Edaravone is believed to act as a free radical scavenger, a compound that works by getting rid of toxic waste generated as a normal by-product the cells function. In ALS it is thought that these by-products are not as effectively removed and the compound may be neuroprotective by relieving the effects of this oxidative stress. The increase in oxidative stress is thought to damage motor neurons (cells that die in ALS).
During ALS, the brain and spinal cord are damaged by inflammation, called neuro-inflammation. Inflammation is an immune defense system that works when tissue is damaged. Outside the central nervous system (CNS), it is characterized by redness, tenderness and swelling, as chemicals and immune cells converge to clean up the damage.
My parents and I live very far apart, but we visit as often as possible. Earlier in my disease, our reunions were more normal: catching up, visiting favorite restaurants, hanging out at the bookstore. We did the things we have always done when we got together. Now, one year after being diagnosed and two years into this disease, it’s getting harder to forget I am sick.
Dr. Andrew Geronimo is a talented young investigator using brain computer interface (BCI) technology to improve the lives of people living with ALS by enhancing their ability to communicate. He and his mentor Dr. Zachary Simmons, also at Penn State Hershey Medical Center, have received a grant from The ALS Association to develop new opportunities for BCI technology.
I put it off as long as I could. I buried myself in friends’ lives, picked at frivolous problems and attended an excessive amount of events. I traveled for three weeks straight. I caught up on every T.V. show I’ve ever enjoyed. I cleaned and organized my entire home. And I sorted through every piece of clothing my children own, which took a full three days in and of itself.
Stress granules are clumps of RNA and protein that accumulate in a cell when it experiences stress. This accumulation allows space for specialized RNA-binding proteins that are designed to clean up the cell. Once the cell is cleaned up, the accumulated RNA and protein disperse out of the stress granule, and the cell goes back to continuing its normal function.
Jeff passed away in 2006, after battling for three years. It was a quick ending to a long journey; my family saw him three weeks earlier, then he was gone. Looking back at it almost 10 years later is still difficult, no one likes to think of their loved ones dying, but also it’s been a time to be thankful ALS came into my life.
It was almost a year ago that I fell in love with Jane Babin’s poetry. Her son, a former student of mine, had given me her book Pearls in the Pond a few months after she had passed away after an eleven year battle with ALS (Lou Gehrig’s disease). I found myself immersed in her poems, lectures, thoughts on life and mortality, and coping with her disease.
All of us living with ALS have to have hope: hope for a treatment and hope for a cure. That’s why I’m excited about a new report that came out today from the Pharmaceutical Research and Manufacturers of America (PhRMA) and The ALS Association, “Medicines in Development for Rare Diseases.”
Meet Dr. James Connor, an ALS researcher who leads a team at Penn State Hershey Medical Center in Hershey, PA that recently received an ALS Association Investigator-Initiated grant to move his exciting research forward. The ALS Association spoke with Dr. Connor about his investigations into how a therapeutic iron solution is potentially protective in ALS and how collaboration at Hershey bridges the lab and the ALS clinic.