By Victor Greto
NEWARK — “P-R-E-T-T-Y C-O-O-L”
It takes Scott Mackler, a medical doctor and research scientist at the University of Pennsylvania, nearly eight minutes to spell two words.
His youthful face and dark eyes are tense with concentration. But the rest of his inert body, frozen by a disease that has stopped his ability to move his muscles, leans back in a wheelchair pulsing with machines that help him breathe, eat, and excrete waste.
In a complex dance between his mind and the latest technology, the 47-year-old Mackler stares at rows of flashing letters and numerals. Sixteen electrodes attached to a cap over his thick shock of gray and black hair try to read his brainwaves to see which intricate spark of thought refers to a corresponding letter or number.
If and when Scott’s brainwaves can be predicted by the computer, the brain-computer interface will allow him the power to use a computer and communicate through the screen display using only thoughts.
It is the latest experimental device Mackler and his family are using to let him interact with the world.
For a neuroscientist who spoke nearly as rapidly as he thought, and who lived to research and teach, the nuanced ballet of communicating with others has become much of what life is about.
“I don’t think you get what you deserve in life,” Scott’s wife, Lynn Snyder-Mackler, says later. “I don’t let myself think about the emotional aspects of this. If I fell apart, Scott would, too.”
Even so, says Lynn, a professor of physical therapy at the University of Delaware, “Aside from this little blip in the curve, it’s a normal life.”
***
This emotional odyssey began for Scott, Lynn, and their two sons, Alexander and Noah, about seven years ago when Scott first showed symptoms of amyotrophic lateral sclerosis. Also known as Lou Gehrig’s disease, from the baseball player who got the disease and died from it in 1941, ALS is a progressive neurodegenerative ailment that attacks nerve cells in the brain and spinal cord, killing off motor neurons that move the body’s muscles.
More than 30,000 people in the U.S. have the disease, according to the ALS Association. About 5,000 Americans are diagnosed each year with the disease.
There is no cure. Neither Scott’s sensory nerves nor his mind — how he feels pain and longs to express himself — have changed. But the progression of the disease has transformed his attitude and feelings toward life.
An athlete, Scott first noticed symptoms in December 1998 when he began to lose his grip on a tennis racquet. But even while he was getting the racquet’s grip repaired, he soon found he could not tell jokes without laughing before telling the punch line, revealing a lack of control of his outward emotions, an early symptom of the disease. This was soon followed by twitching muscles in his right arm, and progressively slower running times at the track.
Although it was four more months before he was officially diagnosed, both Scott and Lynn sensed what was coming. Within a year after those early symptoms, his condition deteriorated rapidly. Now, he cannot even breathe or swallow on his own.
But he still can communicate with his family and with those whom Lynn has hired to help him groom and work.
Before his condition deteriorated, Scott created a system whereby he split the alphabet into three sections — a-h, i-q, r-z — so he could continue to personally speak with others.
***
Face to face, Scott communicates through a series of glances, rolls of the eyes that look at the other person as he or she hits, first, on the correct section of the alphabet, and secondly, on an individual letter within the section.
“It took me maybe a couple months to get to know him,” says Dana Crumpler, a nurse and single mother who has been caring for Scott for four years.
“Once you start to work with him, I figured it out, know what he’s going to say before he says it,” she says.
Like now: Scott’s eyes roll toward Dana. She leans over and, in a conspiratorial whisper, she hashes out the fact that the cap is too tight around Scott’s head.
When the cap is removed, Scott smiles — a killer smile that seems to trump both words and, for now, a disease that will ultimately not be trumped.
For Scott and his family, the future is now.
“Life expectancy of the disease is two to five years” from diagnosis, Scott’s 20-year-old son Noah says. “And he’s almost two years over that. And he’s still here.”
And still working.
***
Scott was 14 when he knew he wanted to be a physical therapist.
That’s the age when he briefly went to one after sustaining a minor injury in a car accident with his mother in Bloomfield, N.J., just outside of Newark, where he was born in May 1958.
But physical therapy was not enough to quench his curiosity. As he became fascinated with science, Scott wanted to do more. When he graduated from Penn in 1980, he earned degrees in both biology and physical therapy, with a minor in international relations. He played on the college soccer team and ran regularly.
“He’s kind of a Renaissance man,” says Lynn, 49, who met Scott at Penn, where she earned a graduate certificate — but only in physical therapy.
Lynn and Scott met in the fall of 1979, in the back row of a classroom where they both sat. “Luckily, we were both back row people,” she says.
They soon became good friends, but did not date until the following summer. They married in 1981.
As a junior at Penn, Scott’s mind, still hungry, turned toward becoming a medical doctor.
“He loved science a lot and wanted to keep going,” says Lynn. “He wanted to do research and was interested in how pain and addiction were related in the brain.”
He also liked working with people.
“He misses that the most, dealing with patients,” Lynn says, brushing back strawberry-blonde hair from her narrow face. She glances over to Scott. “But he still teaches medical students.”
