06/20/13: Taking on ALS

This op-ed appeared in The Virginian-Pilot on the date shown.

THIRTY thousand.

That’s roughly how many Americans died in traffic crashes in 2010, the latest figures available from the National Highway Traffic Safety Administration. The numbers used to be higher: In 1982, more than 40,000 died in traffic crashes. A coordinated effort by automakers, legislators and the public can be credited with the decrease.

Thirty thousand.

That’s roughly how many Americans are living with amyotrophic lateral sclerosis, better known as ALS or Lou Gehrig’s Disease, according to the Centers for Disease Control and Prevention.

The ALS Association describes it as “a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.” As the nerve cells die, the brain can no longer send messages to the muscles, which leads to atrophy.

The disease crosses all racial, ethnic and socioeconomic demographics. Approximately 7 percent of those who suffer from ALS have a record of the disease in their family. For the other 93 percent, it occurs sporadically, meaning there is no trace in family history.

Ten thousand.

That’s roughly how many Americans were killed in alcohol-related crashes in 2010. The number is less than half of what it was in 1982. Such is the success of groups like Mothers Against Drunk Driving, which urged legislators to increase penalties for drunken driving and helped to raise awareness.

Five thousand six hundred.

That’s roughly how many Americans are diagnosed with ALS each year. Yet it’s considered an “orphan disease,” one that affects a small percentage of the population and, generally, draws a small percentage of research dollars.

Experts bristle at this characterization because for nearly every new person diagnosed with ALS, someone living with it dies. Fifty percent of ALS patients live three or more years after diagnosis, 20 percent live five or more years and just 10 percent live 10 or more years.

Because of the high death rate, the disease is often misunderstood. Such was the case this week when former New Orleans Saints safety Steve Gleason, an ALS patient, was mocked on a Georgia radio station.

Gleason was diagnosed in early 2011. As is true with most such patients, his muscles have atrophied but his cognitive abilities remain intact. He used eye-tracking technology to crank out a 4,500-word column for Sports Illustrated that was posted Monday. It took him about four hours — of gazing and blinking — to put the column together.

Three sports radio hosts thought this was funny — and made tasteless jokes, poking fun at Gleason and the disease. They were fired Tuesday.

Three.

That’s the number of people I personally know who are living with ALS. The first was diagnosed in October 1994, the most recent in December 2011.

A presentation by the husband of the most recent person prompted me to contact the wife of the first. I wanted to discuss with my dear friend of 40 years — she was my math teacher in the 8th grade — her thoughts on an exciting breakthrough.

It seems researchers have identified a gene mutation, called C9orf72, that affects about 40 percent of familial ALS patients and about 10 percent of the much larger group of sporadic patients. The possibility of a treatment is coming along quickly, mainly due to more than a decade of research into myotonic muscular dystrophy, which shares the same type of mutation.

Identifying ALS patients with the C9orf72 mutation will be a major effort, and at the forefront is Norfolk’s Steve Kolb, the C9 ID project coordinator and the husband of Virginia, the most recent person I know diagnosed with the disease.

Kolb and others believe that drug trials for those with the gene mutation are just a year away.

Living with the disease long term takes its toll, as my conversation with my friend and former teacher revealed. Far too many times, she and her husband’s hopes were raised, then promising research failed to deliver. A promising drug, Myotophin, was pulled by the FDA. After one of his doctors left the profession — and after witnessing far too many deaths — my friend and her husband withdrew from active participation in the ALS community about 10 years ago.

I wasn’t the first to share this breakthrough with her; others had done so. She had paid no attention, though, fearing another letdown. But by the time we got off the phone, I heard cautious optimism in her voice, a renewed sense of hope.

Over the years, we’ve spent a lot of money to reduce the number of traffic deaths. With a nearly equal number of Americans living with ALS, I hope we are willing to spend an equal amount conquering this disease.

For more information about the C9 ID project, visit www. facebook.com/C9IDNOW