Thursday, March 18, 2010

Loving the Weather and Research Update - Living with Spinal Muscular Atrophy

                                     Lizzy is drainage Position while at the motel in Madison


Sorry, its been over a week since updated. We are finally enjoying this warmer weather. Lizzy and I walked home from my hair appt on Tuesday evening. It was great breathing in that brisk air. She loved it. Now, the time change kicked my hiney and I am not sure why but I was dragging until today. Its been kind of strange around here this week with my phone lines being messed up and a few other issues.

Elizabeth is now using her "Easy Cat " mouse while in her stander. She was upset because she was refusing getting in the stander because she could not do her lap top all on her own. So, I rigged her stander up with a 2x4 block and packing tape  with her Easy Cat Mouse and it works!!

We are hoping the weather stays warmer!! I hope Spring is here to stay. Lizzy has been doing well and NEVER stops surprising me. She is such a great kid. I feel so honored to have her in my life. As, I have said before she is my heart.

Now,  for the latest and greatest SMA RESEARCH!!

A good friend of ours Vince Gaynor( Sophia's Cure)  posted this yesterday and I wanted to share it. He is such a good Daddy. He made it easier for me doing this so I am sending it to you on SMA RESEARCH!!
I feel this is the year the research is so promising. I see great things happening!!
Thanks Vince for doing this!!! Saved me a lot of time.

A few new articles on promising research

Posted by Vincent Gaynor at 7:35pm yesterday
Hi everyone. I wanted to post some information and recent articles that have come out. I wanted everyone to know I am just an SMA parent sharing information on research that I have become aware of. The information I post about is from direct conversations I have with the researchers involved in the studies as well as experts from many different organizations that have been involved with sma research for many years. I can only refer to the comments relayed to me and pass along any information I know. I am not a researcher myself, but for what it's worth, I am also not a nurse, pt, ot, sp, nutrionist, pulmonologist either yet I am teaching local doctors about SMA Type 1 and my daughter Sophia is thriving! I want to make it absolutely clear that NONE of these programs will benefit the majority of our children in the immediate future, however I believe these programs hold the most promise moving forward and give our community as a whole the best chance of one day ending this disease moving forward. On my next post I will be touching on restricted funding to maximize the benefit of your hard earned research dollars, but for this bulletin I want to highlight some of the recent articles published and discuss some of their benefits and questions that remain about each program.

Lets start with the Quinazoline Program here is an article posted on this promising therapy:
http://www.ricercasma.it/index.php?op...

The Quinazoline program in theory will upregulate the SMN 2. It is a very promising therapy designed specifically for sma. The hope is that the triggering mechanism will increase the smn 2 expression by a certain percentage. Thus improvements from this therapy will hopefully be seen in patients with a larger amount of "backup" SMN 2 copies. The question with this program is what benefits, if any, will children with fewer copies see.

The next program is the Motor Neuron Replacement Therapy. Based on timeline we have felt this was the most promising therapy for the most immediate impact. Here is a recent article on Stem Cells:
http://www.cbsnews.com/stories/2010/0...

The MNRP is an exciting and very promising program. The studies done in this experiment have showed a dramatic improvement in mice with spinal cord injuries. The administration of the motor neurons will be through a surgery similar to spinal fusion. The hope is that this treatment will replace the motor neurons that are defective in patients. The questions with this therapy are do motor neurons die or do they just remain dormant once there is an absence of SMN. This is a very important question since research suggests that stem cells seem to act as a crutch to injured stem cells rather than replacing ones that have died. Another question is can the results seen in a mouse model be replicated in a much larger model such as a primate. The length of the pathways that must be regenerated are much shorter in a mouse model.

Our third program is the Gene Therapy Program being run at OSU/ Nationwide here is a recent article:
http://researchnews.osu.edu/archive/s...

This is a wonderful and exciting program as well. This program has shown the ability to actually cure the most severe animal model of SMA. The exciting news is that these results are showing the same translation in a much larger animal model. This program has currently moved into primates and is seeing some amazing results. This program is a gene replacement of SMN 1. Based on virus, and delivery approach this program seems to be easiest to gain FDA approval towards human clinical trials. The virus used to enter the motor neurons is similar to a common cold with the harmful side effects being removed. Because this virus can cross the blood brain barrier it can be injected like an immunization shot. The big question that remains with this program is what is the window of treatment that patients can continue to see results from this therapy. Will it be only the earliest patients treated in the very first days or will we be able to see results in older patients as well.

Here is another promising Gene Therapy Program being funded by Genzyme:
http://www.jci.org/articles/view/4161...

This program has shown some amazing results similar to the work being done at OSU. The same questions remain for this program as well. The big difference is the virus used and the delivery approach. This treatment is using a virus that must be administered through the scull. Will the FDA be more inclined to approve a therapy with a more drastic delivery approach.

Finally here is an exciting article on research being done at University of Pennsylvania using Amino Acids:
http://www.hhmi.org/news/dreyfuss2010...

This program is using amino acids to prevent degredation of SMN 2. The good news is that this research is being fully funded by Merck. Because Merck is funding this research there is not a lot of information being released publicly on this program. This program is in its infancy and is years away from applying for human clinical trials. They plan to test on an sma mice model in the future.

I hope this has been of some assistance to help realize the research that is currently being done so you can make a more informed choice on where you would like your research dollars to go.


Many prayers to our friend Drew!! Recover Quickly!!

"We believe in miracles because we live with one"
www.our-sma-angels.com/elizabeth 

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