Last month I wrote about the Steve Saling ALS Residence. Steve recently posted a video about the technology which is a large part of what makes the Residence so wonderful for PALS. Every PALS should demand an ALS Residence in his/her local community.
First off, there is some wonderful news: Diaphragm pacing system receives FDA approval for use with ALS patients. This is a device which extends time until vent by electrically stimulating the diaphragm. Note that this will not replace a vent as the diaphragm muscle needs neurons to release acetylcholine to the muscle fibers in order for them to contract. However, I urge all PALS reading this to immediately begin talking with your neurologist about whether this would be right for you. Here is some good information regarding the DPS.
Next up is the recent news regarding the Neuralstem trial involving stem cells implanted in the spines of PALS. Readers can see a poster-style synopsis of current trial data here. The initial results are encouraging, and one patient has even shown improvement. The next step in the trial is cervical implantation where the cells will have the chance to impact the phrenic nerve (the “money” nerve that serves the diaphragm and breathing). There is still much to learn about this technique before it is available to but a few. Remember that this is still a safety trial. I would urge readers to consider the words of Neuralstem’s CEO.
I am a huge believer in staying at home while living with ALS, having done so for 4 years now. However, the financial stress is outrageous. I am personally bankrupt and have critically stressed the finances of my family. This is because there are no institutions which would accept me on a vent. Steve Saling, an architect with ALS, proactively designed a residence for people with advanced ALS and MS which utilizes technology to maximize their remaining independence and comfort. Steve currently resides there happily, and last year another PALS on a vent was rescued from a horrific institution and is now safely and happily living there. If I had a Saling Residence anywhere in this state I would have gone to live there instead. Please read the email message below.
Immediate Attention – If you’ve ever wanted to help, here’s your chance. Please spread this to anyone and everyone you can. Please write your letters ASAP. See video here for inspiration: http://www.youtube.com/watch?v=KlQvcw3kQe4
We need your help in establishing a residential living center for those suffering from ALS or MS in Georgia – We need your letter no matter where you are, if we can provide a residence like this here, your state could be next!
We really need your help in generating the information requested below no later than September 15th. Forward this email to anyone you think can help. If we are unable to make a persuasive case for this facility it will not be approved and Georgia families (and everywhere) will be left to fend for themselves in supporting their loved ones afflicted with ALS/MS.
Thanks for your continued support.
We need your help in establishing a residential living center for those suffering from ALS or MS in Georgia
We are in the final stages of securing approval from the State of Georgia to build a skilled nursing facility that will focus on serving residents with neurological diseases or impairments to include an emphasis on providing care to MS and ALS residents. This state of the art facility will utilize technology to provide the maximum personal independence for each resident.
All of the necessary paperwork has been submitted and we are now being asked to provide additional written documentation by September 15, 2011 on the challenges currently being faced by those suffering from these illnesses.
We need letters from individuals and families outlining the difficulty they have had in securing residential skilled nursing care for patients with ALS or MS. We need letters from doctors and case workers showing the difficulty families have in Georgia trying to find suitable living accommodations for those living with ALS or MS. We need to hear from families who have had to go outside Georgia to find suitable care for their loved ones suffering from ALS or MS. It would be very helpful to hear from healthcare providers on the difficulties they face in finding suitable housing options for people living on a vent.
If we cannot convince the State of Georgia there is a pressing need for this type nursing center which is currently not being met by traditional nursing centers, it will not be approved.
Any letters of support, documentation or testimony should be addressed to:
Division of Health Planning, Department of Community Health
Please EMAIL me your letter as soon as possible. I need to receive them before September 15th, 2011, via my email at email@example.com – Please copy Bill Saling at firstname.lastname@example.org
A few weeks ago, Professor Stephen Hawking was quoted in The Guardian newspaper saying “there is no Heaven; it’s a fairy tale.” Shortly after, two radio “shock jocks” in the Houston market decided to take issue with Professor Hawking’s words by belittling his condition and questioning his abilities. I won’t repeat their attack, but it was immature and offensive to all people affected by ALS.
