Back in the late 1990s, Scott Mackler spent his very full days caring for patients, conducting research, lecturing medical students and teaching residents at Penn’s School of Medicine. He was also a devoted husband to Lynn Snyder-Mackler PT’80 G’84 (currently a professor in the Department of Physical Therapy at the University of Delaware) and a hands-on father to their two sons Alexander Snyder-Mackler C’05 and Noah Snyder-Mackler C’07, now pursuing his doctorate at Penn. And he was a serious athlete—serious enough to run the Boston Marathon twice during his grueling, 100 hour/week residency training and to continue to make time, regardless of the demands of his practice and research, to run about six miles or to play tennis every day.
Then—it was in December of 1998, he recalls—he noticed that his tennis racquet had begun to slip out of his hand. His running times started to slow, and he started to experience involuntary contractions of the muscles in his right forearm. Also, he couldn’t tell a joke without laughing before the punchline.
Mackler knew that the twitching, loss of hand strength, and inability to control his outward emotions could be early indications of ALS. But to his surprise, the first neurologist he consulted dismissed his concerns. Frustrated, Mackler sought a second opinion and was tested for other diseases, including multiple sclerosis. Those tests were all negative, even as new symptoms appeared.
Mackler began slurring his words. At first, he could still keep that behavior in check if he concentrated on speaking slowly, but he could no longer disguise his inability to keep up with his lunchtime running buddies. He was sure he had ALS, regardless of what anyone else said—or didn’t say. “Even as my symptoms progressed, everyone continued to suggest other possibilities,” Mackler explains in the computer-generated voice that narrates the lecture, “The Physician as Patient,” that he now presents to first-year medical students. “Denial may be a wonderful ego-defense mechanism, but I was not able to enjoy it.”
Harvey Mackler W’75 doubts that denial would have ever have been part of his younger brother’s coping strategy. “My brother is a control freak with a fierce will to live,” he says. “Yet, he has always accepted the hand that has been dealt him.” According to Harvey, when Scott was definitively diagnosed, in May 1999, he read Elizabeth Kubler-Ross’ famous book, On Death and Dying, about the five stages of dealing with grief or tragedy—only he skipped over denial, anger, bargaining, and depression and went straight to acceptance. “He told me, ‘It is what it is,’ and he never looked back,” Harvey recalls.
What Scott accepted with such rare grace was the fact that a neurodegenerative disease would methodically paralyze the muscles of his limbs and trunk, as well as the ones that controlled his speech, his ability to swallow, and even his breathing—while leaving his senses, brain, and internal organs intact. A disease that struck for no known reason, was without cure, and which would inevitably progress, though according to no consistent timetable.
The ALS Association, an advocacy group, estimates that about 30,000 Americans have ALS. Some 5,600 people are diagnosed each year, which works out to 15 or so new cases per day. The average life expectancy is three to five years after diagnosis, although 10 percent live for more than a decade. Onset is generally between the ages of 40 and 70. Victims are somewhat more likely to be men (60 percent) and are overwhelmingly white (93 percent).
For Mackler, the thought of a life in which he would not be able to communicate or retain some sense of independence was even more devastating than living with the physical limitations that were equally unavoidable. Fully aware of what his future would hold and convinced he would not want to live on a ventilator, nine years ago he recorded a video for his two sons, which was broadcast as part of a 60 Minutes feature on him that first aired in November 2008.
In the video, Mackler (wearing a Penn T-shirt) takes a break from a run in the woods to basically say farewell to the boys he did not imagine he would see grow to adulthood. “I know the future holds lots of love and joy and pride and that life goes on,” he says to the camera. “I’ll be watching you along the way and I love you very much and I’ll see ya.”
Not long after Mackler recorded that video, he and his family took off to travel the world. By the end of 1999, when they had reached South Africa, Mackler needed a wheelchair to get around. “In my last two years of high school, he went from beating everyone running, to walking with arm crutches for a brief period of time, to eventually using a scooter, and then a wheelchair,” Alex recounted in a story that appeared in Philadelphia Weekly in 2005.
The progression of the disease was typical, according to Lynn. “Most patients are ventilator-dependent in two years,” she says. Far less typical has been the way that Scott, aided by technology and an extraordinary human support network, has continued to function professionally and personally. At the time of his diagnosis, no one, least of all Scott himself, imagined a future in which he would still be lecturing (via a computer-generated voice) five years later, or continuing to work and direct his laboratory another five years after that.
What has made the difference, Mackler told CBS correspondent Scott Pelley in the 60 Minutes interview, is the ability to communicate. Prior to the BCI system that he currently uses, Mackler had employed various eye-gazing techniques, in which users visually focus on letters or symbols on a computer screen, to communicate. He had also developed a family shorthand that is sometimes quicker than the computer—for those accustomed to using it. As his sister, Randi Mackler Windheim C’77, explains, “He moves his eyeball to look at you when you give him the correct letter.” The alphabet is divided into thirds, and the object is to break the word down, letter by letter, by naming each one until Scott approves the selection. Alex and Noah are particularly adept at flying through the alphabet and determining when their father is trying to talk. “Mom,” Alex reprimanded his mother when Lynn answered one of my questions for her husband. “Dad is talking.”
July|Aug 09 contents
A Life Worth Llving By Kathryn Levy Feldman
Photography by Chris Crisman C’03
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Family Portrait: Scott Mackler with sons Alex (left) and Noah and wife Lynn at home.