Another Young Man Named Hall, Another Case of ALS

[IndyMichael]

Here's a pic of my group, walking in honor of my sister Kris.

http://www.flickr.com/photos/michael...in/photostream

[rocketdoc]

First I want to send my best to the Hall family and all who have had ALS come into their lives. Last week my cousin's wife was diagnosed with it. What can I say? I send prayers.

We can only hope for a miracle; something new around the corner to fight this.

...Perhaps there is.

Ref: http://neurogenetics.northwestern.edu/

Recently, a study done to a relatively large sample of ALS victims demonstrated that every (every single one) ALS patient had one thing in common; an inability to properly "flush" used, malformed or bad" proteins from their cranial-spinal fluid. This is seen as a major breakthrough in their attempts at killing this disease off.


Major Breakthrough in ALS

CHICAGO --- The underlying disease process of amyotrophic lateral sclerosis (ALS and Lou Gehrig’s disease), a fatal neurodegenerative disease that paralyzes its victims, has long eluded scientists and prevented development of effective therapies. Scientists weren’t even sure all its forms actually converged into a common disease process.

But a new Northwestern Medicine study for the first time has identified a common cause of all forms of ALS.

The basis of the disorder is a broken down protein recycling system in the neurons of the spinal cord and the brain. Optimal functioning of the neurons relies on efficient recycling of the protein building blocks in the cells. In ALS, that recycling system is broken. The cell can’t repair or maintain itself and becomes severely damaged. The discovery by Northwestern University Feinberg School of Medicine researchers, published in the journal Nature, provides a common target for drug therapy and shows that all types of ALS are, indeed, tributaries, pouring into a common river of cellular incompetence. “This opens up a whole new field for finding an effective treatment for ALS,” said senior author Teepu Siddique, M.D., the Les Turner ALS Foundation/Herbert C. Wenske Professor of the Davee Department of Neurology and Clinical Neurosciences at Northwestern’s Feinberg School and a neurologist at Northwestern Memorial Hospital. ”We can now test for drugs that would regulate this protein pathway or optimize it, so it functions as it should in a normal state.” The discovery of the breakdown in protein recycling may also have a wider role in other neurodegenerative diseases, specifically the dementias. These include Alzheimer’s disease and frontotemporal dementia as well as Parkinson’s disease, all of which are characterized by aggregations of proteins, Siddique said. The removal of damaged or misfolded proteins is critical for optimal cell functioning, he noted.

This breakdown occurs in all three forms of ALS: hereditary, which is called familial; ALS that is not hereditary, called sporadic; and ALS that targets the brain, ALS/dementia.

In related research, Feinberg School researchers also discovered a new gene mutation present in familial ALS and ALS/dementia, linking these two forms of the disease.

Siddique has been searching for the causes and underlying mechanism of ALS for more than a quarter century. He said he was initially drawn to it because, “It was one of the most difficult problems in neurology and the most devastating, a disease without any treatment or known cause.” Siddique’s efforts first showed in 1989 that molecular genetics techniques were applicable to ALS, then described the first ALS gene locus in 1991, which led to the discovery of SOD1 and engineering of the first genetic animal model for ALS.

ALS affects an estimated 350,000 people worldwide, including children and adults, with about 50 percent of people dying within three years of its onset. In the motor disease, people progressively lose muscle strength until they become paralyzed and can no longer move, speak, swallow and breathe. ALS/dementia targets the frontal and temporal lobes of the brain, affecting patients’ judgment, the ability to understand language and to perform basic tasks like planning what to wear or organizing their day. “These people in the prime of their lives and the peak of their productivity get this devastating illness that kills them,” Siddique said. “The people who get ALS/dementia, an even more vicious disease, have a double whammy.”
BROKEN DOWN RECYCLING SYSTEM

Feinberg School scientists found the cause of ALS by discovering a protein, ubiquilin2, whose critical job is to recycle damaged or misfolded proteins in motor and cortical neurons and shuttle them off to be reprocessed. In people with ALS, Feinberg researchers found ubiquilin2 isn’t doing its job. As a result, the damaged proteins and ubiquilin2 loiter and accumulate in the motor neurons in the spinal cord and cortical and hippocampal neurons in the brain. The protein accumulations resemble twisted skeins of yarn -- characteristic of ALS -- and cause the degeneration of the neurons. Researchers found ubiquilin2 in these skein-like accumulations in the spinal cords of ALS cases and in the brains of ALS/dementia cases.

The scientists also discovered mutations in ubiquilin2 in patients with familial ALS and familial ALS/dementia. But the skein-like accumulations were present in people’s brains and spinal cords in all forms of ALS and ALS/dementia, whether or not they had the gene mutation.