Now, Scott presents lectures for medical students and young doctors through a PowerPoint presentation, speaking through a voice synthesizer that clothes his words in a deceptively gentle voice.
“That’s our latest fun thing to do,” Lynn says, “finding different voices for him.”
***
One lecture, which characterizes the progression of his illness and what he has learned as a doctor from the experience, veers from periods of comic relief to a continued frustration and barely-suppressed anger toward those who refused to quickly admit to him the truth of his condition.
Peppered among the nine rudimentary lessons he says he’s learned — from always listening to the patient to warning that you never can have enough insurance — are frustrated asides, including one where he calls a doctor an “idiot” for being blind to his condition and his feelings.
“Hey stranger who is looking at me as if I am retarded or I have a communicable disease,” one slide reads. “I only recently was running long distances, climbing volcanoes, and speaking to large audiences. You may think I am a sad sack, but that only reveals your pigheadedness and ignorance.”
In fact, soon after his diagnosis but before his condition deteriorated, in 1999 Scott and his family traveled to Chile to climb a volcano. They also traveled to Africa and Europe the following year.
But it also was on these trips where Scott began to realize the limitations of the physical reality of ALS.
As the deterioration increased and his body froze, the intensity of his longing to communicate increased proportionately.
One cliché Scott includes in his lecture — “ Never judge a book by its cover” — is qualified by his own understated truth: “There may be emotional or intellectual fires hidden inside.”
During the mid-1980s at Penn’s graduate school, Scott enrolled in a combined degree program. In 1986, he earned a doctorate in anatomy, and became a medical doctor. By then, he and Lynn had had two children.
The family moved to Boston where Scott finished his residency in internal medicine, and Lynn received a PhD in anatomy and physiology at Boston University. While Lynn found a job at the University of Delaware in 1989, Scott juggled offers from Boston’s Tufts University, Penn, and Baltimore’s Johns Hopkins.
Lynn’s UD choice acted as a geographical inspiration. “This was in between, so it was great if he went either to Philadelphia or Baltimore,” Lynn says.
Scott chose his alma mater. “He bleeds red and blue,” Lynn says, referring to Penn’s colors, but also to two of the three colors of his beloved New York Giants.
***
In two years, Scott got a permanent job on the faculty of Penn’s Department of Medicine, and, later, a dual appointment at the Department of Pharmacology where he has remained researching the biology of addiction.
During the mid-1990s, Scott identified a protein in the brain he called NAC-1 (for a part of the brain where it’s found) an excess of which he has proven to reduce a rat’s likelihood of showing the behavioral aspects of addiction to cocaine.
“Over the years, we’ve been trying to figure out the significance of it,” says Scott’s colleague, Dr. Michael Stromberg, a behavioral pharmacologist at the Veterans Administration Hospital in Philadelphia. Stromberg has known and worked with Scott for more than a decade. “Scott is interested in the molecular events, and I’m interested in the behavior.”
Ever since his find, Scott and his colleagues have been experimenting with the protein and gene that produces it, but nothing has been applied to people as yet.
Now, they’re working with mice. “It’s like an onion,” Stromberg says of the research. “You start peeling layers off one at a time, and that’s what my relationship with Scott has been.”
Stromberg also has worked with Scott writing the grants to continue their mutual research.
In order to work, write and use a computer, Scott uses a device called VisionKey, an “alternative keyboard,” which, when he wears it, makes him look like a cyborg, his son Alexander says.
A miniscule infra-red camera lens reads reflections from Scott’s pupil as he “dwells” or concentrates on one letter or another through the two-ounce device, mounted on eyeglasses. The letter is then typed on a computer screen.
Programmable “rate-enhancement” software predicts the remaining letters of the words he’s trying to write. He can backspace, or erase words, if the computer guesses wrong.
Scott does much of his communication this way, writing e-mails to friends, family and co-workers.
Time-consuming and painstaking, it’s with this device that he co-wrote with Stromberg two successful grants for his research. He was awarded one, from the National Institute for Drug Abuse, for more than $1 million over three years.
Although his condition has leveled off in the past year, the rate of speed with which he writes has lessened over time. That compelled Scott and Lynn to try out the more sophisticated technology of the brain-computer interface system, part of a program offered by the New York Department of Health, which requires no eye movement.
They found out about the New York program from the Internet. “We always keep an eye open for new technology,” Lynn says.
They also were lucky because Lynn’s UD department boss knew the scientist in charge of the brain-computer interface program in New York.
“They communicated, and over six months time, we were able to set this up,” Lynn says. “If he can speak faster (than VisionKey) with this, it’ll be wonderful,” Lynn says. “To have a real-time conversation — wouldn’t that be wonderful?”
***
It’s just as wonderful that Lynn’s position at the university allows her to e-mail incoming physical therapy students and ask if they want to help care for Scott.
Sheathed within a body that cannot move or speak, Scott receives help from his family and from the students Lynn hires each year to help him get ready for work, and ready for bed.
“We pay them,” Lynn says, “but nowhere near what they’re worth.”
