A View of ALS from Behind the Camera
My name is Mikaelyn Austin. My business partner Rob Marcolina and I run Philly Philms, an independent video production company based out of Montgomery County. For the past year, we have been incredibly fortunate and honored to produce quite a bit of video content for the national ALS Association as well as the ALS Association Greater Philadelphia Chapter. This work has also helped immensely in the development of our independent documentary project WHEN THE ICE MELTED — ALS and the Real Challenge Beyond the Bucket (visit www.WhenTheIceMelted.com for more information).
Our most recent film shoot took us to Lehigh Valley Health Network following Karen Delaney Shideleff (Greater Philadelphia Chapter Patient/Advocate) in her clinical rounds visit. We filmed and quietly observed as Karen and her husband Bob spoke with PT’s, Nurses, Patient Services Reps from the Chapter, and of course, her Doctor. What made this particular shoot so memorable was a simple test that many of us would, quite frankly, take for granted.
You see, Karen’s family is one of the largest known cases of Familial ALS in the world, which basically means ALS runs in her family…to the extent of over 30 known cases. Karen has literally known since the age of 20 years old that she had a 50/50 chance of one day being diagnosed with ALS. Just think about that for a second. What were YOU thinking about at 20 years old? At 20, I was a junior in College at the University of Pennsylvania and most of my thoughts were consumed by homework, studying and not being late to basketball practice. Needless to say, very few 20 year olds are forced to think about their own possible fate at the hands of such a terrible disease.
So this particular shoot with Karen at Lehigh Valley Health Network will remain engrained in my brain long after we are done producing this documentary. For the past couple of months, Karen has been telling us about a very promising study at Johns Hopkins in Maryland called the “Antisense Oligonucleotide,” which is a trial drug that aims to block the production of a mutant protein that causes an inherited form of ALS (Familial ALS). In order to be eligible to partake in this trial, Karen needs to score over 50% in her Forced Vital Capacity (FVC) test, which measures the maximum amount of air a person can expel from their lungs after a maximum inhalation. To put this into perspective, an average healthy adult has a FVC of over 80%. For Karen, ALS is increasingly restricting her lungs from filling to their normal capacity of air. Scoring below 60% is considered “severely” abnormal, but on this day for Karen Delaney Shideleff, scoring a 53% was a huge victory. We watched and filmed as Karen fought to expel every bit of air from her lungs, a function that many of us take for granted each and every day. If she can repeat this performance when the trial opens soon for enrollment, she will be able to participate.
Will participating in this trial cure Karen’s ALS? No, not likely. What many people don’t realize is that even if a trial drug shows extreme promise in testing, it takes at least a billion dollars and as long as 10 years to bring that drug to market. The ALS Ice Bucket Challenge raised $220 million. The average lifespan of an ALS patient is 2 to 5 years. Karen was diagnosed with ALS in 2011 and is currently (as I type) on a trip with two close friends and one of her sisters that she is calling her “F&$% You ALS 5-Year Anniversary Trip.” Karen is desperate to repeat her FVC performance and qualify for the trial at Johns Hopkins because it brings her one step closer to insuring her sisters would have a fighting chance of avoiding her fate should any of them be diagnosed with ALS.
Since we began working on this documentary, I have found myself wondering almost every day how I would handle living with ALS. I wish I could say with confidence that I would be even half as positive, upbeat and determined as Karen has been, but I sincerely doubt I would. In the nearly 4 years we have known and been privileged enough to film with Karen, she has not just inspired us, she has taught us. Taught us to live, taught us to love, and taught us to laugh. As she always says (quoting Abraham Lincoln), “In the end, it’s not the years in your life that count. It’s the life in your years.”