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PULTENEY STREET SURVEY - FALL 2018

Dr. Carol Pappas

Dr. Carol Pappas ’71

neurologist

At the helm of innovative research and treatment for nearly 40 years, Dr. Carol Pappas ’71 pushes the boundaries of what is known about the brain. Her advancements in the treatment of Parkinson’s disease are a testament to the power of endless curiosity and so we turned to Pappas to ask:

Q: What have we learned about Parkinson’s disease lately and how has this translated into treatment?

A: “Traditionally Parkinson’s disease was thought to be primarily expressed through motor symptoms. As we’ve gotten better at treating these motor symptoms, we’ve learned there are many, many non-motor symptoms involved. These range from GI tract issues to psychosis to REM sleep behavior disorder, which is when you essentially act out your dreams, as well as anosmia, or the lack of sense of smell, and autonomic dysreflexia, when autonomic functions like blood pressure control can be impaired.

If you said to someone ‘what’s Parkinson’s?’ they probably wouldn’t come up with that constellation of symptoms.

When a person is first diagnosed, they’ve probably lost about 70 percent of the neurons that produce dopamine in the substantia nigra. As the disease progresses, those neurons eventually die and the brain becomes totally dependent on pills that contain Levadopa, which is widely considered to be the most effective medication, to provide any dopamine. As a consequence of lacking continuous dopamine, patients experience a peak of dopamine that falls off as the pill is metabolized, like a yo-yo. The consequence of this pulsatile stimulation is that patients develop all kinds of motor complications. When you see Michael J. Fox twisting for instance, it’s because his receptors have a hyperactive response to the pills. It’s not the disease; it’s the treatment. He’s over-treating himself because if he didn’t he wouldn’t be able to move.

Modern approaches try to create continuous treatment, which more closely approximates the non- Parkinsonian brain. These approaches include patch therapy in which a medication is absorbed by the skin over 24 hours, deep brain stimulation and continuous pump therapies.

There are now several studies about the Parkinson’s Associated Risk Score, which calculates the risk of developing Parkinson’s Disease later in life. A drug to halt progression of disease or prevention of disease would be ideal, but is not yet available.

The disease is always going to progress, though we’re doing a great job with advances in treating symptoms. We have so many questions and are getting some answers, which lead to many more questions. Nobody’s saying the word ‘cure’ yet, but we’re really teasing down deep into molecular changes.

The Doctor Is In

A leading expert on Parkinson’s and other neurological diseases, Dr. Carol Pappas ’71 has spent nearly 40 years pushing treatment research into new frontiers, conducting surgery and deep brain stimulation, teaching students and residents, and treating her patients in private practice, about half of whom have degenerative disorders. For her, the science has become personal, and the stakes are higher than what CT or MRI scans can show.

“The nervous system makes us who we are,” says Pappas, who became captivated with the subject at William Smith after initially pursuing an English major.

When Pappas graduated, she enrolled in the Ph.D. program in neurophysiology at SUNY Upstate Medical Center, but found that her passion for working with people made her better suited to be a physician and decided to pursue her medical degree, too.

The nervous system was then and “still is somewhat uncharted territory,” Pappas says. “Things I was taught in the beginning of grad school have been totally disproven since. Even with my Ph.D., I did work that was written in stone since 1920, and guess what? It was wrong. I got to be part of that change, which was really exciting.”

Among her many accomplishments, Pappas was the first in the country to administer the Duopa procedure. Duopa, an intestinal gel version of Levadopa, is alternative treatment that circumvents some of the gastrointestinal complications that can prevent people with Parkinson’s from absorbing the pill form, a groundbreaking development replacing an avenue that “wreaks havoc” on the body.

Instead of a pill, patients have a small feeding tube implanted into the duodenum where Duopa is pumped. Pappas performed the procedure on a mountain climber who had scaled Mount Everest before she developed Parkinson’s. Because of the disease, she had fallen and broken her neck but with the Duopa procedure, the patient “went from being on a ventilator to walking the next day,” Pappas says.

In another case, she was treating a 62-year-old man who’d had Parkinson’s for 20 years. “He had a tube for feeding, couldn’t talk, couldn’t move,” Pappas says. “I remember meeting him and being devastated at this otherwise healthy guy,” but after deep brain stimulation, and the institution of Duopa therapy “now he walks and he talks, doesn’t have a tremor. These therapies have been life changing for him. There’s nothing more rewarding when patients undergoing deep brain stimulation are awake — they’re shaking like mad and you turn the stimulator on, and the tremors stop immediately. They can use their hands, wriggle their fingers, write.”

Like the other efficacious treatments, deep brain stimulation is one that can be finely tuned to a patient’s needs. Electrical stimulation is delivered through an electrode that is placed in tiny motor nuclei in the brain. DBS uses electrical stimulation, rather than medication to treat the symptoms of Parkinson’s Disease. There is a generator, much like a pacemaker, placed under the collarbone. The electrical output of the electrode can be adjusted in frequency, voltage and configuration through the skin overlying the generator.

“You can adjust the programming of each electrode as patient’s symptoms progress,” Pappas says. Similarly, medications can be adjusted over the course of the disease to keep the symptoms and complications in check.

That evolving approach to therapy is important because Parkinson’s, she says, is “not a moment in time — it’s a lifetime, and the more we can change therapies to honor the progress of disease, the better we’ll be.” –Andrew Wickenden ’09