participate in meetings where there is not a clear picture of who is being paid by whom to be there and to "partner" and to "collaborate."

Obligations for transparency go far beyond annual reports and tax forms.  People need to understand who is being paid by whom to do what.  Every time that the word "partnership" is used in an arrangement where there is an exchange of money, we have a right to know that.  Every time a "collaboration" is a business arrangement, we need to know that.  Whenever people are paid to participate in a meeting, that should be on the table.  It's material information to understand an individual's or an organization's perspective.

We need to cut the blindfolds on "partnerships" and "collaborations."

What Does Every American Need To Learn Today?

Yesterday @alsadvocacy tweeted a question --

"What's the most important fact that every Jane or Joe Doe needs to know about ALS?"

That's the kind question that public relations and communications firms ask before they design campaigns.

Almost immediately @MrChuckMorris, a gentleman with ALS, tweeted back --

"Incurable, terminal ALS could affect them, or someone they love, any day."

That's it.  Nail hit on the head.  Clarity. No expensive p.r. firm needed.

During a few days of marvelous celebration of the 75th anniversary of Lou Gehrig's historic, beautiful farewell, we've learned much about Lou Gehrig -- the man, the athlete, the celebrity, the American.

As baseball fans leave ballparks this week after all of the celebrations and festivities, will they have any idea that incurable, terminal ALS could affect them, or someone they love, any day?

As Americans hear the messages of hope and celebration will they grasp that incurable, terminal ALS could affect them, or someone they love, any day?

As we are told to contribute to all of the promising work and research that done by ALS organizations, will Jane and Joe make the leap that ALS is still terminal and incurable and likely to strike after all these 75 years?

There are some history lessons that America is missing today.  Lou Gehrig died less than two years after that speech.  Hundreds of thousands of people have been killed by it since.  They still die today.  The prognosis is the same that Lou and Eleanor Gehrig faced in 1939.  That's outrageous. It's a difficult disease. There is no known cause. There is no effective treatment.

Incurable, terminal ALS could affect YOU, or someone you love, any day.

We Delivered, On Time, Every Time

Yes, we American taxpayers deliver the funds for the CDC to do its work.  We deliver every April 15.  Every year.  On time.

Yes, we ALS advocates delivered the requests for the CDC to produce an ALS Registry.  We've delivered over $40 million dollars.  Every year we answer the ALSA call.  On time.

As recently as May we were promised at the ALSA Advocacy Conference (yes, we showed up on time and went to Capitol Hill to request more funds on time) that the first data sets from the Registry would finally be published in MMWR in June.  We assumed that was June, 2014.

Every week in June, we looked at http://www.cdc.gov/mmwr/ to find the data they promised.  Nothing.

We paid $40 million.  They didn't deliver anything... even an excuse.