In response to their attack, a Facebook group was formed to boycott the radio personalities and the advertisers on their network of stations. The goal was formed to not just demand an apology but to make this a teachable moment by demanding that the parent company, Clear Channel, also air multiple PSAs. In addition, Clear Channel agreed to a 30-minute interview featuring Steve Perrin, the CEO of ALS-TDI. All of the boycott group’s demands were met.
This marks a major event in awareness for ALS. An organized grassroots movement was formed via social network tools and used those tools to achieve a goal long-sought in the community. This is only the beginning, and a perfect example of the kind of the cooperative relationship we need to foster with media, local and national.
As an Information Technology professional I tend to accumulate equipment. I have a couple of old laptops lying around (some dating back to 1998). One of the slightly newer ones I connected to my TV via the external SVGA port (with an associated stereo sound cable connected to the headphone jack). Now I can stream Netflix and other online content to my big flatscreen TV while not using up the resources on my communication system. For independence I installed VNC which gives me full control over the remote machine. All I need is someone to power on the laptop and I am up and running.
Currently I am watching live surfing being streamed from Bells Beach, Australia. Think I’ll crack a Foster’s and enjoy!
This is why we do it.
Olya in the Ukraine needed a biPAP which she couldn’t possibly afford. She contacted ALS Guardian Angels. I chat with the ALSGA coordinator quite frequently, heard the story, and donated my old biPAP. ALSGA shipped it to Poland and Olya’s friends got it to her from there.
Another PALS given more time alive by cooperation and action within the global PALS family. Although I appear in this story, it is not about me: It is about how we as PALS can change our own lives and the lives of each other. Without the new communications technologies now available (and the ability to use them) this could not have happened.
The following is an email trail following receipt of the biPAP and a technical issue resolved. Some of the discussions took place over real-time chat and so aren’t shown here. Total time from request to receipt and use was less than a month.
Subject: I want to say thank you
Date: Thu, 31 Mar 2011 00:19:27 +0400
Hello, Lisa! I can’t wait to share my happiness with you. Today I’ve used
the bipap for an hour or so for the first time and I felt excellent. Much
better than without it. I learned how to do settings. I find my mask to be
comfortable too. I feel deep relief now. I’m not scared any more not being
able to breath normally and sleepless nights are the past. I’ve attached a
picture of me while bipap therapy. Now I’m thinking back and understand
that is would not be possible without ALS GuardianAngels, Stu and your
priceless help. There were obstacles to ship the bipap and receive it, but
you were so patient and sympathetic to me and to all this process. I
sometimes can’t believe it’s true. When I tell people, that an American
organisation helped me with the bipap and tell them this story,they say:
“It’s something unbeliveble, it’s really hard to believe that miracles do
happen”. You can’t imagine how much your help means for me and my family.
I’m writing this and understand that I can’t find right words to express
how grateful I am.I also want to tell Eric (the donar) that his donation
means ‘”surviving” for one more PALS (for me). That this is more than a
gift! That I will always be grateful to him and never forget it. I want
to cry out THANK YOU FOR EVERYTHING YOU ARE DOING FOR PALS! IT’S
PRICELESS! Happy Olya 29.03.2011, 17:18, “ALS GuardianAngels”
<@.com>:YAY, I am so relieved! Here is the companies
Respironics Inc – 6 reviews – Place page
www.respironics.com – 1001 Murry Ridge Drive, Murrysville, PA – (724)
Subject: Re: Olya: Mission accomplished!
Date: Tue, 29 Mar 2011 15:09:30 +0400
Hello Lisa, It was voltage issue (the user guide info was right). My
husband bought another adapter and the bipap works with the new one.
