Living life, one letter at a time

Scott Mackler is trapped.

It’s a Wednesday afternoon, and Scott is putting the final touches on a grant proposal — the periodic, cymbal-like sputtering of his ventilator cutting through the silence of an otherwise empty room.

Sitting back about 45 degrees in a wheelchair with his hands folded neatly across his lap, Scott is where he has worked for the past decade: in his ground floor lab in the Perelman School of Medicine.

I’m joking around with Scott’s nurse for the day, Adam Czerwinski, when we realize that Scott is trying to tell us something.

Adam leans up against Scott’s electric chair, his elbows pressing into the machine’s soft padding. His eyes no more than a foot away from Scott’s, he begins to rattle off a series of questions.

“First, second or third? Is it first?”

Scott’s eyes shift slowly toward us — a sign that we have the right answer.

“It is first.”

Then, we begin to go through the first third of the alphabet.

“A, B, C, D, E, F,” Adam says in a low, methodical voice.

Once again, Scott’s eyes move in our direction. We’ve arrived at the correct letter: “F.”

Based on the fact that it’s noon — right around lunchtime for Scott — we’re able to deduce fairly quickly that he’s trying to spell out “food.”

Coming up with the word takes us about 13 seconds.

Scott hasn’t been able to speak for 13 years.

***

Scott, a 54-year-old professor in the Medical School, was diagnosed with Amyotrophic Lateral Sclerosis — more commonly known as Lou Gehrig’s disease — in 1999.

While some ALS patients retain basic movement or speech functions as the disease progresses, Scott’s wife, Lynn Snyder-Mackler, describes her husband’s state as a “worst-case scenario.”

Scott is essentially “locked in” — his mind is as brilliant as ever, but he’s unable to convey any of that brilliance to his friends or family by himself.

Other than his slight eye movements and an occasional ability to crack a smile, Scott can’t move a single part of his body, forever confined to his chair unless helped by somebody else.

He can’t breathe without a ventilator, he can’t eat without a feeding tube and he can’t speak without one of his nurses running through the alphabet with him — a tiring, painstaking task to complete dozens of times every day.

Scott is among 30,000 people nationwide who have ALS, according to the ALS Association. There is no known cure for the disease.

“His mind is so superior to all of ours,” says his older brother, 1975 Wharton graduate Harvey Mackler. “His mind knows exactly what he wants to say, but he’s trapped — a prisoner inside his own body. If that was me, I would’ve checked out a long time ago.”

While the vast majority of ALS patients don’t live for more than five years after their diagnosis, giving up on life is the furthest thing from Scott’s mind.

From Monday to Thursday every week, Scott still makes the hour-long trip from his Newark, Del., home to Penn’s campus, continuing to direct his lab on the ground floor of the John Morgan Building.

Although he’s no longer able to lecture or work with patients, he’s still on the cutting edge of drug addiction research, one eye movement at a time.

Recently, his team found that NAC-1 — a protein in the brain that Scott discovered in 1996 as part of his addiction studies — may play a role in the cause of ALS.

He continues to research NAC-1 today.

I ask him over email why, despite all of the challenges, he keeps on going, day after day.

“I have no desire to slow down,” he replies, dictating his responses to Lynn through his usual eye-shift method. “I am a scientist. I have so many ideas and want to see this work continue and even explore new areas. I have fulfilling work.”

***

We’re sitting in Scott’s office again, just finishing up lunch.

Scott is wearing a blue-striped shirt that is neatly tucked into a pair of black pants. His hair has all but finished the graying process, a sign that he’s slowly approaching 60.

A dense white cloth supports the back of his head on the chair, while a long blue tube is protruding from his neck, expanding in and out with every breath he takes.

Adam and I are playing around with Scott, poking fun at his favorite baseball team: the New York Mets.

Scott smiles.

It’s little more than a faint curl of the end of his lips, but it’s a powerful moment — a true register of emotion without Scott having to worry about moving his eyes.

We’re getting ready to take a walk outside, but Scott wants to send one last email before we go.

Like he’s done thousands of times before, Adam begins his routine to determine what Scott is trying to say.

He starts by asking who the email should be sent to.

“First, second or third? Is it third?”

To communicate, Scott divides the alphabet into thirds: A-H, I-Q and R-Z. Once you’ve landed on the correct third — which you’re able to determine once he looks in your direction — you begin to go through each individual letter one by one, until he looks at you again.

Guessing a word based on context can sometimes speed up the process, but Scott has made it clear over the years that too much guessing bothers him.

The process is far from foolproof. Sometimes, Scott’s eyes — barely even slits — will “freeze up,” making it exceedingly difficult for him to look in one direction or the other. Other times, you’ll come up with an entire string of words, only to discover that you have to start over after Scott spells out “wrong.”