“This study provides robust evidence showing a defect in the protein degradation pathway causes neurodegenerative disease,” said Han-Xiang Deng, M.D., lead author of the paper and associate professor of neurology at the Feinberg School. “Abnormality in protein degradation has been suspected, but there was little direct evidence before this study.” The other lead author is Wenjie Chen, senior research technologist in neurology. About 90 percent of ALS is sporadic, without any known cause, until this study. The remaining 10 percent is familial. To date, mutations in about 10 genes, several of which were discovered at Northwestern, including SOD1 and ALSIN, account for about 30 percent of classic familial ALS, noted Faisal Fecto, M.D., study co-author and a graduate student in neuroscience at Feinberg. The study was supported by the National Institute of Neurological Disorders and Stroke, the Les Turner ALS Foundation, the Herbert and Florence C. Wenske Foundation, the Blazeman Foundation for ALS and other sources.


My wife died of ALS and I tend to follow things that involve neuro-related diseases.

My thoughts go out to everyone that this disease has touched.

[rknt50a]

Very nice feature with Sarah Fisher, Carey Hall...

http://www.theindychannel.com/sports...11/detail.html

What a fine person SF is!

Chris Pisano from WRTV did a nice job on the piece. He lost his father-in-law to ALS.

[mattf765]

Really nice piece there. SFR is the embodiment of what Indycar should be as far as I'm concerned.

[R22]

my sister-in-law's brother died last week, while i was at the chili bowl, from ALS... same ole story, young man gone much too soon. family and friends left behind hoping that something can be done so others don't have to suffer from this terrible dease.

so, just keeping this thread going to remind all that the need for funds and support continues.

just because, C n B has ended doesn't mean that we can't still support the cause in our own ways in our own home towns.

please do your best to give what you can to fight this dease.

[#7batmobile]

my sister-in-law's brother died last week, while i was at the chili bowl, from ALS... same ole story, young man gone much too soon. family and friends left behind hoping that something can be done so others don't have to suffer from this terrible dease.

so, just keeping this thread going to remind all that the need for funds and support continues.

just because, C n B has ended doesn't mean that we can't still support the cause in our own ways in our own home towns.

please do your best to give what you can to fight this dease.

Very sorry that you lost a loved one. ALS has touched my family, as well.We have always donated to ALS, in hopes that the medical community will find a cure. (On a personal note, I've always respected you,plus, you always make me laugh.)Our family will include your family in our prayers. That's a promise. God bless,R22. And, to every loved one who has passed and their families who are hurting, you are in our prayers tonight.

[rknt50a]

There is going to be a huge boost of ALS awareness tomorrow in the Super Bowl pregame show on NBC --

http://www.alsa.org/news/archive/sup...game-show.html

Here is nice coverage of them leaving New Orleans for Indy yesterday. The video speaks volumes about ALS. --

http://www.wdsu.com/r/30374359/detail.html

Finally, they are definitely doing cool stuff in Indy (with Graham Rahal) --

https://twitter.com/#!/team_gleason/...106944/photo/1

Please spread the word so that people learn about ALS on the Super Bowl pregame show. Thanks.

[brians.mom]

Thank you so much for your continuing to be involved w/ "our" disease - ALS.....

We still do not have a cure or much to help our patients - altho we continue to find ways to make them more comfortable.

It is totally a Small World - who woulda thought that our moving to South Carolina and the Hilton Head area, that we would
not only run into someone who has a son w/ ALS but is also from the Indy area!

This is so exciting not only for this family but for all of our ALS families to participate in this years Super Bowl in Indianapolis.

We wish them God speed as they move thru this disease.

Brian's family

[rknt50a]

Latest reports on twitter are that the segment will run around 4:37 PM EST on the NBC pregame show today.

Today will be a huge day for ALS awareness!

http://www.washingtonpost.com/sports...6pQ_story.html

[rknt50a]

Nice coverage...

http://www.nola.com/superbowl/index....reman_hav.html

[trevor83]

In less than 11 hours we will surprise a member of the Indycar community's family with a surprise remodel of their house. Carey Hall, former Sarah Fisher Racing crew member, has ALS. We will remodel the master shower for better accessibility and install laminate flooring throughout the house. You can see some of the coverage Saturday morning on WTHR 13 Sunrise. First segment live interview with Sarah Fisher somewhere around 8:45-8:55am. Footage of reveal at 9:55am and full story on the 6pm news. I believe Fox59 will catch the reveal live which will happen close to 9am. Jake Query will be impersonating Ty Pennington for our Extreme Makeover INDYCAR Edition. Home Depot has been the MVP of this project, they did things on their own accord that we didn't even ask for, incredible! Many thanks also to The Tile Shop in Castleton, Brozinni (brozinni.net) in Indianapolis and Chris Lechner. We will post updates when possible thru the weekend. You can follow updates thru the weekend on Twitter @trevor8383 & @KelseyUSA.