Actually, at first we used an adapter too (an American one, as we have
American product and it does with the device), but the bipap didn’t work
with it, but Ukrainian adapter is good.Thank you, Lisa, for your help,
anyway. I was really worried. But I still need the contact of a provider
in case something goes wrong in future.Model ð 1003986PCA1SN:3074661My
phone number +38 095 700-68-67 29.03.2011, 02:25, “ALS GuardianAngels”
<@.com>;:Also, please send me the model number and
serial number…any other information would be great. A detailed reply
about what is happening will also be helpful. Send me your phone number
again as well.
Subject: RE: Olya: Mission accomplished!
Date: Mon, 28 Mar 2011 18:23:01 -0400
For now, bring it in to your doctor at your appointment…they may have
some advice. I’m hoping it’s not a voltage issue between US and Ukraine
products…we looked into that and we were certain it wouldn’t be an
issue. I can look for contact info, I already called them to see where
the device was originally made (needed that for Customs). I will call
them tomorrow as they are closed already today.
Subject: Re: Olya: Mission accomplished!
Date: Tue, 29 Mar 2011 01:59:47 +0400
Picture is attached. This is what I see on the screen, when trying start
the bipap. 29.03.2011, 01:07, “ALS GuardianAngels”
<@.com>;:Olya, here is the user guide:
me know if this helps or not. Otherwise, I can definitely ask the Donor
if he has any suggestions.
Subject: Re: Olya: Mission accomplished!
Date: Tue, 29 Mar 2011 00:51:09 +0400
Hello Lisa, We have just tried to start the bipap and failed. It does turn
on, but in several seconds long alarm sound and red solid and yewllow
solid turn on while system self test. I think we should adress the
provider, but we don’t have one. Could you advice what to do? 28.03.2011,
17:58, “ALS GuardianAngels” <@.com>;:I just received
this wonderful update form Olya! Also, since Olya wished to thank the
donor I included him on this message. Olya, his name is Eric Valor…and
he’s a wonderful man and PALS from Santa Cruz, California. Thank you
all, and Olya I look forward to more updates on how you are doing! –
Subject: Re: Need help to live with ALS
Date: Mon, 28 Mar 2011 16:53:01 +0400
Hello, Lisa! I have news. I’ve just received the bipap and the mask. I
didn’t try it yet. I’m going to see a doctor tomorrow and she should do
all the settings. But I’m very excited I have the bipap. I will do my best
to get accustomed to it. There is a nice surprise. When I opened the box,
I found a humidifier there.I can’t express how grateful I am to you and
Stu and the donor and to all the people who helped me to get it and of
course to God, who arranged my meeting ALS GuardianAngels. I’d love to
thank the donor who agreed to donate it to me. I hope you can deliver my
thanks to him. I am really very very excited. I can’t believe everything
is done.I will write you how I am doing with the bipap. Olya
In the past few months a group of ALS patients (PALS) and caregivers (CALS) have banded together via social media to support each other, spread awareness, educate themselves, and effect real change in their lives. This is more than blog posts and Facebook status updates but rather a concerted and united effort to fill some of the void in the research and treatment of their disease. Using email and messaging, individual projects have combined as a force-multiplier and are already beginning to see results. The ease of using and linking various social media technologies have allowed the infirm to reach out to and engage a global audience in a way and with an impact never before possible. They can do this because they are young, angry, and embrace technology to support and replace lost physical abilities. This movement represents a potential paradigm shift in the way ALS is perceived and dealt with.
The list of projects includes (but is certainly not limited to) my project to draft Hugh Laurie to be an ALS spokesman, Thomas Ohlson’s Petition to ALSA National, the remarkable and unprecedented ongoing serial documentary following Timothy Lafollette from diagnosis to current time, and the recent various and coordinated efforts to rescue a quadriplegic PALS from an abusive nursing home situation (in which this blog also participated).
There are many other projects with various strategies, but all with the goal of increasing awareness, research funding, and support efforts. Where in the past these efforts would be singular, isolated, and have very limited impact, with the use of social media (Facebook, Twitter, blogs, online forums, email and messaging/chat systems) they are now being coordinated and their message distributed all over North America. As the number and scope of projects increase, there can only be a positive effect. If you have or know of a project to increase ALS awareness, please hook up with us via social media!
