Doggone It!

As my readers might know, I have a PEG tube for feeding. I also have a little dog who is more like a 5-year-old in a dog suit. She is quite devious, even having learned how to stomp the foot lever that opens the lid on the trash can. For the past few months, ever since I stopped using medical formula to correct the diabetic condition it created, I have been giving her a few tablespoons of my meals. Last Friday, I was working on some email while lunch flowed into me. Out of the corner of my eye I noticed some movement and realized it was the rolling stand from which the feeding bag was suspended. I didn't feel the earthquake that must be responsible for the stand rocking back and forth and nothing else seemed to be moving (especially my computer which is suspended from the ceiling). A quick mental inventory revealed I wasn't on any drugs that I was aware of. Motor neuron disease doesn't involve hallucinations. I didn't feel possessed... Suddenly I felt a tug on the feeding tube and heard the tell-tale clicking of canine toenails on hardwood. That little thieving pooch was chewing the tube trying to get more of the clam chowder I had shared 30 minutes prior! I swear she knew that I couldn't do anything to stop her. But I also knew that, being 17 years old, she is a little hard of hearing. So rather than ring my call bell which she can hear and knows means trouble for her, I made my computer call in my caregiver on the sly. Busted doggie!

PEACaboo

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 a Residence in his/her local community.

Chair the Love

Wondering about options for wheelchair-modified vans? My friend over at ALS Everyday Living has a very informative post on the subject. I urge my readers to hop over there and sign up for his updates.

Assaultrocytes

In my post Prion, Garth! I had talked about mutant or misfolded SOD1 (mSOD1) spreading like a prion and infecting neighboring cells. A recently released study from Johns Hopkins shows for the first time in vivo that astrocytes expressing mutant mSOD1 damage the motor neurons they are supposed to protect. In the study, the authors transplanted SOD1G93A glial-restricted precursor cells—glial progenitors capable of differentiating into astrocytes—into the cervical spinal cord of rats to reveal how mutant astrocytes influence WT motor neurons and other cells types (microglia and astrocytes) in an in vivo setting. The G93A mutation of SOD1 is the most studied version of that particular genetic mutation responsible for many inherited (or familial) ALS cases.

This is the strongest evidence to date that "assaultrocytes" are primary culprits in ALS. It also further suggests that even some sporadic ALS can be caused by misfolded SOD1 which escapes proteasomal degradation and gets into the extracellular space. It also suggests that targeting mSOD1 and stem cell implantation of astrocytes can be viable treatment methods.

EDIT: Here is a more in-depth article by Amber Dance at the ALZ Forum.

Headlines

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.

Prion, Garth

in a previous post, I had discussed the possibility of misfolded SOD1 aggregating with TDP43 resulting in depletion of TDP43 from the nucleus which caused disruption of cellular processes and cell death. Further, misfolded SOD1 induces glial activation seen in ALS which poisons otherwise healthy neurons. A new study explores the mechanism behind this prion-like behavior, and further indicates that extracellular misfolded SOD1 can be a cause of even sporadic ALS. It also points to a molecular target which could halt progression cold. The study was done at the Brain Research Centre based at the University of British Columbia and the Vancouver Coastal Health Research Institute, in collaboration with researchers at the University of Alberta. The research was supported in part by Amorfix Life Sciences which has a "vaccine" against misfolded SOD1 already in development.

Now Is The Time

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. Bill Saling Immediate Attention- 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 kherron@sas-ga.org - Please copy Bill Saling at b.saling@yahoo.com