[trevor83]

Due to storm coverage tomorrow tv change. One live segment somewhere around 8:45-8:55am. No live reveal. Another spot at roughly 9:55am with footage of reveal. Then a complete news story sometime on the 6pm news. Switch to Fox59 right after Sarah's interview to catch the live reveal then come back to WTHR for recap at 9:55am. You can also catch Sarah on Sunday night 11:35pm on Channel 13 Sports JAM.

[Indy Soup]

One of my best friend's mother AND brother both died from ALS and I'd like nothing more than to see it cured and prevented. I applaud Jake Querey and Sarah Fisher et al who are involved with the home modification project, and my wish is that we can all take care of each other like the IndyCar family does.

The following may be a touchy subject and might not square with everyone, but I feel I cannot keep silent about what I've experienced in my life and the lives of others. And, I happen to live in a state that allows alternative herbal remedies and I acknowledge that others may not. That stated, there is a plant that either was created or evolved on earth (makes no difference) that has amazing curative properties. It is one of man's oldest pharmacological agents and God and/or Darwin saw fit to make sure it grows naturally all over the planet. Unfortunately, this gift of nature has been marginalized, trivialized and demonized in the last 100 years. I have personally seen and experienced miraculous healings - if not outright cures - but definite improvements in symptom reduction and increase in quality of life for people with MS, MD and Spinal Cord Injuries. I hope that we, as a compassionate species, can put prejudices and superstitions aside and open our minds to something right in front of us.

But don't take my word for it...here's what the National Institute for Health says about cannabis and ALS:
http://www.ncbi.nlm.nih.gov/pubmed/20439484

[rknt50a]

trevor83, you're a good egg.

This is wonderful in many ways.

[trevor83]

Sorry everyone, broadcast dates/times were what I was told by the station. We got bumped by storm damage, rightfully so. There will be coverage of the story tonight on Fox59 10pm. There might be some coverage during Sarah Fisher's segment on Sports JAM Channel 13 at 11:35pm. What an incredible weekend. Home Depot was amazing. The Tile Shop, Chris Lechner and Brozinni Pizzeria all hit home runs in their assistance. Some things I expected and many things I didn't. Really hard to explain without experiencing it. I met Brian Hall after he was already bed ridden. This is much more emotional since Carey is still somewhat mobile. I know where its headed. He's funny though, he had me cracking up a few times. I did this in memory of Chris Griffis who Carey was good friends with. I had a lot of respect for Chris having worked as a spotter at Indy for him the last 6 years. This was time to pay Chris back. We included Mari Griffis in the project and she was awesome. We have one more day of work to tie up some of the stuff and will probably finish out the bathroom next weekend.

[trevor83]

Been informed that story for WTHR 13 will air next Sunday, 3/11. Not sure what broadcast time. We have unedited footage shot by @KelseyUSA that should uploaded soon.

[aowzone]

Bravo, guys. Carey is the man!

[rknt50a]

Nice article from Jake Query --

http://www.wnde.com/pages/query-schu...rticle=9854537

[trevor83]

Listen to Query & Schultz today from 3-7pm to get Carey & Carla's perspective of living with ALS and also the home remodel from the weekend. 1260am in Indy or stream online http://www.wnde.com/cc-common/listenlive/

[cheeseczar]

Good stuff Trevor. I'm proud to call you friend.

[R22]

wtg trevor.. good job!

[trevor83]

Pictures

http://www.viewthrumylense.com/CareyHallHomeRemodel

[trevor83]

Story planned for Channel 13 Sports JAM this Sunday night. Sometime between 11:20 and midnight.

[trevor83]

See above for coverage tonight.

For the record, this disease SUCKS! Carey still goes to work every day, he is truly an Iron Man! If you could see what he has to do just to get in the truck, its inspiring to see his fight even as this takes his muscles away. I was sore and tired last weekend, only getting 3 hours of sleep. But it was nothing compared to what he has to go thru every day. That kept me going.

[trevor83]

Great interview with Josef and Sarah. Video of Carey Hall's home makeover at the end. http://www.wthr.com/video?autoStart=...clipId=6831928

[dog-ring]

Goferboy.....................You done good......................

[trevor83]

Goferboy.....................You done good......................

I was thinking and realized you were a part of this. My primary driver was to do something for the Griffis family which ended up helping Carey. If you hadn't told Chris to hire me in 2006 this might have never come together.

[trevor83]

I asked @KelseyUSA to preserve the weekend for the family with video footage. She went above and beyond my expectations and created this:

[R22]

AWESOME TREVOR!!!! what a great job by all and a blessing to Carey and his family!! 382

[PHJIndy]

AWESOME TREVOR!!!! what a great job by all and a blessing to Carey and his family!! 382

AMEN!!!!!!!!!!!