This year, UD students Angela Hutchinson, earning a doctorate in physical therapy and Jessalyn Ciampa of Albany, N.Y., have been hired to help out during mornings and evenings. They are aided by live-in help, Jill Heathcock and her husband Bob Cardillo, and Crumpler, the nurse.
Crumpler takes Scott to Penn four days a week, is with him while he works, and returns home with him in the evening.
“Sometimes at first you don’t know what to think because you don’t get any type of reaction,” she says of relating to Scott. But one gets used to the communication system and she has become good at it, she says.
“I still make mistakes,” Crumpler says, “but you learn something new every day.”
In fact, Scott seems adept at giving advice on relationship issues, something which Crumpler and Hutchinson, who has been caring for Scott for nearly two years, says they’ve discussed with him.
“Upon first meeting him and starting to work with him, I thought I had no idea what I was getting myself into,” Hutchinson says, unsure of the extent of Scott’s needs. “Then, slowly, I picked up how to care for him, including transferring him (from the chair into the bathroom), getting him dressed, ready for work, shower, all that type of stuff.”
She says it took her nearly a year to “get really comfortable” with communicating with Scott.
But Hutchinson’s gaze toward Scott as they “speak,” her thick black hair hanging expectantly past the front of her shoulders, reveals a growing intimacy.
“It seems that he’s very interested in getting to know me as a person,” she says. “Sometimes we talk about deeper things, and that’s come with time and experience.”
***
Those deeper things include personal issues life in general, and his life in particular. “He’s completely comfortable with me asking him about his disease and how he’s dealt with it and how it’s affected his family,” she says.
For Ciampa, a first-year helper, this process is just beginning. “I would say the first month or two months I just focused mainly on getting the routine down pat and making sure I was doing everything correctly with him,” she says.
In the morning, that includes shaving, showering, giving him his medication and feeding him through a tube.
Heathcock was one of the first of Lynn’s students to help the family with Scott. She started more than five years ago, and moved in shortly afterward. She’s been living with the Macklers since 2001, and feels like a part of the family.
She even married while living there, and shares a basement apartment with her husband Bob. But she will be graduating in the spring, and is expecting her first child. They will move out by the summer, she says.
“If I think about it a lot, it’s sad,” Heathcock says. “But it’s like normal life for everyone. In general, it’s just the way things are. Everyone goes to work and goes to movies on weekends, and we live pretty normally.”
It’s a normalcy defined by the fight within Scott to continue to research, and to say what he thinks and feels.
Part of that fight includes the $750,000 they’ve raised over the years for the Scott A. Mackler, MD, Ph.D. Assistive Technology Program of the ALS Association’s Greater Philadelphia Chapter. The money goes to others toward purchasing the expensive communication devices insurance almost never covers.
“We’re fortunate to be able to afford the technology,” says Lynn. “Many others are not.” The VisionKey system that Scott uses cost about $5,000.
Aside from the essentials that keep Scott breathing, enhanced communication remains the family’s goal.
But Scott’s communication with his family is not limited to whispered, intimate exchanges, or to a technologically potential real-time.
***
Thinking ahead, Scott made several videos before he lost the power of speech to be shown to members of his family when they reached milestones.
On the days of their high school graduations, Alexander and Noah saw videos of their father.
Alexander saw another video of his father after he recently graduated from Penn.
Neither son will say what their father said in the video, but their anticipation of the next videos is palpable.
“I have no idea what he has set out for us, but I know I’ll have another one for when I graduate college,” says Noah, a junior at Penn. “But I don’t know what else to expect.”
But he does know what he’s learned.
“What happened to my father was a horrible thing,” Noah says. “But — I know this sounds weird — it was one of the best things that could have happened to me. When bad things happen in my life, I take a step back. I know I’ve gone through worse, and I know I can deal with it.”
Noah’s attitude did not appear overnight, but, “Once we accepted what it was and when it happened, it became a part of our normal lives.”
It became that way, he says, because of Lynn and Scott.
“Our parents are really strong,” Noah says. “Dad was happy with the way he lived his life. He was content.”
Lynn’s strength is deceptive at times.
“I cry in the shower, so nobody notices it,” she says. But those crying jags end when she turns off the water. “There’s too much to do.”
She’s also happy with her career. “We’re both productive and my career is at a stage where people want my time,” she says.
Lynn and Scott seem to have mastered the art of immersing themselves in their work. Lynn and her colleagues have published papers on the risk factors of knee replacement surgery, as well as work on arthritis.
While their careers eat up most of their waking hours, Lynn and Scott’s insistence on him not only living, but living as normally as possible, seems to begin and end with the family.
“I accept it,” Scott says of ALS, through Lynn, as she picks out the letters of the alphabet through his eyes. “It beats the alternative.”
It’s an attitude that transcends technology, videos and even their careers.
In his lecture, one of the slides shows a standing and healthy Scott, passionately kissing Lynn.
Underneath the picture, Scott wrote a caption: “The real reason for my serenity.”
There actually are times when there is nothing left to say