But it’s the best system Scott has.

It takes Adam about 10 minutes to get three letters from Scott — S, E and T — when I suddenly realize whose name he might be trying to spell.

“Is it Seth?” I ask, somewhat amazed and embarrassed by the prospect that it could have taken me this long to recognize my own name.

Scott shifts his eyes to look straight at me — a clear yes.

It turns out that Scott had wanted to send me a copy of some PowerPoint slides I’d requested from him a day earlier.

If it wasn’t for Scott, I probably would’ve forgotten about the slides.

“I forget things all the time, but Scott, what an incredible memory,” Adam says. “To do what he still does in his condition has given me a whole new outlook on life. I think it gives us all a new outlook.”

***

Scott first began to suspect that he had ALS near the end of 1998, when one day he was playing tennis and had trouble maintaining a firm grip on his racket.

Soon after, he began slurring his speech, finding it increasingly difficult to form sentences in the way he wanted.

His inability to control bouts of laughter, his involuntary muscle contractions — they were all signs of the onset of ALS.

In his younger days, Scott was the definition of active.

A man who could once run a mile at a 4:47 clip, Scott played on Penn’s soccer team during his first few years as an undergraduate at the University in the 1970s. When his two sons, Alexander and Noah — both Penn graduates as well — were growing up, he coached their soccer teams, never missing a weekend game.

“He was their super dad,” Lynn says.

Although Scott was formally diagnosed midway through 1999, he and Lynn had been almost certain for some time that he had ALS.

“ALS is a bad diagnosis, so doctors avoid it,” says Lynn, who is a professor in the University of Delaware’s Department of Physical Therapy. “It’s a diagnosis of exclusion.”

After the diagnosis became real, Lynn and Scott faced one of their toughest challenges yet: telling their friends and family.

“I don’t think any of us will ever forget the email that broke the news,” says Julie Blendy, a professor of pharmacology at Penn and a friend of Scott’s. “I remember it was long, and I remember having to read it at least three or four times for it to really sink in. It was heartbreaking.”

A few days earlier, Julie remembers, Scott had missed the cocktail hour of a reception at which he had been scheduled to give a speech. When he arrived an hour later, his words were a bit slurred — an odd speech pattern for somebody Julie describes as “extremely articulate.”

Nearly everybody in Scott’s life says they remember exactly how they reacted when they first learned of Scott’s diagnosis.

He told his two sons on a family vacation to Chile in May 1999.

The family had just finished hiking up a mountain outside the city of Pucon — “Dad kicked our asses as usual,” says Alexander, a 2005 College graduate — when Scott sat down to tell them.

The next day, while walking down from a volcano, Scott slipped and fell, tearing his meniscus. He hobbled down the rest of the volcano on his own, limping his way through the remainder of the week in Chile.

After that, Scott’s condition deteriorated rapidly. Within a year, he was already confined to his chair, unable to move most of his body.

“He was able to make it through that first year, and after that I knew it was a sign of how tough he was,” Alexander says. “We knew then that he’d fight through this.”

***

“Please don’t think I’m an inspiration, because anyone could have done what I’ve done.”

Scott is “speaking” in an automated voice — which he recorded through a special dictation program he uses — to a group of first-year medical students at Penn.

It’s early April, and Scott is giving his annual talk to the students.

As Lynn stands beside her husband — like she’s done resolutely since the two met at Penn in 1979, a year before Scott earned his undergraduate degree — about 40 students look on in awe as Scott begins to tell his story.

Scott uses the talk not just as an opportunity to discuss ALS, but also to impart some practical advice on the students.

“I just enjoy the experience,” Scott says of why he began giving the lecture years ago. “In the first year, med students learn a lot about science and little about actually working with patients. I am their worst nightmare in some respects. This lecture makes a terrible situation — facing terminal illness and chronic disability — approachable and not so scary. Humor helps.”

Humor has been a powerful tool for Scott, both in his lecture and in everyday life.

During one part of the lecture, Scott speaks of how nothing bothers him more than when he meets somebody who equates his physical condition with his intellect.

On the screen above him, he flashes a friendly message addressing those people: “I have a note from my mother allowing me to tell you to FUCK OFF. If I need help, I will ask.”

Over the years, Scott has traveled to several other schools to give his lecture.

Scott maintains that he’s not an inspiration, but few who have met him would agree.

“It’s tough to put into words what’s he’s done,” says College freshman Allison Jegla, who is from Michigan. “He’s a hero to me.”

In November 2008, Allison was watching “60 Minutes” on CBS when she saw a segment on Scott. She was awestruck by Scott’s outlook on life and decided to write him an email.

Soon after, Scott wrote her back, inviting her to an upcoming lecture at the University of Michigan.