I recently came across a video of a talk given by Dr. Hans Keirstead, whose work I have mentioned in a previous post. In the video, posted June 29, 2009 (almost exactly a year ago), Dr. Keirstead showcases his research on a stem cell therapy for SMA. He shows how he was able to grow functional motor neurons (proving it by showing them innervating muscle fibers) and explaining the then-current status of a proposed human trial to replace damaged tissue.
The proposed trial is for infants who suffer from Type 1 SMA. SMA (Spinal Muscular Atrophy) occurs from a malfunctioning SMN gene and the Type 1 (infantile) version is rapidly fatal. Like with a form of ALS called PMA (Progressive Muscular Atrophy), the motor neurons between the spinal cord and muscles die leaving the person paralyzed. When the diaphragm muscle is eventually denervated the person dies of respiratory failure. Regenerative medicine offers a way to replace lost tissue and restore function. In lab and animal tests it appears Dr. Keirstead has been successful. now for the next step.
There seem to be good reasons to try this in SMA infants. I am going to be coldly blunt but I beg the reader to take a breath and stay with me. First, the infants are going to die very soon so the trial length is short; you will see quick benefit or have rapid access to post-mortem tissue (also the reason Neuralstem included late-stage ALS patients in its trial). Because of the infants small size, the grafted neurons don’t have far to go to innervate muscle. In an adult such as myself motor neurons would have to grow a bit over a meter to reach fingers and toes. Even the phrenic nerve (the “money” nerve in neurodegenerative disease) would need to grow nearly half a meter to innervate my diaphragm muscle. Nerves grow slow so that could take as much as two years (for the more distal muscles). Infant bodies are still also in a rapid growth mode which may assist the grafts (mentioned in the Stanford study in a previous post). I could also guess that an immature immune system may be beneficial for anti-rejection purposes (pure speculation on my part). Time is in critically short supply in SMA (and ALS) so having short trial lengths is crucial. June 22, 2010 update on program progress.
Because SMA is so similar to ALS, if this trial goes well it would be huge news for PALS. In fact, ALS is the next disease in line for this treatment. With this program and the concurrent ongoing programs by Brainstorm, TCA, and Neuralstem either soon to be or currently in trial, I have great hope for regenerative medicine.
As always I invite responsible comments or questions.
In a previous post I made mention of the Neuralstem clinical trial currently ongoing at Emory University. Recently, CNN ran a story on the trial which provided welcome news to the ALS community. Three implantations had been performed with no complications so far.
Today I received a press release which carried forward the good news. Based on the results so far with the first three, they are going ahead with a fourth with double the injections (both sides of the spine). This is great news as it is showing the procedure and product to be safe (the primary objective of this trial). It is still too early to determine any efficacy and the trial isn’t designed to measure that anyway.
With each success the barriers are pushed back a little further. The success of the Neuralstem trial will pave the way for other trials.
A few days ago, Matt White, who lives with ALS, addressed the Butler NCAA basketball team prior to their Final Four victory over Michigan St. Normally I don’t care about basketball (I am a hockey fan) but a Facebook friend posted the Youtube link above and I viewed it. The thing that struck me about Mr. White’s address was his promotion of Sir Ernest Shackelton as a role model. If you don’t know who he is or don’t know his story, please take a moment to read the previous link (most notably the portion on the Trans-Antarctic Expedition).
Sir Ernest is a perfect role model for PALS because of his dogged determination, his cool ability to make rational choices in grim situations, and his use of those characteristics to survive a lethal predicament (multiple predicaments, actually). Not only did he survive but he ensured his men would as well. Contrast the experiences of Scott and, worse, Franklin. Even though Shackelton was forced to abandon his original goal, he achieved an even greater goal by surviving.
I would like to thank Mr. White for including Sir Ernest in his address and would like to concur. Even though PALS may be forced to abandon their goals, it is my hope that many can join me in “pulling a Shackelton” and doing what is necessary to survive an impossible situation.