Two new pieces of information have emerged from researchers working to find the cause, therapies and eventually a cure for ALS. I’ve edited both into a readers digest version. These findings are the most promising I’ve seen yet.

#1: Researchers found that stem cell-derived motor neurons are killed by the glial cells that are supposed to serve them. The glial cells that normally surround and nourish motor neurons proved lethal if they had the mutation linked to some inherited forms of ALS.   The scientists took stem cells from mice with a normal human gene and also mice with a protein defect that produces the disease. The study demonstrated that glial cells carrying an ALS-linked mutation have a direct effect on motor neuron survival and provide a powerful tool for studying the mechanisms of neural degeneration. The scientists believe the stem cell derived system “could provide cell-based positive results for the identification of new ALS drugs.” The motor neurons from stem cells of mice with the mutant protein show, in culture, the hallmarks of the disease at the cellular level. They have abnormal protein deposits, consisting of the mutant protein, and increased amounts of the molecular tag that cells use to signal that damaged protein needs to be removed. Also, the motor neurons from mutant mouse stem cells show activation of the cell death pathway, another parallel to what takes place in the disease. It’s reported that the mutant glia are secreting a substance that is toxic only to the motor neurons and not to other cells. Future investigation with the new strategies developed by these research groups will undoubtedly produce important progress in the search for therapeutics to treat ALS effectively.

#2: Researchers have added to a growing body of evidence that implicates the immune system in ALS. Researchers found that mouse motor neurons carrying mutated genes known to cause ALS make proteins that are part of the immune system’s “classic complement pathway.” These proteins then signal neighboring immune-system cells called microglia, which may attack and kill the motor neurons. The data imply that therapies that interfere with the complement pathway or with microglial activation would likely be beneficial in ALS. Although it isn’t known whether these findings apply to nongenetic forms of ALS, there is evidence to suggest that these two major forms of the disease differ only at the very beginning and quickly converge.

Both sets of findings have important implications if stem cell therapies are to be attempted to treat ALS.

“It’s now clear that in inherited ALS the neurons are genetically damaged and start making proteins normally made by immune cells. But ALS is not just a disease of motor neurons. Neighboring cells are key to disease progression. These non-neuronal cells initially respond to the injured neurons, but when they’re damaged themselves, they turn them into clumsy, unfriendly neighbors whose action actually accelerates disease progression. One implication of this is that stem cell therapies to replace the damaged neighbors may be a very effective approach for slowing disease progression.

There is Hope!  

The issue of unanswered prayers came up after Linda’s mother passed away. Lexy asked with so many prayers for her grand mother to get well why she died. With my ALS continuing to progress the question has arisen of why God has not intervened, as many prayers across the globe have asked Him to do so. After Lexy asked her question I sent an email to four Church of Christ preachers for their understanding of unanswered prayers. I thought most would be interested in their response. I will post one response each month. The first is below. It’s from the preacher at the church Linda’s mom attended.

Response # One:

The true answer is “God’s will is always better than ours.”  But still, we pray that “our will” be done.  Jesus tells us that God knows what we need before we even ask Him (Mt 6:8) but still the Lord knows that we “need” to ask Him (pray).  Jesus prayed in the Garden of Gethsemane that the Father “remove the cup” from Him (death on a cross) but knew that God’s perfect will might mean that He might have to die on the cross so He closed His prayer with “your will be done.” 

You see we just don’t know God’s will so we continue to pray.  All of us will one day die and transition from “life” on earth to everlasting “life” (as Ursula did – Heb 9:27).  God blessed her with a long, happy life (Ps 90:10). 

Did God hear the prayers of the family and friends?  Oh yes!  Did He answer our prayers?  Oh yes!  His answer was: “I know you love your mother, grandmother, friend … and so do I … but there is so very much you don’t know and I do.  It is time for my daughter to come home.”   

I’ll post another next month.

After our shopping adventure, we headed to a nice visit with my oldest sister and her two sons. It was a great time hanging out with them. Salem/Roanoke is a neat part of our country with its history, landscape and generally easygoing folks.  

Its was a short visit as we needed to return home to attend Dani’s first Ti Kwon Do tournament. On Saturday morning we headed out to a high school gym about an hour north of us. Luckily there was a Starbucks along the way. Well, was I surprised. There were six sparing mats (read rings) and hordes of kids. The place was active the entire day with the several hundred kids competing for bronze to gold metals. I did some quick math in my head. If this was a conversation my wife would quip dryly if this was painful. Based on the entrance fees alone the promoting of these events is a pretty big business. This is way more serious and sophisticated then I imagined.  

We signed in and then we waited and waited…at 3:00pm Dani’s match began. Apparently there are not many beginners (white belt) at Dani’s age who do tournaments. There was only one other girl her age close enough in ability (yellow belt) for her to compete with. Even though it was a lot of effort for one round, it was good for a first time out as she got a lay of the land for next time and hey, she was guaranteed to win a metal.  

Her sparring partner was taller and one belt higher then her, both causes for concern. When the match started Dani’s opponent came out kicking, seemingly without thought. Dani was clearly surprised by the barrage, but smartly leaned into her opponent negating any force and contact of her blows. More importantly no points were being scored. As the kicks were going awry some landed on Dani’s ankle and after a minute Dani was limping in pain. The referee stop the match, Dani was attended to and back in the ring ready to continue quickly. I don’t know what was said to Dani but forty five seconds after the second start Dani had knocked her opponent down, scored seven points and the matched called in her favor. Her opponent walked off the mat crying and bleeding. It happened so quick we stood there a little stunned and confused.  

Dani collected her Gold metal. As we where walking out I asked Dani what happened. She looked up at me with the eyes of a worrier and said; she made me mad.  

The boys are in deep trouble.