At the time, Allison wanted to be an engineer.

Today, she wants to be a doctor.

She says she found her way to Penn because of Scott.

“He’s essentially a complete stranger to me, and yet his story led me to this place and career,” she says. “It’s 100 percent because of Scott Mackler that I’m here.”

***

You could say that Scott’s story is one that’s made up of a collection of “what ifs.”

What if he could somehow speak on his own again? What if he could move just one arm, or a hand or a finger?

Scott’s family, though, would take issue with those “what ifs.”

“You deal with reality when these are the cards you’ve been dealt,” Harvey says. “To us, this is the norm.”

In their eyes, Scott’s story is more like a tale of “wows.”

“His will to live, to continue to be productive in his work and make people understand the nature of this disease — this man is incredible,” says 1980 Wharton graduate Ron Perilstein, one of Scott’s longtime friends.

While Perilstein acknowledges that his relationship with Scott has changed since the two played soccer and were brothers at Pi Kappa Alpha together as undergraduates, much between them remains the same.

“Hey, asshole, how are you?” Ron will say to his old friend whenever the two get together.

While Scott is no longer able to go anywhere on his own, there’s rarely a dull moment in his life today.

During the day, he’s almost always with either Adam or his other nursing assistant, Dana Williams. At his Newark home, he has an “army” of physical therapy students — all of whom are in Lynn’s program at the University of Delaware — at his disposal.

The students help to feed Scott, bathe him, get him ready for work — all basic functions that he’s no longer able to do on his own.

“We have insurance, good friends, good employers and a great network of folks,” Lynn says. “We are really lucky.”

***

It’s a warm spring afternoon as Scott, Adam and I are returning to the lab after a stroll on Locust Walk.

We arrive back to his office, where the dozens of photographs lining the walls serve as a constant reminder of one thing: Scott’s family.

“Our parents instilled in us that family is number one,” says 1977 College graduate Randi Mackler Windheim, Scott’s older sister. “He’s not fighting this alone.”

Most of Scott’s fondest memories from the past decade came when he was with family.

When Alexander got married last year, Scott had a front row seat.

When Alexander and Noah earned their undergraduate degrees in 2005 and 2007, Scott was lined up on Locust Walk like any other professor, dressed in his full commencement robes.

It was one of his proudest moments as a father.

Every year, many of Scott’s family members come out to Newark to participate in the Scott Mackler 5K Run/Walk, which has been held annually since 2000. Through the race — as well as through the Scott A. Mackler, M.D., Ph.D. Assistive Technology Program — the family has raised more than $1 million to assure that no ALS patient in the Philadelphia area goes without some form of communicative technology.

For his part, in addition to communicating through his traditional eyeball-shifting mechanism, Scott has the option of using a device called a Brain Computer Interface, or BCI.

Although Scott rarely uses the BCI today because it is slow and cumbersome to put on and off, the device essentially operates as a mind reader.

To begin using the BCI, Scott puts on a rubber blue cap with rainbow-colored wires coming out of it. The cap is fitted with electrodes that pick up Scott’s brainwaves.

Once the BCI is turned on, a monitor in front of Scott begins to flash, with different letters of the alphabet becoming illuminated for split seconds apiece. When Scott sees the letter he wants to select, he concentrates on it in his mind. The BCI ultimately registers what letter Scott is thinking about and displays it on the screen.

Communicating with the BCI can be frustrating — it may take Scott six months to write a grant proposal that, years ago, he would have finished in a few weeks — but he says the technology is remarkable nonetheless.

One day, as Adam, Dana and I are trying — and failing — to get the BCI running so that I can see how it works, we notice that Scott is trying to tell us something.

“E, T, H,” he begins to spell out.

He’s trying to say “Ethernet.”

We’d forgotten to plug in the internet.

In a room with three able-bodied people, it hits me at that moment: the smartest person in the room is the one sitting in the chair, unable to utter a word.

***

Scott Mackler is free.

We’ve just finished typing an email to his son, Noah, when Scott indicates that he’s ready to go home for the day.

Tomorrow — and for days after that — Scott will get in a van at around 8:30 a.m., which will take him to the place he’s long considered his second home: Penn.

At one point in time, though, even Scott didn’t think he’d be where he is today.

Soon after his diagnosis, Scott recorded a video to his sons, assuming he would never have the chance to see them grow into adults.

“I know the future holds lots of love and joy and pride and that life goes on,” he says to the camera as he begins to run off into the distance. “I’ll be watching you along the way, and I love you very much, and I’ll see you.”

I ask Scott one final question: What do you miss most about your life before ALS?

His answer is simple and not unexpected.

“Speaking and kissing my family.”

And with that, we turn our attention back to another run through the alphabet, putting Scott’s thoughts into words — one letter at a time.

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